Emergency Medicine: The Core Curriculum

Contents
.......
Contributors
Preface
Acknowledgments .
1.3.5
xxlll
xli
1.4
1.4.2
Wesley
1.1.1 MotorAbnormalities.... 4
1.1.2
1.1.3
StructuralDisorders
....
Disorders
1.5.3
1.5.4 Tirmors
Wesley
1.1.4 InfectiousDisorders.... 8
1.2 Liver..
1.6.2
Wayne J. Farnsworth
1.6.3
David G. Heisig
1.2.1 Hepatitis
l0
1.6.4
David G. Heisig
1.2.2 Cirrhosis.
1.2.3
Prince
1.3.1 Cholecystitis
Louise A. Prince
1.3.2 Cholangitis
LouiseA. Prince
1.3.3 Cholelithiasis and
Choledocholithiasis. . . .
Louise A.
Prince
....
Inflammatory Disorders
Paul E McGuire
InfectiousDisorders
....
33
34
35
37
39
Paul E McGuire
Failure.
Gallbladder and Biliary Tract . . . .

Louise A.
StructuralDisorders
Paul F McGuire
1.6.5 Tumors
Hepatic/Hepatorenal
14
1.7
David G. Heisig
1.3
Paul E McGuire
33
Paul E McGuire
12
David G. Heisig
1.2.4 Tumors
David G. Heisig
1.2.5 Abscess.
David G. Heisig
32
P Eilbert
1.6 SmallBowel..
1.6.1 MotorAbnormalities....
Wayne J. Farnsworth
26
P Eilbert
Inflammatory Disorders 28
Wesley P Eilbert
Peptic Ulcer Disease. . . .
30
Wesley P Eilbert
Wesley
Wayne J. Farnsworth
1.1.5 Tumors
24
P Eilbert
1.5.2
Wayne J. Farnsworth
Inflammatory
23
1.5.1 StructuralLesions......
Wayne J. Farnsworth
Ilayne J. Farnsworth
Carcinoma
1.5 Stomach
Gary A. Johnson
1.1 Esophagus
20
Louise A. Prince
Tumors and
Louise A. Prince
Disorders
19
1.4.1 Inflammatory
ABDOMINALANDGASTROINTESTINAL
Chapter Editor: John B. McCabe
1.0 AbdominalandGastrointestinal
pancreas.
Louise A. Prince
xliii
DISORDERS
l9
Tumors
LouiseA. Prince
David G. Heisig
1.7.1 MotorAbnormalities. . .
David G. Heisig
14
15
.
17
.
17
1.3.4 Gallstonelleus........ 18
Louise A. Prince
vll
39
40
1.7.3 Colitis.
15
15
1.6.6 VascularDisorders .....

Bowel.
Large
42
43
David G. Heisig
1.7.5 Tumors
1.8
44
David G. Heisig
Rectum and Anus

llayne J. Farnsworth
Structural Disorders . . .
Wayne J. Farnsworth
1.8.1
1.8.2
Inflammatory
44
Disorders
45
48
Wayne J. Farnsworth
1.8.3 Tirmors
Wayne J. Farnsworth
50
viii /
CoNrrNrs
CARDIOVASCULARDISORDERS ....
Chapter Editor: E. Jackson Allison, Jr.
2.5.3 Ll,rnphatics
52
2.0
Cardiovascular Disorders
Marc C. Restuccia
2.1
Pathophysiology....
2.1.1 CongenitalDisorders....
52
2.6
Congenital Abnormalities of the

Cardiovascular System
2.6.1
FamiliaVGenetically
TransmittedDisorders.
RichardA. Craven
54
2.6.2
Marc C. Restuccia
2.1.3
2.2
Effects ofAging on
the Heart.
2.7
Acquired
2.2.1
57
Cardiac Failure .
John T Meredith and
Charles K. Brown
2.2.3
John T. Meredith and

Charles K. Brown
Ischemic Heart
65
2.9
2.2.4 Endocarditis
79
2.10
Michael K. Kerr and

Charles K. Brown
Valvular Heart Disease .
Michael K. Kerr and
Charles K. Brown
81
150
152
154
154
156
Robert L. Brown and
Wlliam J. Meggs
2.10.3 Rheumatologic
95
157
Robert L. Brown and

IVilliam J. Meggs
PericardialEffirsion/
Tamponade
2.10.4
96
John T Meredith
Diseases of the Conduction System
(Disturbances of Cardiac
Renal
158
Robert L. Brown and
llilliam
2.10.5
Rhythm).
98
John E. Gough and

E. Jacl<son Allison, Jr
J. Meggs
Toxic Exposures. . . . . .
Robert L. Brown and
159
Ililliam J. Meggs
2.11
2.4.1 Dysrhyhmias
100
John E. Gough and

E. Jackson Allison, Jr
ConductionBlocks. . . .
John E. Gough and
Treatment
2.11.1
Therapy.
.
160
160
Richard C. Hunt, and
111
Francis L. Counselman
2.11.2 PharmacologicAgents...
Jr
Acquired
2.5.1 Arterial
Modalities
Thrombolytic
Robert L. Brown,
Diseases of the Circulation,
ll4
ll4
2.11.3
Peggy E. Goodman and

Amy A. DeStefano
Pegglt E. Goodman
144
Diseases
94
John T. Meredith
2.5.2 Venous
142
Robert L. Brown and

lVilliam J. Meggs
Endocrine and Metabolic
John T Meredith
2.5
139
MyocardialManifestations
of Systemic Diseases
Robert L. Brown and
2.10.2
Pericardium
E. Jacl<son Allison,
Gary S. Setnik and

Arshad Khan
2.8.2 Chronic Hypertension . .
Gary S. Sernik and
Arshad Khan
PrimaryTumorsoftheHeart . . ., .
G. Richard Braen
Diseases of the
2.4.2
..
AcuteHypertensive
Crisis .
92
2.3.1 Pericarditis
2.4
.. . ..
Lltilliam J. Meggs
John T. Meredith and

Charles K. Brown
2.3.2
134
2.10.1 Infections
2.2.6 Myocarditis.
2.3
Gary S. Setnik and

Arshad Khan
2.8.1
David P Hightower and

Charles K. Brown
2.2.5
CardiacTransplantation .
62
Disease
131
RichardA. Craven
2.8 Hypertension
57
2.2.2 Cardiomyopathy
..
Disorders Due to
Anatomic Anomalies. . .
57
Marc C. Restuccia
Diseases of the Myocardium,
130
RichardA. Craven
52
52
Marc C. Restuccia
2.1.2 AcquiredDisorders.....
129
Pegg E. Goodman
125
Robert L. Brown,
CardiacPacemakers ....
Robert L. Brown,
161
163
CoNrnvrs
2.11.4 Surgicallnventions..... 165
Robert L. Brown,
3.2.4 Vir.f..
3.3
l7l
CUTANEOUS DISORDERS . . .
Chapter Editor: RichardVAghababian
3.0
3.1
Disorders
and
Ciottone
Dermatitis
Eric W. Schmidt and
Constance G. Nichols
3.1.1 Acne. .
Cutaneous
Eric W Schmidt
Gregory R.
Constance G.
.l
.2
3.3.2
172
3.3.3
172
Nichols
3.4
173
Dyshydrotic Eczema. . .
Nichols
3.1.6 Lichen Simplex
Chronicus
Eric W Schmidt and
3.1.7 Psoriasis
Eric W Schmidt and
3.1.8 Seborrhea
Eric W Schmidt and
3.4.5
174
l8l
181
Nichols
l8l
181
181
Douglas Scudder
3.5.2
174
ErythemaNodosum . . .
182
Eric W Schmidt and
174
3.6
Douglas Scudder
Vesicular/Bullous Lesions
3.6.1 Pemphigus/Pemphigoid.
Eric W Schmidt and
174
Scalded Skin
Syndrome
3.7
Cancers
3.7.1
182
E Siraco and
Basal Cell Carcinoma . .
Steven E Siraco and
3.7.3 Melanoma.
3.7.4
176
177
182
Schmidt and
Douglas Scudder
175
175
175
182
l(
Steven
175
182
DouglasScudder
3.6'2
Eric
Eric W Schmidt and
Nichols
3.1.10 Actinic Keratosis/
Photosensitivity
EricW Schmidt and
3.1.11 Nummular
Eczema.
Eric I4! Schmidt and
Infections
3.2.1 Bacterial
Eric W Schmidt and
Douglas Scudder
3.2.2 Fungal.
EricW Schmidt and
Douglas Scudder
3.2.3 Parasitic
Ericll! Schmidt and
Douglas Scudder
Nevi . .
Eric W Schmidt and
Erythemas
3.5.1 Erythema
Multiforme
Constance G.
3.2
181
Eric W Schmidt and
Keratoacanthoma...... 174
180
Douglas Scudder
3.5
Eric W Schmidt and

Stasis
Urticaria/Angioedema. . .
Eric W Schmidt and
173
Constance G.
3.1.9
180
Douglas Scudder
EricW Schmidt and
Constance G.
Douglas Scudder
PapularA'{odular Lesions
3.4.1 Epidermoid Inclusion
CYst . .
Eric W Schmidt and
Douglas Scudder
173
Eric W Schmidt and
3.1.4
Douglas Scudder
Purpura and Petechiae. .
Eric W Schmidt and
Douglas Scudder
Millium.
Nichols
3.1.3 Contact
Nichols
180
171
Eric W Schmidt and
Constance G.
180
3.3.1 PityriasisRosea........
Eric W Schmidt and
Atopic.
Constance G.
Douglas Scudder
Maculopapular Lesions
Eric W Schmidt and
Eric W Schmidt and
178
EricW Schmidt and
183
183
Steven F Siraco and

Squamous Cell
Carcinoma
184
Steven E Siraco and

Pigmented Lesions of
the Skin
Steven E Siraco and
BenignNeoplasms.'...
Steven
F Siraco and
184
185
/ ix
x /
CoNrBNrs
4.0 ENDOCRTNE, METABOLIC,AND

NUTRTTTONAL DISORDERS .........
4.1
4.2
4.3
4.4
4.5
4.6
4.7
4.8
tB7
Disturbances
187
Singer
4.1.1 Metabolic
189
Andrew Singer
4.1.2 Mixed Acid-Base
Disorders
192
Andrew Singer
4.1.3 Respiratory
193
Andrew Singer
Adrenal Disease
195
Mark D. Crockett
Fluid and Electrolyte
Disturbances
197
Daniel M. Joyce
4.3.1 Calcium
197
Daniel M. Joyce
4.3.3 Magnesium
200
Daniel M. Joyce
4.3.4 Phosphorus
201
Daniel M. Joyce
4.3.5 Potassium
202
Daniel M. Joyce
4.3.6 Sodium
204
Daniel M. Joyce
4.3.7 Water .
207
Daniel M. Joyce
4.3.8 Syndrome of Inappropriate
Secretion of Antidiuretic
Hormone.
207
Daniel M. Joyce
Hypernatremia 208
Daniel M. Joyce
Glucose Metabolism.
210
4.4.1 DiabetesMellitus...... 210
Sandra M. Schneider
4.4.2 Hypoglycemic
Syndromes
210
Sandra M. Schneider
Hyperglycemia....,... 212
SandraM. Schneider
Nutritional Disorders
217
Rob J. Edwards
4.5.1 Wernicke/Korsakoff
Syndrome
218
Rob J. Edwards
4.5.2, Vitamin Deficiency and
4.5.3 Vitamin Excess .
219
Rob J. Edwards
Parathyroid Disease
221
Tomer Feldman
Hyperparathyroidism .
222
Tbmer Feldman
Hypoparathyroidism. .
223
Tomer Feldman
Pheochromocytoma . . .
223
Michael A. Pellegrino
Pituitary Disorders
225
Jerry R. Balentine
4.8.1
panhypopituitarism. . . .
4.8.2
Jerry R. Balentine
Growth Hormone
Acid-Base
Andrew
Abnormalities
226
226
Jerry R. Balentine
Acromegaly
227
Jerry R. Balentine
4.8.3 Tumors
4.9
22].
Jerry R. Balentine
Thyroid
Disorders
228
Gary A. Johnson
4.9.1
4.9.2
4.10
Hyperthyroidism/
Thyroid Storm. .
22g
Gary A. Johnson
Hypothyroidism/
Myxedema
229
Gary A. Johnson
Endocrine Manifestations
Neoplasia.
of
229
Jerry R. Balentine
Syndrome of Inappropriate
DietaryHormone......
230
Jerry R. Balentine
Hypercalcemia
230
Jerry R. Balentine
Hypoglycemia
231
Jerry R. Balentine
ENVIRONMENTAL DISORDERS..... 233

Chapter Editor: E. JacksonAllison, Jn
5.0
5.1
Disorders
Environmental
Eunice M. Singletary
Diving Emergencies/Dysbarism . .
Acute Gas Embolism . .
Decompression
5.1.1
5.1.2
5.2
234
236
240
Sickness
241
Submersion
E. Jacl<son Allison, Jr and
Incidents
242
MichaelB.Seim
5.2.1 NearDrowning........
5.2.2
5.3
Injuries.
Electrical
Gordian W O. Fulde and
5.3.1
5.4
E. Jackson Allison, Jr
and Michael B. Seim
Cold Water Immersion .
E. Jackson Allison, Jr. and
Michael B. Seim
Lightning Injuries . . . . .
243
243
244
247
Gordian W O. Fulde and

High-Altitude Illness .
249
Eric M. Kardon
5.4.1 Acute Mountain Sickness 251
Eric M. Kardon
5.4.2 High-Altitude Cerebral
Edema.
Eric M. Kardon
253
Cox-rrNrs
5.4.3
5.5
5.6
5.7
5.8
5.9
Pulmonary
Edema.
255
Eric M. Kardon
259
Radiation Injury. .
Ralph B. Leonard
268
Poisonous Plants. .
Jerry D. Thomas and
Robert P Ferm
280
Smoke Inhalation. . . .
Ralph B. Leonard and Roy L. Alson
Temperature-Relatedlllness...... 285
285
5.8.1 Heat . .
Ll'alter C. Robey III and
Hervy B. Kornegay, Jr
291
5.8.2 Cold..
Walter C. Robey III and
Michael B. Seim
298
5.8.2.2 Frostbite
Walter C. Robey III and
Rffi V Terzian
302
Bites and Stings .
George Podgorny and
303
5.9.1 Arthropods
George Podgorny and
306
5.9.2 Mammals
George Podgorny and
EuniceM.singletary
5.9.3 MarineOrganisms...... 308
JohnE.Gough
315
5.9.4 Reptiles.
GeorgePodgornyand
6 HEAD,EAR,EYE,NOSE,THROAT
323
DISORDERS
6.0 Hea4 Ear, Eye, Nose,
323
Throat Disorders . . .
IVilliam J. Levin
326
6.1 Ear....
William J. Levin and Vijai V Chauhan
326
6.1 I
Cellulitis
IfilliamJ. Levin and
VijaiV Chauhan
326
6.1.2 Foreign Bodies .
Wtilliam J. Levin and
Vijai V Chauhan
327
6.1.3 Labyrnthitis.
William J. Levin and
Vijai V Chauhan
6.1.4 Malignant Otitis Externa 327
IVitliamJ. Levin and
VijaiV Chauhan
327
6.1.5 Mastoiditis
VijaiV Chauhan
6.1.6 M6nidre's Disease. . . . . . 328
Itilliam J. Levin and
Vijai V Chauhan
Otitis Externa
lililliam J. Levin and
Vijai 11 Chauhan
Otitis Media
6.1.7
High-Altitude
6.1.8
328
328
VijaiV Chauhan
6.1.9
Tympanic Membrane
Perforations.
329
Mlliam J. Levin and

VijaiV Chauhan
6.2 Nose...
330
James M. Leaming
6.2.1
Epistaxis
Anterior.
330
James M. Leaming
6.2.2
EpistaxisPosterior......
James M. Leaming
6.2.3
Nasal Foreign Body . . .
332
333
James M. Leaming
6.2.4 Rhinitis.
334
James M. Leaming
6.2.5 Sinusitis
6.3
335
James M. Leaming
Oropharynx/Throat. .
W John Zehner
Foreign Bodies
W John Zehner
6.3.1
338
338
6.3.2 Gingivitis
339
W John Zehner
6.3.3
Larynx/Trachea.....
WJohnZehner
'. '
6.3.4 Ludwig'sAngina.....'.
339
341
W John Zehner
6.3.5
OralCandidiasis......
'
342
W John Zehner
6.3.6 Pericoronitis
6.3.7
342
WJohnZehner
Periodontal
Abscess.
342
lT John Zehner
6.3.8,
Tonsillitis/
6.3.9 Pharyngitis
WJohnZehner
342
6.3.8
Peritonsillar Abscess . . .
W John Zehner
6.3.10
RetropharyngealAbscess 343
6.3. I
343
W John Zehner
Sialadenitis
344
W John Zehner
6.3.12 Sialolithiasis
344
W John Zehner
6'3'13 Stomatitis
344
W John Zehner
6.3.14
Temporomandibular Joint
Disorders
345
W John Zehner
6.3.15 Uvulitis.
6'4
EYe. . .
James M' Leaming
6.4'l
345
W John Zehner
345
External Eye. .
James M. Leaming
345
/ xi
xii /
CoNrrNrs
6.4.2
Anterior Pole. . .
James M.
Leaming
7.6.1
350
DISORDERS
Chapter Editor: G. Richard Braen
7.1 Hemostatic Disorders
JelfreyR.Suchard
7.1.1 Clotting Factor
Disorders
Jeffrey R. Suchard
7.1.2 Disseminated
Intravascular
Coagulation.
Jeffrey R. Suchard
7.1.3 Platelet Disorders . . . . . .
Jeffrey R. Suchard
7.1.4 von Willebrand's
Disease
Jefftey R. Suchard
7.2 Lymphomas
Michael W Ardagh
HEMATOLOGIC
Hodgkin'sDisease......
W Ardagh
Non-Hodgkin's
Lymphoma
Michael W Ardagh
7.3 Pancytopenia
Jeanne M. Basior
7.4 Red Blood Cell Disorders
7.4.1 Anemia.
Jeanne M. Basior
7.4.2 Polycythemia
Jeanne M. Basior
7.5 Transfusions.
7.5.3 ComponentTherapy....
Anthony J. Billitner Ill
Robert E Reardon, and
Douglas R. Migden
7.5.2 Complications
Anthony J. Billittier IV
Robert E Reardon, and
Douglas R. Migden
Alternatives to
Transfusion of Blood
Products
Anthony J. Billittier Ill
RobertE Reardon, and
Douglas R. Migden
7.6.2
7.6
392
392
Thomas Nowicki and
7.6.4
361
Marc Borenstein
MultipleMyeloma
.....
393
Thomas Nowicki and
Marc Borenstein
361
362
IMMUNESYSTEMDISORDERS
8.0
8.1
367
8.2
369
8.3
Immune System
.....
Disorders
395
395
Michael S. Beeson
Humoral
Immunity.
395
Michael S. Beeson
Cellular Immunity
Michael S. Beeson
396
Chemical
396
Mediators
Michael S. Beeson
8.4 Complement.
370
8.5
371
Diseases
Autoimmune
Paul T. Preisz
Acute Rheumatic Fever .
8.5.2
373
39't.
Michael S. Beeson
8.5.1
372
397
398
PaulT.Preisz
Collagen Vascular
Diseases
399
Paul T. Preisz
8.5.5 Vasculitis
374
8.6
374
374
PaulT Preisz
8.7
381
381
401
401
HIV Disease/AIDS . . . . .
401
Immune Deficiency Syndromes. .

Dietrich V K. Jehle
8.6.1
381
Dietrich
K. Jehle
Transplant-RelatedProblems..... 414
8.7.1
Transplant Rejection. . .
414
Susan P. Graham
8.8 Hypersensitivity.....
8.8.1 Anaphylactic/Anaphylactoid
Reactions
384
8.8.2
8.8.3
8.8.4
387
.... 387
IV
and
Migden
. 388
BloodTranstusion..
Anthony J. Billittier
Robert F Reardon,
Douglas R.
White Blood Cell Disorders . . . . .
MarcBorenstein
Leukemoid Reaction. . .
Thomas Nowicki and
Marc Borenstein
7.6.3 Leukopenia.
Michael
Jehovah's Witnesses and
388
Thomas Nowicki and
6.4.3 PosteriorPole......... 354

James M. Leaming
6.4.4 Orbit. .
357
James M. Leaming
6.5 Cavernous Sinus
Thrombosis
357
Peter W MaxweU
7
Leukemia
417
417
Richard S. Krause
Angioedema and
Urticaria
420
Richard S. Krause
Allergic
Rhinitis
421
Richard S. Krause
Drug and Food
Allergies
421
Richard S. Krause
8.8.5 SerumSickness........
422
Richard S. Krause
SYSTEMIC INFECTIOUS DISORDERS 423

Chapter Editors: RichardVAghababian
and GregoryA.Volturo
9.0 Systemic Infectious Disorders . . .
Gregory A. Volnro
423
.
9.1 Bacterial.
424
9.1.1 Botulism.
424
ThomasGermano
9.1.2 GonococcalDisease.... 426
Thomas Germano
9.1.3 Sepsis .
430
9.1.4
9.1.5
Thomas Germano
Mycobacterial Diseases.
Thomas
Germano
Meningococcemia. . . . .
. 434
. 442
Gregory A. Volturo
9.1.6 Plague.
444
Gregory A. Volturo
447
9.1.7 Tetanus
Ajeet J. Singh
9.1.8 Toxic Shock Syndrome. . 448
Ajeet J. Singh
9.1.9 Spirochetes
450
9.1.9.1 Lyme Disease
450
Thomas Germano
Leptospirosis.
453
Thomas Germano
454
9.1.9.2 Syphilis.
Thomas Germano
459
9.1.10 Chlamydia.
Valerie Schevon Nicoletti
9.3 Protozoan-Parasites . .
461
461
9.3.1 Malaria
9.5.8 Roseola.
Laura Peterson
American
Trypanosomiasis
(Chagas' Disease). . . . .
Laura Peterson
African Trypanosomiasis
(Sleeping Sickness). . . .
Laura Peterson
9.5.9 Varicella-2oster........
9.5.10
A. Volturo
Gregory
A. Volnro
10
9.5.2
9.5.3
495

l0.l Bony Abnormalities . .
Rania Habal
495
10.1.1
Aseptic Necrosis
the
Rania Habal
Hip
10.1.2
Osteomyelitis
10.1.3
10.1.4
Tumors
498
10.1.5
Rania Habal
Bone
Rania Habal
Cysts
499
Osteoporosis
500
10.1.6
Rania Habal
10.1.7
Osteomalacia
500
10.1.8
Rania Habal
Bone
Rania Habal
501
Spurs
Paget'sDisease..
.... ..
10.2 JointAbnormalities..
501
10.2.1
469
Carl M. Ferraro
Disorders of the
James D. Kocjancic
10.3
501
Artlritis
467
501
Spine
10.3.1
503
504
Spondylosis/Spondylolysis/
Spondylolisthesis . . . . .
James D. Kocjancic
505
DiskDisorders
...... ..
505
Ankylosing Spondylitis.
James D. Kocjancic
470
10.3.2
470
10.3.3
472
James D. Kocjancic
Low Back Syndromes. . .
507
James D. Kocjancic
10.3.5
474
Spinal Stenosis..
.... ..
509
James D. Kocjancic
10.4
Infectious
Mononucleosis........
482
Ajeet J. Singh
Influenza.
Ajeet J. Singh
483
Overuse
10.4.2
10.4.3
10.4.4
486
10.4.5
487
Tendonitis
510
Bursitis
510
Lauren Pipas
485
Ajeet J. Singh
509
Lauren Pipas
484
Ajeet J. Singh
Syndromes
Lauren Pipas
10.4.1
Ajeet J. Singh
Laura Peterson
497
Rania Habal
9.5.7 Rubella
496
Rania Habal
Rania Habal
Virus..
9.5.6 Rabies.
495
Osteogenesis
10.3.4
9.5.5 Poliomyelitis
of
Imperfecta.
Humanlmmunodeficiency
9.5.4 Mumps
492
(NONTRAUMATIC).
Viral
9.5.1
489
MUSCULOSKELETAL DISORDERS
10.1.9
9.4.2 Ehrlichiosis.
9.5
LauraPeterson
Herpes Simplex Virus . .
465
9.4 Rickettsial
9.4.1 Rocky Mountain Spotted
Fever..
Gregory
488
Laura Peterson
Laura Peterson
9.3.2 Toxoplasmosis
CoNrENrs
Fibrositis.
Lauren Pipas
Muscle Strains
Lauren Pipas
Carpal Tunnel
5l I
5ll
Syndrome
5l I
Lauren Pipas
/ x\ii
xiv /
CoNrnNrs
10.5
Muscle
Lauren
Abnormalities
Pipas
10.5.I
Muscular Dystrophies . .
Lauren
Pipas
512
11.5.2
. 512
10.5.2 Rhabdomyolysis.......
Pipas
10.5.3 Myositis
Lauren Pipas
512
L6
ll.7
Lauren
10.5.4
10.6
11
Myositis Ossificans. . . . .
Pipas
Infections.
l1.l
Peripheral
L. Anthony
Neuropathies
544
546
Cirillo
AcuteSpinalCordCompression...
513
11.8 Hydrocephalus.....
513
Eustacia Su
11.8.3 CNS Shunt
549
550
Malfunction
514
10.6.1 Necrotizing Fasciitis . . . . 514
Christopher J. Marhts
10.6.2 Gangrene
515
Christopher J. Marlan
10.6.3 Paronychia
516
Christopher J. Markus
10.6.4 Felon. .
516
Christopher J. Markus
10.6.5 FlexorTenosyovitis..... 516
ChristopherJ. Markus
552
Eustacia Su
11.9 Seizures
556
Robert C. Reiser
ll.9.l
Status Epilepticus. . . . .
551.
Robert C. Reiser
FebrileSeizures........
558
Robert C. Reiser
Alcohol-related
Seizures
559
RobertC. Reiser
ll.l0 Headache
519
ll.l2
Disorders
519
l1.l.l.l Cerebral Aneurysm. . . . . 519
Patrick Brunett
Cerebrovascular
Eustacia Su
Lauren
SoftTissue
NERVOUS SYSTEM DISORDERS. . . . .

Chapter Editor: John C. Moorhead
Myasthenia Gravis . . . .
ScottW Jolin
560
Christopher J. DeFlitch,
Gretchen K. Lipke, and
Francis P Renzi
Tumors of the Central Nervous
System.
565
Philip D. Anderson and

Ron Medzon
11.1.1.2 Arteriovenous
ll.l.2
Malformation
Brunett
Patrick
Hemorrhagic Stroke . . .
Patrick
520
. 521
Brunett
ll.l.3 IschemicStroke....... 525
Patrick Brunett
I 1.1.4 Transient Ischemic
Attack .
531
PatrickBrunett
ll.2 CranialNerveDisorders......... 532
L. Anthony Cirillo
11.2.1 Seventh Nerve Palsy/
Bell's Palsy
532
L. Anthony Cirillo
11.2.2 Trigeminal Neuralgia (Tic
Douloureaux)
534
L. Anthony Cirillo
Visual Disturbances
Due to Cranial Nerve
Disorders
534
L. Anthony Cirillo
I1.3 Demyelinating Diseases
536
L. Anthony Cirillo
ll.4.l Brain Abscess
538
Eustacia Su
11.4.1.2 EpiduralAbscess....... 540
Eustacia Su
I1.5 Neuromuscular Disorders.
542
Scott W Jolin
11.5.1 Landry-Guillain-Barr6
Syndrome
542
Scott W Jolin
12
OBSTETRICSAND DISORDERS OF
PREGNANCY
577
12.0
Obstetrics and Disorders
Pregnancy
of
571-
Chris J. Michalakes
12.1 Contraception
578
Chris J. Michalakes
OralContraceptives.....
578
ChrisJ. Michalakes
Subdermal and Injectable
Contraceptives
12.2
Chris J. Michalakes
Intrauterine Devices . . .
Chris J. Michalakes
Pregnancy,
581
Uncomplicated
581
582
Chris J. Michalal<es
12.3
12.3.1
Complicated
Ectopic
12.3.2
Chris J. Michalakes
Hyperemesis
Pregnancy,
Gravidarum.
585
585
582
Chris J. Michalakes
12.3.3
Abortion
587
Chris J. Michalakes
12.3.4 AbruptioPlacentae.....
12.3.5
12.3.6
Chris J. Michalakes
Placenta Previa
Chris J. Michalakes
pregnancy-Induced
Hypertension.
Chris J. Michalakes
589
589
590
CoNrrNrs
Stridor.
12.3.8 HydatidiformMole
(MolarPregnancy) . . . . .
Chris J. Michalakes
Guidelines for Describing
Drugs in Pregnancy. . . .
Chris J. Michalakes
12.4
12.5
Uncomplicated
Labor,
Mark R. Pundt
Labor, Complicated .
12.5.1 Premature Rupture
I2.7
Mark R. Pundt
Preterm Labor. .
Mark R. Pundt
FailuretoProgress . . . . .
Mark R. Pundt
Distress
Fetal
1,2.5.5
Mark R. Pundt
RupturedUterus. . .
Mark R. Pundt
12.7.2
.5
12.7.6
12.7.7
12.'l
.8
593
Howard M. Corneli
l3.l
595
13.1.2
596
13.1.6
Complicated
Dystocia
Mark R. Pundt
Uterine Inversion . . . . .
Mark R. Pundt
Multiple Births .
Mark R. Pundt
3.
.8
597
Sigmund J. Kharasch
Intussusception. . . . . . .
l3.l.l0
13.1.11 Midgut
Hemorrhage
Endometritis
13.2.3
Mastitis.
13.3
PEDIATRIC DISORDERS
Chapter Editors: Gary R. Fleisher,
Mary Christine Bailey, and
Mariann M. Manno
13.0
Pediatric
Disorders
Endocrine/Metabolic .
13.3.1.2 Emergency Management
of Diabetic
Children
694
Mariann M. Manno
13.3.2.1 Congenital Adrenal
599
Mariann M. Manno
Hyperplasia.
13.3.3
Inborn Errors
Metabolism.
603
Debra L. Weiner and

Mark S. Korson
13.4 Hematology/Oncology
13.4.1
698
of
602
SickleCellDisease. .
701
707
707
...
Kristen J. Paddon
Inherited and Acquired
Bleeding Disorders . . . .
Mark G. Roback
605
607
686
694
Ketoacidosis
13.5 Neurologic
13.5.2
Abdominal Pain in
Donna M. Bhisitlai
Apnea
Susan B.
681
Disease
II
E lliley
598
598
Kerryann B. Broderick
13
681
Brian A. Bates
Acquired Heart
James
12.8.4
672
677
13.2.1 Dysrhythmias
598
12.8.3
Volvus
13.2 Cardiac.
12.8.2
Paula J. Schweich and

L. Mason Cobb
598
598
667
Jacalyn S. Maller
597
Emergency Cesarean
Complications
12.8.1 RetainedPlacenta. . . ...
Gastrointestinal
Bleeding
Mark R. Pundt
Postpartum
657
663
596
Mark R. Pundt
12.8
Tucker
Gastroenteritis
13.1.4.4 Pyloric Stenosis

David T. Bachman and
Craig W Lillehei
596
. 597
Section
654
654
Carmen Teresa Garcia
597
Stillbirth
650
.....
Appendicitis
Jffiey
640
643
Jay Fisher
Gastrointestinal
..
Vomiting.
596
Mark R Pundt
12.'7.3 ProlapsedCord........
12.'l
Lisa S. Etzwiler
of
12.5.4
Delivery
592
595
Membranes
12.5.3
Judith K. Lucas
Vaginal Bleeding in
Prepubertal Females . . .
Dehydration
12.5.2
634
592
607
710
715
Meningitis and
Encephalitis
715
Peter L. J. Barnett
615
Tbrrey
618
Fever..
Mariann M. Manno
Intractable Crying in Infancy
622
and Childhood
Robert G. Bolte
630
Limp..
Stephen J. Teach
13.5.3
Seizures
721
Douglas S. Nelson
Neurosurgical
Emergencies
13.5.4
June G. Hanly
Ventricular Shunts. . . . .
June G. Hanly
Neoplasms
June G. Hanly
729
730
733
xv
xvi /
CoNrnNrs
13.8.3 Depression/Suicide.....
Cerebrovascular
Disorders
734
June G. Hanly
PseudotumorCerebri. . .
June G. Hanly
Increased Intracranial
Pressure
13.8.5 BehavioralDisorders....
735
735
13.9 Respiratory.
Infections
736
13.9.6
736
739
Louis M. Bell, Jr
Legg-Calv6-Perthes
Disease/Avascular Necrosis
ofthe Femoral Head . . . . 741
Carol Ledwith
Septic Joint.
741
Louis M. Bell, Jr
Slipped Capital Femoral
Epiphysis
13.9.3
..
..
13.6.7
13.10
l3.l
743
743
Purpura.
744
746
David H. Dorfman
13 .7
.5
Nasopharyngitis (Upper
13.7.6
David H. Dorfman
Otitis Externa
David H. Dorfman
Otitis Medea
David H. Dorfman
13;7.7
Pharyngitis and
13.7.8
David H. Dorfman
PeritonsillarAbscess..
David H. Dorfman
746
7
Tonsillitis
..
13.7.9 Sinusitis..........:..
David H. Dorfman
13.7.12 Tracheitis/Bacterial. . . .
David H. Dorfman
13.8 Psychiatric
Michael J. Fairley and
Ralph M. Hanson
13.8.2 Eating Disorders. . . . . .
Ralph M. Hanson
47
777
777
MralExanthema.......
782
Genitourinary.
786
Catherine E. Pewon
13.13.2 Penile Problems . . . . . . .
Catherine E. Perron
13.13.3 TesticularProblems. . . . .
Catherine E. Perron
13.13.4 Urinary Tract Infections . .
Alison St. Germaine Brent
Sexually Transmitted
787
788
791
747
Diseases
793
748
Alison St. Germaine Brent

Nephritis and Nephrosis .
797
749
749
Richard Malley
13.13
Laryngotracheobronchitis
RespiratoryInfection)...
775
Ochsenschlager
744
David H. Dorfman
13.7.4
774
Daniel Warne
13.12.4
(Croup).
772
Eileen A. Keneck
Henoch-Schiinlein
Eileen A. Keneck
Lyme Disease
Eileen A. Keneck
13.12 Skin and Soft Tissue Infections . .
Richard Malley
13.12.2 Infectious Rashes . . . . .
David H. Dorfman
13.7.3
767
Meumatologic.
Carol Ledwith
13.7.1 Epiglottitis
Sepsis
Arthritis
Carol Ledwith
Osgood-Schlatter
HeadandNecklnfections . . . . . . .
765
Eileen A. Keneck
l3.l l.l Juvenile Rheumatoid
742
Carol Ledwith
Traumatic Pediatric
13.7
761
Pertussis
Bacteremia and
Richard Bachur
l3.ll.3
Orthopedics
Fibrosis
Susan B. Tbrrey
742
Disease
Cystic
Richard M. Ruddy
13.9.7
Carol Ledwith
13.6.6 Tumors
759
Tamara Ingrid Pottker and
742
Toxic Synovitis. . .
Pneumonia
Susan B. Tbrrey
Carol Ledwith
13.6.5
758
Susan B. Torrey
June G. Hanly
13.6 Orthopedic
13.6.3 Osteomyelitis
13.6.4
759
Lower Respiratory Tract
Compression.
13.6.2
755

Ralph M. Hanson
June G. Hanly
Spinal Cord
13.6.1
753

Ralph M. Hanson
Mananda S. Bhende
Pediatric Sedation. . . . . .
Baruch S. Krauss
Pediahic Human
Immunodeficiency Virus
750
(HIV) and Acquired
751
Immunodeficiency
Syndrome (AIDS). . . . .
802
807
Heidi M. Pinkert
752
Newborn Resuscitation
in the Emergency
Department .
Laura S. Fitzmaurice
814
CoNrrNrs
14
14.7.1
PSYCHOBEHAVIORAL
DISORDERS
Disorder(ASPD) . . . . . .
Thought Disorders
Katherine Thomas
817
Pamela Edwards and

Katherine Thomas
14.1.1
818
Disorder
821
Pamela Edwards and

Katherine Thomas
Chapter Editor: John C. Moorhead
l4.l
14.2 MoodDisorders.....
14.8.4
Katherine Thomas
14.2.2
MajorDepressive
Disorder
Bipolar
822
Episode.
. 82'7
Disorders
15
RENAL
15.0
15.1
Disorder
828
Pamela J. Edvards and

Katherine Thomas
14.3.4
14.3.1
Obsessive-Compulsive
Disorder
Pamela J. Edwards and
Katherine Thomas
Posttraumatic Stress
830
Disorder
830

Katherine Thomas
14.3.3
14.6
Addictive
14.7.5
847
847
849
15.3.1 Glomerulonephritis.....
854
15.4,
15.5
Substance Abuse
833
. 834
.
Nephrotic Syndrome.. .
15.6
856
857
857
862
Gerald Patrick Igoe

Acute and Chronic Renal Failure
and Interstitial Tirbular
Necrosis
Acute Renal Failure . . .
Richard S. Krause
Chronic Renal Failure. .
Richard S. Krause
831
Nephritis
Acute Interstitial
Ronald M. Moscati
864
15.7 Tumors.
Nephroblastoma.......
837
866
866
Tanvir M. Dara
866
.. .
867
Renal Cell Carcinoma. .

838
Tanvir M. Dara
Tumors of the Urinary
838
Collecting System.. .
Tanvir M. Dara
Adenocarcinoma
of
Prostate.
BorderlinePersonality
Disorder (BPD).
Pamela Edwards and
Katherine Thomas
847
853
830
831
Disorders
Katherine Thomas
PersonalityDisorders
Pamela Edwards and
Katherine Thomas
Renal Disorders
Robert D. Hong and
Leonard G. Gomella
Structural Disorders
15.1.1 Renal Calculi
Robert D. Hong and
Leonard Gomella
15.1.2, Obstructive Uropathy and
15.1.3 Renal Obstruction.. . .. .
Robert D. Hong and
Leonard Gomella
15.3.2
BulimiaNervosa.......
14.7
847
Angeline D. Brunetto
Glomerular Disorders.
Gerald Patrick Igoe
831
Abuse
14.6.2
DISORDERS
Gerald Patrick Igoe
Behaviors
Drug Abuse.
Katherine Thomas
Opioid
Katherine Thomas
Eating Disorders. . . . . .
Katherine Thomas
Diagnostic Criteria
forAnorexia Nervosa . .
Katherine Thomas
Diagnostic Criteria for
845
849
849
15.3
Katherine Thomas
14.6.1
15.2 Infection.
15.2.1 Pyelonephritis
GeneralizedAnxiety
Disorder and Phobias . .
Katherine Thomas
845
827

Katherine Thomas
Panic
841
826
Katherine Thomas
Criteria for Major
14.3.2
.. . ..
Katherine Thomas
Criteria for SubstanceAbuse . . . .
Katherine Thomas
824
Katherine Thomas
Criteria for Manic
Anxiety
IntoxicationandWithdrawal.
Dependence
Disorder
Depressive Episode. . . .
Katherine Thomas
841
Katherine Thomqs
Criteria for Substance
Katherine Thomas
14.2.1
840
14.'7.2 HistrionicPersonality
Schizophrenia
Katherine Thomas
14.3
AntisocialPersonality
8T7
867
Tanvir M. Dara
839
15.8
Complications of Dialysis
868
xvii
xviii /
CoNrsNrs
16
THORACIC-RESPIRATORY
16.6.6
DISORDERS
Chapter Editor: E. JacksonAllison, Jn
16.1 Acute Upper Airway
873
Obstruction
874
16.7
N. Heramba Prasad and

E. Jackson Allison, Jr.
16.1.1
Environmental/Industrial
Exposure.
Andrew T McAfee and
Rita A. Manfredi
Physical and Chemical
Irritants/Insults. . .. . .
16.7.1 ChemicalAgents.......
Tracheostomy/
Complications
890
16.2 BreastDisorders.....
16.7.2
891
16.7
.3
..
892
16.8
892
16.9
Kathryn H. Brinsfield
16.2.2
Tumor.
Thea L. James and
16.2.3
Infections
893
Wall
L. Kristian Arnold
16.3.2
Mediastinal Masses. . .
PulmonaryEmbolism/
Infarction
Mediastinitis
16.9.2 Fat...
894
James Ducharme and

Robert C. Beveridge
16.9.3
896
898
Empyema
James Ducharme and

Robert C. Beveridge
899
16.10 PulmonaryInfections
Pleurisy.
l6.l0.l
Stacy Sperling
901
..
903
905
16.5
Pneumomediastinum.. .
Steven G. Crespo
16.3.7 Pneumothoraces . .. . .
Stacy Sperling
Hyperventilation Syndrome . . . . .
Robert E McCormack
NoncardiogenicPulmonary
Edema/Adult Respiratory Distress
Syndrome.
16.6
908
Todd C. Rothenhaus
Obstructive/RestrictiveLung
Disease.
16.6.1 Asthma
912
912
Eric S. Nadel and

Rita A. Manfredi
16.6.2
Bronchitis
920
Kathleen A. Raftery and

Rita A. Manfredi
16.6.3
ChronicObstructive
16.6.5
Pulmonary Disease. . . .
Kathleen A. Raftery
InterstitialFibrosis . .. .
Nicole Bruner and
Rita A. Manfredi
949
Garry J. Wlkes
Bacterial
Garry J. Wlkes
16.10.2 Fungal.
Garry J. Wilkes
16.10.3 Mycoplasma (and Other
Atypical Agents)
Garry J. Wilkes
16. 10.4 Lung Abscess
Garry J. lVilkes
16.10.5 Bronchiectasis
Garry J. Wilkes
16.10.6 Opportunistic
Garry J. Wilkes
16.10.7 Septic Emboli
Garry J. llilkes
16.10.8 Tuberculosis
Garry J. llilkes
901
16.3.6
16.4
948
16.9.4 AmnioticFluid........
PleuralEffirsions/
Stacey Sperling
16.3.5
Septic
James Ducharme and

Robert C. Beveridge
Steven G. Crespo
16.3.4
945
Thromboembolism.....
Steven G. Crespo
16.3.3
939
Jimmy B. L. Gutman
893
..
93s
James Ducharme and

Robert C. Beveridge
L. Kristian Arnold
16.3.1 Costochondritis........
Aspiration of Gastric
PulmonaryHypertension
David Langleben and
Kathryn H. BrinsJield
Disorders of Pleura, Mediastinum, and
Chest
934
James Ducharme and

Robert C. Beveridge
16.9.1 Venous
Thea L. James and
16.3
Foreign Bodies
Stephen H. Thomas
Contents
Stephen H. Thomas
..
933
Stephen H. Thomas
Thea L. James and
Fibrocystic Diseases
Thea L. James and
933
Stephen H. Thomas
N. Heramba Prasad
and E. Jackson Allison, Jr.
16.2.1
931
16.10.9 Viral.
950
962
963
963
Garry J. Mlkes
16.11 ThoracicOutletSyndrome.....
923
928
16.12
16.13
Marco L.A. Sivilotti

Pulmonary Tumors
Marco L.A. Sivilotti
Sarcoidosis.
Jacques S. Lee
974
CouuNrs
16.14
Sleep Apnea
Syndromes.
976
17.2.14 Curdiouur.ulu.Drugs . .
/ xix
1069
l7 .2.1 4.2 Centrally Acting
Jacques S. Lee
Antihypertensive
l7 TOXICOLOGIC DISORDERS
........
Chapter Editor: Chrislopher Keyes
17.1.1
Poison Centers:
A Resource Guide
Sandra
981
Vivek Chander
17.2.14.4 Calcium Channel
Blockers
L. Gffin
17.2.15
Alan H. B.
17.2.16
Wu
17.1.3
Toxidromes: An APProach
to the Poisoned Patient . . 992
Maria I. Rudis and
Christopher KeYes
17.1.4.3 Gastric Decontamination. 998
Mahesh Shrestha
17.1.5 Withdrawal Syndromes. . 1005
John E HaYnes
17.2.1 Acetaminophen. . .' . . . . 1010
Caustics
Alcohols
Cocaine.
Douglas M.
17
.2.4
HydrogenSulfide......
17.2.20
and Robert Skoglund

Toxicologic Information
Resources
Equipment
1028
Rodenticides
1107
l'l
.2.21
RoY,
and Robert Skoglund

Heavy Metals
Kimberlie A. Graeme,
Steven C. Curry, and
1113
Michael Shannon
1030
17.2.22 Household Industrial

......
Lithium.
Poisons
1032
1035
1122
Andrds M. Lugo
17.2.23 Steroid and Thyroid
Richard J. Kozak and
Hormones
Martin J. Smill<stein
Michael lil'ainscott
17.2.24
17.2.7.2 Monoamine Oxidase
Inhibitors-Toxicity .. . .
Judith Kassner Lucas

17.2.7.3 CyclicAntidepressants .
1045
Intoxicationby
AntiparkinsonDrugs.
1130
17.2.25 Hypoglycemics/Insulin..
Smeel<s
ll34
and
Leslie R. Wolf
...
1048
17.2.27
Iron...
1139
Kim Sing
17.2.9
17.2.10
Miguel C. Ferndndez
Intoxication and Overdose
with AntiPsYchotic
Agents .
1051
1054
Saundra Gilfillan
l7.2.ll Bronchodilators........
1061
17.2.29 LocalAnesthetics . . . . . .
Douglas M. Hill
17.2.30 Locally Acting Drugs . .
Lewis S. Nelson
17.2.31 Toxic and Irritant Gases
Robert J. Geller
17.2.32 MarineToxins. .. . . .
ll43
. ll45
.
1148
I 153
Collin S. Goto
Brian A. Bates
1063
17.2.33 Methemoglobinemia.... lI57

Robert W Wolford and
Lance K. Freeman
Hill
17.2.13 Carbon Monoxide
Poisoning
Hydrocarbons
Frank C.
Jimmy Quir6z Rodriguez

Antihistamines . . . . . . .
Cannabis.
ll2'7
Lena Williams,
and Christopher Keyes
1041
Robert L. Norton
Douglas M.
RoY,
and Robert Skoglund

Personal Protective
1026
Robert Rodriguez
17.2.12
1097
David B. Chandler Robert
0t4
Christopher Keyes
Anticoagulant
1'7.2.6 Anticonwlsants.
l't..2.8
Hazardous Material Spills 1090

David B. Chandler Robert RoY,
David B. Chandler Robert
Shu Shum
17.2.7.1
1085
Ross E. Jones
Hill
Toxicity of the
Anticholinergic
Agents .
17.2.5
1081
Robert W Derlet
17.2.17 Hydrogen Cyanide,
Cyanide Salts, and
Craig R. Warden
17.2.3 Analgesics/Anesthetics..
1079
Patricia B. Rosen
Jackson Smood
t
1075
Christopher J. DeFlitch
Diagnostic Modalities for the

Toxicologic Patient . .' . . 984
Charles A. McKaY and
17.2.2
1069
lT.2.14.3DigitalisToxicity. . . . . . . 1072
for
Toxicologiclnformation
17.1.2
Agents .
Neil S. Meehan
979
1064
Michael J. Dreschet Marc

Bayer and Charles A. McKaY
17.2.34 MushroomToxicity.
... . ll59
Christopher Keyes and
Harold W Keller
Y,x
CoNrnNrs
17.2.36
17.2.37
Nonsteroidal
Antiinflammatory
Drugs.
La,vis S. Nelson
18.2.4 Angiography.
1166
Steven
18.2.6
Il74
18
1183
18.0
Traumatic Disorders .
Richard V Aghababian
Gregory
A.
Volturo
and
. .
1204
DiCastro
Trauma.
Head
18.4.3
RobertW Derlet and

James E Holmes
Injuries of the Spine . . . .
18.4.3.1 Spinal Fractures . .. . ..
1205
l2l0
1210
Armida Nufiez-Finley and
18.4.4
TRAUMATIC DISORDERS
1191
ChapterEditors: RichardVAghababian,
Gregory A. Volturo, and
John C. Moorhead
Ultrasonography . . .
18.4.1
ll77
James H.
1204
A. DiCastro
Steven A.
Jackson
Bryan
17.2.41 Stimulants.
Craig R. Warden
1204
StevenA. DiCastro
Magnetic Resonance
Imaging
. ll72
17.2.40 Sedative-Hypnotic Drugs
1203
StevenA. DiCastro
18.2.5
Salicylates.
Smood
Computed Tomography
Scan..
1164
Organophophate Poisoning
Oliver L. Hung and

Lewis S. Nelson
17.2.38 Opioids and Opiates . . .
Rama B. Rao and
Lewis S. Nelson
17.2.39
18.2.3
. I2l7
Gretchen K. Lipke
18.4.5
SoftTissueFacialInjuries 1220
18.4.7
Gretchen K. Lipke
Ophthalmologic Trauma
Gretchen K. Lipke
Otologic Trauma
18.4.8,
Penetrating Neck
18.4.6
1192
18.1 PrinciplesofTraumaCare....... 1192

18.1.5 TeamResponse........ ll92
RichardVAghababian and
Gregory A. Volturo
l8.l .2 Triage .
I 193
Richard V Aghababian and
GregoryA.Volturo
18.1.3 Resuscitation and
Stabilization
1194
RichardVAghababian and
Gregory A. Volturo
18.1 .4, Diagnosis and Treatment in
18.1.7, the Emergency
18.1.8 Departrnent.
1196
GregoryA.Volturo
18.1.9 Indications for
Consultation
ll97
Gregory A. Volturo
18.1.10 Disposition from the
Emergency
Department.
1198
Gregory A. Volturo
18.1.11 Injury Prevention and
Control
1198
RichardV Aghababian and
Gregory A. Volturo
18.2 Radiologic Evaluation of the Trauma
Patient.
1200
Steven A. DiCastro
18.2.1 Plain Radiography l20l
StevenA.DiCastro
18.2.2 Contrast Radiography . . . 1202
Steven A. DiCastro
Richard S. Hartoch
Facial Fractures. . . . . . .
18.4.8.2
. l22l
1223
Trauma
1224
ErinM. Burnhamand
RichardJ. Mullins
18.4.9
Laryngotracheal
Injuries
1228
Gretchen K. Lipke
18.4.10
ChestTrauma
Lilly
1229
C. Lee and
RichardV Aghababian
18.4.11 AbdominalTrauma... . . 1238

Maryanne lI1 Lindsay
Specific Abdominal
Injuries
1244
MaryanneW Lindsay
18.4.12, Upper and Lower
1250
18.4.15 PelvicFractures........
1261
18.4.13 ExtremityTrauma.....
Gregory R. Ciottone
Steyen A.
18.4.
16
18.4.17
DiCastro
Genitourinary Trauma . .
George Kondylis and
Kevin M. Klauer
Cutaneous Wounds and
Injuries
18.5
1265
1267
Neil S. Meehan
Trauma in Pregnancy
1277
James E Holmes and
EdwardA. Panacek
18.5.2.1 Anatomic, Physiologic, and
Laboratory changes with
Pregnancy.
James
lZjT
F Holmes and
Edward A. Panacek
AbdominalTrauma.....
James E Holmes and
Edward A. Panacek
1278
CoNrENrs
Burns
1279
20.1.2 Research.
and
Edward A. Panacek
James E Holmes
N. Clay Mann
Automation and
Electricallnjuries...... 1280
Informatics
James E Holmes
Todd B. Taylor
and
Edward A. Panacek
l3l7
Continuous Quality
MaternalAbuse........ 1280
and
EdwardA. Panacek
1313
Improvement.
James E Holmes
1324
Christopher M. B. Fernandes
andA. Roy Magnusson
Evaluation and History . .

James E Holmes and
Edward A. Panacek
Fetomaternal Hemorrhage
James E Holmes and
1280
20.6
1282
Hospital Administration
1327
Michael Rapp
20.6.1 Departmental Interaction 1328
Michael Rapp
20.6.2 Governance.
Panacek
Emergency Department
Perimortem Cesarean
1283
Section
James E Holmes and
EdwardA. Panacek
1328
Michael Rapp
EdwardA.
20.6.3 Structure
1329
Michael Rapp
Managed Care . .
1329
Robert W Derlet, Gary P Young, and
John R. Richards
Discharge Planning/
1334
Case Management . . .
19 UROGEI\-ITAL/GYNECOLOGIC
1285
DISORDERS
1285
l9.l Genital Tract/Female
19.1.1 OvarianDisorders...... 1285
19.1.2 VaginaandVulva...... 1287
Kathryn H. Brins/ield
1287
19.1.3 Uterus.
Kathryn H. Brins/ield
1290
19.1.4 Cervix.
20.9
Kristi Vaughn
Medical Staff.
Michael Rapp
1335
20.9.1 Committees.
1336
MichaelRapp
20.9.2 Credentialing
20.9.3
1336
MichaelRapp
Disciplinary Policy . . . . . 1337
Michael Rapp
20.9.4 Structure.
1338
Michael Rapp
. 1290
Brinslield
1295
19.2 Genital TracVMale
1295
19.2.1 Congenital
Michael DiBella
1297
19.2.2 Stn:ctural
Michael DiBella
19.2.3 Inflammation/Infection. . 1299
Michael DiBella
1301
19.3 Sexual Assault
Mary K. Bennett and
G. Richard Braen
1306
19.4.2 Genital Lesions
Dietrich T K. Jehle
1309
19.4.1 Chancroid
DietrichV K. Jehle
19.4.2 Granuloma Inguinale. . . . 1309
Dietrich V K. Jehle
19.4.3 CondylomaAcuminalata 1309
Dietrich V K. Jehle
19.1.5
Infectious Disorders . . .
Kathryn H.
20
ADMINISTRATMASPECTS
OF
EMERGENCY MEDICINE
Chapter Editor: John C. Moorhead.
20.1
Academic Emergency Medicine. .

20.1.1 Faculty Development. . .
Nicholas J. Jouriles
1311
. l3l I
. l31l
2l
EMERGENCY MEDICAL
SERVICES/DISASTER MEDICINE. . .
Chapter Editors: E. JacksonAllison and
Richard V Aghababian
21.0 Emergency Medical Services
/Disaster
Medicine
Kenneth A.
L34l
1342
llilliams, Marc C. Restuccia,
E. JacksonAllison, Jr, JuanA. March,
2l.l
and Richard C. Hunt

1342
EMS System
1342
System Components . . .
Kenneth A. Wlliams,
Marc C. Restuccia, E. Jackson
Allison, Jr, Juan A. March,
and Richard C. Hunt
21.1.2 Categorization and
Organization
2l.l.l
Designation of Levels
of
Service
1344
KennethA. Ililliams,
and Richard C. Hunt
21.1.3
Specialized Care Centers 1344

Kenneth A. llilliams,
Marc C. Restuccia, E. Jacl<son
and Richard C. Hunt
/ xxi
xxii /
CoNrrNrs
21.2
Operations.
EMS System
1345
Kenneth A. Ililliams, Marc C. Restuccia,
E. Jackson Allison, Jr., Juan A. March,
21.4.3
and Richard C. Hunt

21.2.1 Communications
Crush Syndrome/Injury.
Taryn E. Kennedy
1345
A. ll/illiams,
Patient Care Protocols. .

Kenneth A. Mlliams,
TransportVehicles. . . . .
Kenneth
21.2.4
1347
A. llilliams,
AwarenessTraining.....
Training
EMS Continuing
21.3.4

John E. Gough
Injury Prevention and
Education
Management
After
Disaster
Scott R.
Disasterlnformation
21.4.5
John K. Gaflney and

Paul B. Roth
DisasterEducation . . . .
David E. Hogan and
Lester Kallus
Services
l38l
1385
John K. Gaffiey and

Paul B. Roth
21.4.7
InternationalRelief
Assistance.
13
87
Frederick M. Burkle, Jr.
21.5 Research
1393
Assessment of New Methods
andProcedures........
1395
Lucille Gans and
1353
21.5.2
Richard C. Hunt
Gsting of New Equipment
and Technologic
Advances
t354

John E. Gough
1396
Lucille Gans and

Richard C. Hunt
1354
21.4.1.1 Rapid Assessment of Emergency
.....
l37B
DisasterMedicalAssistance
21.5.1
HealthCareNeeds
l3i4
Lillibridge
21.4.4
AssessmentofEnvironmental,
Biologic, and Toxicologic
Medicine
l3j0
Kevin S. yeskey
21.4.3.5 Public Health Issues
Teams(DMAIs).......
1353
Children).
1368
21.4.3.4 Medical Supply/Equipment
21.4.6

John E. Gough
EMS-C (EMS
Disaster
Injuries.
Lucille Gans and

Richard C. Hunt
for
1366
Taryn E. Kennedy

John E. Gough
Hazards.
21.4
Blast
1353
Safety.
1360
Casualties
1350

John E. Gough
21.3.3
.......
Taryn E. Kennedy
Radiation Exposure
1349

John E. Gough
21.3.5
1358
Phuli L. Cohan
21.3 EMSEducation.....
1349
21.3.1 CardiopulmonaryResuscitation,
First Ai( and EMS
EMT
PulmonaryCasualties... 1362
1347
Marc C. Restuccia, E. Jaclcson

Allison, Jr, JuanA. March,
and Richard C. Hunt
21.3.2
1357
Lucille Gans
Pediakic Casualties... . . 1364
A. Williams,
Marc C. Restuccia, E. Jaclcson

Allison, Jr., JuanA. March,
and Richard C. Hunt
Medical Control
1348
Kenneth
Taryn E. Kennedy
MassBurnCare
Lucille Gans

Allison, Jr, JuanA. March,
and Richard C. Hunt
21.2.3
135'7
CompartmentSyndrome 1359

and Richard C. Hunt
21.2.2
Lucille Gans
System
Kenneth
Disaster Medical Care. .

Shock and Its Treatment
inFieldSituations. ... .
21.5.3 DataCollectior/Analysis
1396
Lucille Gans and
1354
Richard C. Hunt
Lucille Gans
21.4.2.8 Critical Incident Stress
Debriefing(CISD) .....
Taryn E. Kennedy
1355
Appendix:TheCoreCurriculum
Subjectlndex.
......
.....
1399
l43l
Contributors
Richard
V.
David T. Bachman, M.D.
Aghababian, M.D.
Professor and Chairman

Department of Emergency Medicine
(Jniversity of Massachusetts Medical Center
55 Lake Avenue North
Worcester, Massachusetts
65
E. JacksonAllison, Jr., M.D., M.P.H.

Clinicql Professor
Executive Vice President and Chief Medical
Officer
Emergency Res ource Management, Inc.
University of Pittsburgh Medical Center
230 McKee Place, Suite 400
Pittsburgh, Pennsylvania I
I 3-490
Roy L.Alson, M.D., Ph.D.

Assistant Professor
Bowman Gray School of Medicine
Medical Center Boulevard
Winston-Salem, North Carolina 27 I 5 7- I 089
Clinicql Instructor
University of Massachusetts Medical Center
0
Richard Bachur, M.D.

Division of Emergency Medicine
Childrenb Hospital
300 Longwood Avenue
Boston, Massachusetts 021
I5
Mary Christine Bailey, M.D.

tructor of Pediatrics
Harvard Medical School;
Childrenb Hospital
Ins
300 LongwoodAvenue
Boston, Massachusetts 02 I I 8
Jerry R. Balentine, D.O.

Associate Director and Assistant Professor
Philip D. Anderson, M.D.
55 LskeAvenueNorth
Associate Professor of Pediatrics

University of Vermont College of Medicine;
Maine Medical Center
36 Oalonont Drive
Falmouth, Maine 04105
65
Michael W. Ardagh, M.B.Ch.B.' D.Ch.

Senior Lecturer in Emergency Medicine
Christchurch School of Medicine
Private Bag 4710
Christchurch, New Zealand
L. Kristian Arnold, M.D.

As si s t ant C linic al P rofe s s or
Boston University School of Medicine
Boston Medical Center
818 Harrison Avenue
Boston, Massachusetts 02 1 l 8
St.
Barnabas Hospital
4422 Third Avenue

Bronx, NewYork 10457
Peter L. J. Barnett, M.B.B.S., M.Sc.

Deputy Director
Royal Children b Hospital
Flemington Road
Parkville, Victoria 3 0 5 2, Australia
Jeanne M. Basior, M.D.
Clinical As sistant Profes s or
School of Medicine and Biomedical Sciences
State University of New York at Buffalo
Buffal o G eneral Hospital
100 High Street
Buffalo, NewYork 14203
xxiii
v,xiv / CoNrnrsuroRs
Brian A. Bates, M.D.
Director Children's Emergency Center
Donna M. Bhisitkul, M.D.
Methodist Womenb and Childrenb Hospital

8 I 09 Fredericksburg Road
San Antonio, Texas 7 82 29
Marc Bayer, M.D.

University of Connec ticut
nt C linic al P rofe s s o r
State University of New York at Buffalo School
Medicine
Erie County Medical Center
462 Grider Street
Bufalo, NewYork 14215
As s is ta
60 3 0
Michael S. Beeson, M.D., M.B.A.

Assistqnt Professor
Northeastern Ohio Universities College
Medicine
Eastern Virginia Medical School /

Children's Hospital of the King's Daughters
601 Childrenb Lane
Norfolk, Virginia 2 3 5 07
Anthony J. Billittier IV M.D.
John Dempsey Hospital, MC-5380

263 Farmington Avenue
Farmington, Connecticut
Assistant Professor
D epartment of Ped ia tr i c s
of
4209 State Route 44
Rootstown, Ohio 44272
Louis M. Bell, Jr., M.D.

Associate Professor
Department of Pediatrics
Univ er s ity of Pennsy lv ani a ;
The Childrenb Hospital of Philadelphia
34th Street and Civic Center Boulevard
Philadelphia, Pennsylvania I 9 I 04
Mary K. Bennett, M.D.

Clinical Instructor
State University of New York at Buffilo
100 High Street
Buffalo, New York 14203
of
Robert G. Bolte, M.D.

Professor
Departments of Pediatrics and Pediatric
Emergency Medicine
University of Utah
100 North Medical Drive
Sak Lake City, Utah 84113
Marc Borenstein, M.D.
Program Director of Residency in Emergency
Medicine
Vice Chair of Department of Emergency Medicine
St. Luke's-Roos evelt Medical Center
1000 Tenth Avenue
New York, Na,v York I 00 I 9
G. Richard Braen, M.D.

Professor and Chairman
uffal o G enera I H o spi t a I
100 High Street
B
Robert C. Beveridge, M.D., M.Sc.

Associate Professor of Emergency Medicine
Assistant Professor of Medicine
Dalhousie University
Tucker Park Road
Saint John, New Brunswick E2L 4L2, Canada
Mananda S. Bhende, M.D.

Associate Professor
Departments of Pediatrics and Pediatric
Emergency Medicine
University of Pittsburgh /
Childrenb Hospital of Pittsburgh
3705 Fifth Avenue
Pittsburgh, Pennsylvania I 52 I 3
Alison St. Germaine Brent, M.D.

C linic al As sistant P rofes s or
Department of Pediatric s
University of South Florida College of Medicine;
All Childrenb Hospital
801 Sixth Street South
St.
Petersburg, Florida 3 37 3 I -892 0
Kathryn H. Brinsfield, M.D.

Assistant Professor
818 Harrison Avenue
.
Kerryann B. Broderick, M.D.
Assistant Professor
State University of NewYork at Buffalo
462 Grider Street
Bufalo, New York I42l5
Charles K. Brown, M.D.

Associate Professor
East Carolina University School of Medicine
Pitt County Memorial Hospital
2100 Stantonsburg Road
P.O. Box 6028
Greenville, North Carolina
2 78 3
5-6028
CoNTRTBUToRS
Frederick M. Burkle, Jr., M.D., M.P.H.

Professor ofPediatric Surgery and Public Health
Chair of Division of Emergency Medicine
Director of Center of Excellence in Disaster
Management and Humanitarian Assistance
University of Hawaii, John A. Burns School of
Medicine
I319 Punahou Street
Honolulu, Hawaii 96826
Erin M. Burnham, M.D.

Resident

Oregon Health Sciences University
3I8I Southwest Sam Jacluon Pqrk Road
Portland, Oregon 97 20 I -3098
Robert L. Brown, M.D.

Brody 4W-54
Greenville, North Cqrolina 27858
David B. Chandler, Ph.D.

Director of the Hazardous Materials Program
Nicole Bruner, M.D.
Oregon Poison Center;

3 l 8 I Southwest Sam Jackson Park Road
Portland, Oregon 9 7 2 0 I - 3 09 8
2708 Royster Court

Virginia Beach, Virginia 23454
Vivek Chander, M.D.

Patrick Brunett, M.D.

Assistant Professor
3181 Southwest Sam Jacl<son Park Road, UHN-52
Portland, Oregon 97 201 ;
St aff Em ergency P hy s ic i an
Portland
Veterans
Affairs Medical Center
55 LakeAvenueNorth
Worcester, Mas sachusetts
65
VijaiV. Chauhan, M.D.

Chief Resident Department of Emergency
Medicine
New York Medical College
Metropolitan Hospital Center
NewYork, NewYork 10021
Angeline D. Brunetto, M.D.
Gregory R. Ciottone, M.D.
Emergency Physician (Resident)

State (Iniversity of New York at Buffalo Hospital
Assistant Professor
55 LakeAvenue North
Worces ter, Massachusetts 0 I 6 5 5
Consortium
1599 Country Haven Trail
Mount Juliet, Tbnnessee 37122
James H. Bryan, M.D.

3 I 8 l Southwest Sam Jackson Park Road
Portland, Oregon 97 20 I - 3 098
L. Anthony Cirillo, M.D.

Clinical Assistant Professor of Surgery
Memorial Hospital of Rhode Island

Brown University Medical School
Ill
Bra,vsterStreet
Pawtucket, Rhode Island 02860
/ xxv
xxvi /
CoNrnrsuroRs
L. Mason Cobb, M.D.

Attending Surgeon
Department of Surgery
Mary Bridge Children's Hospital
314 South Martin Luther King Jr Way, #306
Thcoma, I4/as hington 9 8 4 0 3
Steven C. Curry, M.D.

Department of Medical Tbxicologlt
Good Samaritan Regional Medical Center
925 East McDowell Road, 2nd Floor
Phoenix, Arizona 85006
Tanvir M. Dara, M.D.

Clinical Instructor
Phuli L. Cohan, M.D.

Laurence General Hospital
General Street
Lawrence, Massachusetts
I 84 2
Howard M. Corneli, M.D.

Associate Profes sor of Pediatrics
Departments of Pediatrics and Emergency
Medicine
Primary Children's Medical Center

Salt Lake City, Utah 841I3
Francis L. Counselman, M.D.

Associate Professor and Chairman
Eastern Virginia Medical School

Raleigh Building, Room 304
600 Gresham Drive
Norfolk, Virginia 2 3 5 07 - I 99 9

State Uniyersity of NewYork at Buffalo
Buffalo General Hospital

100 High Street
Christopher J. DeFlitch, M.D.

Westmoreland Regional Hospital
532 West Pittsburgh Street
Greensburg, Pennsylvania I 5 60 I - 2 28 2
Robert W. Derlet, M.D.
Professor
University of Califtrnia, Davis
2315 Stockton Boulevard, PSSB Suite 2100
Sacramento, Califurnia
958
AmyA. DeStefano, M.D.

Wake Medical Center
3000 New Bern Avenue
Richard A. Craven, M.D.

Eastern Virginia School of Medicine
600 Gresham Drive
Norfolk, Virginia 2 3 5 07 - I 9 99
Raleigh, North Carolina 27610
Michael DiBella, M.D.

Clinical As sis tant Profes s or
State University of NewYork
100 High Street

Steven G. Crespo, M.D.
Assistant Professor
One Boston Medical Center Place
Mark D. Crockett, M.D.

Department of Emergency Medicine M/C724
University of lllinois at Chicago
Room 618, College of Medicine West
l8l9
West Polk Street

Chicago, Illinois 606 I 2-7 3 5 4
Steven A. DiCastro, M.D.

Clinical Instructor of Emergency Medicine
55 LakeAvenueNorth
Worcester, Massachus etts 0 I 6 5 5
David H. Dorfman, M.D.

Assistant Professor
D ep artment of Pe diatr i c s
818 Hanison Avenue, Dowling 3 South
Boston, Massachusetts 02 1 1 8
CoNrRrBUroRs
.
Michael J. Drescher, M.D.
Assistant Professor
Departments of Traumatology and Emergency
Medicine
University of Connecticut School of Medicine
Hartford Hospital
Syracuse, New York I 32 I 0
80 Seymour Street
PO. Box 5037
Hartfurd, Connecticut
Wayne J. Farnsworth, M.D.

Assistant Professor of Emergency Medicine
State University of NewYork at Syracuse
Health Scienc e Center- Syracus e
750 EastAdams Street
06 I 0 2 - 5 0 3 7
James Ducharme, M.D., C.M.

Associate Professor
Dalhousie University;
Atlantic Health Sciences Corporation
P.O. Box 5200
Saint John, New Brunswick E2L 4L4, Cqnada
Pamela J. Edwards, M.D.
Assistant Professor of Psychiatry
Director of Adult Psychiatry Clinic

Southwest Sam Jacl<son Drive
Portland, Oregon 97201
3l8l
Rob J. Edwards, M.B.B.S.

Consultant in Emergency Medicine
Westmead Hospital
Hannesbury Road
Tomer Feldman, M.D.

State University of New York at Syracuse
H e alth S cienc e C enter- Syracus e
750 East Adams Street
Syracuse, New York I 3 2 I 0
Robert
P.
Ferm, M.D.
Assistant Professor
Deparhnent of Emergency Medicine
University of Massachusetts Medical School
55 LakeAvenue North
Worcester, Mass achusetts
6 5 5 -02 2
Christopher M. B. Fernandes, M.D.

Clinical Associate Professor of Surgery
University of British Columbia;
St. Pqulb Hospital
1081 Burrard Street
Vancouveri British Columbia l/62Jy6, Canada
Westmead, New South Wales 2145, Australia
Wesley P. Eilbert, M.D.

Cl inic al As s i s tant Profe s s or
University of lllinois, Chicago
1740 West Thylor
Miguel C. Fernfndez, M.D.

South Tbxqs Poison Center
University Hospital
7703 Floyd Curl Drive
Sqn Antonio, Texas 7 8 2 84-7 8 3 4
Chicago, Illinois 606 I 2
Lisa S. Etzwiler, M.D.

Assistqnt Clinical Professor of Pediatrics
St. Louis University School of Medicine;
Attending Plrysician
Cardinal Glennon Childrenb Hospital;
Director of Pediatric Emergency Services
St. John's Mercy Medical Center
615 South New Ballas Road, Suite 6006-8
St. Louis, Missouri 63141
Michael J. Fairley, M.B., B.S.

Departmenl of P sychologicql Medicine
The New Childrenb Hospital
PO. Box 3515
Parramatta, New South Wales 2124, Australia
Carl M. Ferraro, M.D.

C linic al As s o ciate Profes s or
Mercy Hospital qnd Medical Center
University of lllinois
2 5 2 5 South Michigan Avenue
Chicago, Illinois 606 I 6
Jay Fisher, M.D.

Clinical Assistant Professor of Pediatrics
University of Nevada School of Medicine;
Pediatric Emergency Servic es
University Medical Center
Las Vegas, Nevada 89102
/ xxvii
xxviii /
CoNrnreuroRs
Laura
S. Fitzmaurice,
M.D.
Associate Professor
D epartment of Pediatrics
Section of Emergency Medicine
University of Missouri, Kansas City;
Children b Mercy Hospital

2401 Gillham Road
Kansas City, Missouri 64108
Lucille Gans, M.D.

Clinical Instructor
55 LakeAvenue North
Worcester, Massachusetts 0 I 65 5-02 28
Carmen Teresa Garcia, M.D.

Attending Physician
D ep artment of Pe di atric s
Maricopa Medical Center
Gary R. Fleisher, M.D.

s s or of Pe di atri c s
Profe
Chief of Emergency Medicine

Children's Hospital
300 LongwoodAvenue
Lance K. Freeman, M.D.

Assistant Professor
St. Lukeb Hospital / Michigan Stqte University
700 Cooper
Saginaw, Michigan 48 60 2
Gordian W. O. Fulde, M.B.B.S.

Director of Emergency Medicine
St. Vincentb Hospital Sydney;
Senior Lecturer
University of New South Wales
Victoria Street
Darlinghurst, New South Wales, 2010, Australia
Richard J. Gabor, M.D.

Assistant Clinical Professor of Emergency
Medicine
Tufts University;
Director of Pediatric Emergency Department
Baystate Medical Center
759 Chestnut Street
Springfield, Massachusetts
2601 East Roosevelt Street

Phoenix, Arizona 8 5 008-4 9 7 3
Robert J. Geller, M.D.

Emory University School of Medicine;
Medical Director
Georgia Poison Center
80 Butler Street Southeast
Box 26066
Atlanta, Georgia
Clinical Instructor
55 LakeAvenue North
65
Sandra L. Giffin, R.N., M.S.

3l8l Southwest Sam Jacl<son Park Road
Saundra Gilfillan, D.O.

0
I I 99
John K. Gaffney, B.B.A., N.R.E.M.T.-P.

Director
Rural Emergency Medical Service and Disaster
Medicine
University of New Mexico
Health Sciences Center
Ambulatory Care Centeri 4 West
Albuquerque, New Mexico 87 I 3 I -5 24 6
Thomas Germano, M.D.
University of Texas Southwest Medical Center

5323 Harry Hines Boulevard
Dallas, Texas
3 03 3 5 - 3 8 0
75 2 3
5-9070
Leonard G. Gomella, M.D.

The Bernard W Godwin, Jr. Associate Professor of
Prostqte Cancer
Director of Urologic Oncology

Kimmel Cancer Center
Thom as Jeffer s o n Un iv ers i ty
l0I5
Walnut Street #I102
.
Peggy E. Goodman, M.D., M.S.
Assistant Professor
Brody Medical Sciences Building

Greenville, North Carolina 2 78 5 8-4 3 5 4
CoNTRIBUToRS
/ xxix
June G. Hanly, M.D.

Assistant Professor
D ep artm ent of Pe diatric s
55 LakeAvenue North
Worcester, Massachusetts 0 I 6 5 5
Ralph M. Hanson, M.B.B.C.H., M.P.H.,
Collin
S. Goto,
M.D.
Assistant Professor
Division of Pediatric Emergency Medicine
University of Tbxas Southwestern Medical Center
at Dallas / Children s Medical Center of Dallas
Dallas, Texas 7 5 23 5-9063
M.R.A.C.M.A.
Sydney University
The Royal Alexandra Hospital for Children
Hawkesbury Road
Westmead, New South Wales 2124, Australia
Richard
S. Hartoch,
M.D.
Assistant Professor
John E. Gough, M.D.

Assistant Professor and Assistant Medical
Director
E ast C aro lina Univ ers i ty
Building A, Physicians' Quadrangle
Greenville, North Carolina 27858
Kimberlie A. Graeme, M.D.

Department of Medical Tbxicology
Good Samaritan Regional Medical Center
925 East McDowell Road, 2nd Floor
Portland Veterans Administration Medical Center;

3 1 8 1 Southwest Sam Jackson Park Road
Portland, Oregon 97 20 I - 3098
John F. Haynes, Jr., M.D.,A.B.M.T.

Clinical Assistant Professor
Departments of Emergency Medicine and Medical
Toxicology
Texas Tbch University
6090 Sanity Drive, Suite 412

El Paso, Texas 79905
David G. Heisig, M.D.

Susan P. Graham, M.D.
Clinical Associate Professor of Medicine
Department of Medicine, Division of Cardiology
State (Jniversity of New York at Buffalo / Buffalo
General Hospital
100 High Steet
Associate Professor
Departments of Medicine and Emergency
Medicine
Health Science Center-Syracuse
David
Jimmy B. L. Gutman, M.D.

Physimed Clinic
6363 Transcanada I2I
Saint Laurent, Quebec H4T 109 Canada
Rania Habal, M.D.

Assistant Professor
New York Medical College / Metropolitan Hospital
l90I FirstAvenue
P.
Hightower, M.D.

Shawnee Mission Medical Center
9100 West 74th Street
Shawnee Mission, Kansas 66204
Douglas M. Hill, D.O.
Professor of Emergency Medicine
University of Health Sciences, Kansas City;
Co-Director of the North Suburban Medical
Center
919l Grant Street
Thornton, Colorado 802 29-4 3 6 I
CoNrmsuroRs
David E. Hogan, D.O.
Dietrich V. K. Jehle, M.D.
Assistant Professor
Wce Chairman and Associate Professor

State University of NewYork at Bulfalo / Erie
County Medical Center
462 Grider S*eet
Oklahoma University Health Sciences Center

University Hospital
800 Northeast Thirteenth Street
Oklahoma City, Oklahoma 73104
James R Holmes, M.D.
Assistant Professor
Department of Internal Medicine
University of California, Davis

I 5 Stockton Boulevard
23
Sacramento, Califtrnia
958
GaryA. Johnson, M.D.

Associate Professor
University Hospital
Robert D. Hong, M.D.
ScottW. Jolin, M.D.
Resident in Urology
Assistant Professor
Department of Urology
Thomas Jefferson University Hospital
Jeferson Medical College
I I I South I lth Street, Suite G6220
Yale University School of Medicine
464 Congress Avenue
New Haven, Connecticut 06519
Oliver L. Hung, M.D.
Ross E. Jones, M.S., M.P.H., Ph.D.
Fellow, Medical Tbxicology

New York City Poison Control Center
Director of Corporate Tbxicology
Bellevue Hospital Center;

New York University Medical Center
462 FirstAvenue
Nau York, New York 10016
Occupational and Preventive

Health Services
Texas Instruments
7839 Churchill Way, Mailstop 3938
Dallas, Texas 75251
Nicholas J. Jouriles, M.D.
Richard C. Hunt, M.D.

Associate Professor and Vice Chair
Moye Boulevard
Greenville, North Carolina 2 7 8 5 8-4 3 5 4
Gerald Patrick Igoe, M.D.

C linical As s is tant Profes s or
462 Grinder Street
Thea L. James, M.D.
Assistant Professor
Case Western Reserve University;
Associate Residency Director
MetroHealth Medical Center

2500 MetroHealth Drive
Cleveland, Ohio 44109
Daniel M. Joyce, M.D.

Assistant Professor
State University of NewYork
at Syracuse
Health Science Center
Syracuse, Nan York I3210
Clinical Instructor
81 8 Harrison Avenue
Lester Kallus, M.D.

State University of New York at Stonybrook
Hospital L4 515
Stonybrook, New York I I 7 94 -7 400
CoNrrusuroRs
Eric M. Kardon, M.D.
Associate Professor of Clinical Emergency
Medicine

Athens Regional Medical Center
Kevin M. Klauer, D.O.

As s i s t qnt Res i dency Director
Alcron General Medical Center
400 N. Wabash Avenue
Alcron, Ohio 44307
1199 PrinceAvenue
Athens, Georgia 30606
James D. Kocjancic, D.O.

Professor
Hamot Medical Center
201 State Street
Erie, Pennsylvania I 6 5 0 l
HaroldW. Kellet Ph.D.

Office of Research and Biotechnology
University of North Texas
3500 Camp Bowie Boulevard
Fort Worth, Texas 7 6 I 07-2699
George Kondylis, M.D.
Clinical Instructor
Eileen A. Keneck, M.D.
Assistant Professor

55 LakeAvenue North
65

I Boston Medical Center Place
Boston, Massachusetts 02 1 I 8
Hervy B. Kornegay, Jr., M.D.

Taryn E. Kennedy, M.B., B.C.H.' B.A.O.

Clinical Instructor
2700 Wayne Memorial Drive

Goldsboro, North Carolina 27534
Department of Emergency Medicine .

55 LakeAvenue North
Worcester Mas s achus etts 0 I 6 5 5 -02 2 8
Wayne
Memorial Hospital
Mark
S. Korson, M.D.
tructor in Pediatrics
Ins

Carroll County General Hospital
Assistant in Medicine (Genetics)

Childrenb Hospital
300 LongvoodAvenue
200 Memorial Drive
Boston, Massachusetts 021 15
Michael K. Kerr, M.D.
Westminster, Maryland
I I 57
Christopher Keyes, M.D.' M.P.H.

C hief, S e c ti on
of
Tbxi
c o I ogy

University of Tbxas Southwest Medical School
Dallas, Texas 7 5 23 5-8579
Richard J. Kozack, M.D.

3181 Southwest Sam Jacl<son Park Road
Portland, Oregon 97 20 I - 3 098
Richard
Arshad Khan, M.D.
Instructor in Medicine
S. Krause, M.D.
al Profes s or
As s i s t ant Clinic

State University of NewYork at Bufalo
Harvard Medical School

330 MountAuburn Street
Cambridge, Massachusetts
100 High S*eet

02
I38
S. Krauss, M.D.,
Ed.M.
Sigmund J. Kharasch, M.D.
Baruch
Assistant Professor
Boston University School of Medicine;
Director of Pediatric Emergency Medicine
Instructor
818 HawisonAvenue
300 Longwood Avenue

D ep artm
nt of
Pe
diatric s
Hqrvard Medical School;

Childrenb Hospital
/ xxxi
xxxii /
CoNrnrsuroRs
David Langleben, M.D.
CraigW. Lillehei, M.D.
Associate Professor of Medicine

D ep artment of C ardi o I o gy
McGill University / Sir M. B. Davis Jewish
General Hospital
3755 Cote Saint Catherine
Montreal, Quebec H3T IE2, Canada
Assistant Professor of Surgery
Associate in Surgery
Childrenb Hospital
James M. Leaming, M.D.
AID-OFDA
Clinical Instructor and Research Fellow

State University of NewYork at Syracuse
Washington, D. C.
Harvard Medical School ;
300 Longwood Avenue

Scott R. Lillibridge

Syracuse, New York I 3204
2 052 3 -
4 43
Maryanne W. Lindsay, M.D.

Medical Center Boulevard
Winston-Salem, North Carolina 2 7 I 5 7- I 089
Gretchen K. Lipke, M.D.
Clinical Instructor of Emergency Medicine
Carol Ledwith, M.D.

Childrenk Hospital
1056 East l9thAvenue, Box 251
Denver, Colorado 80218

55 LakeAvenue North
Worcestef Massachusetts
Jacques S. Lee, M.D., M.Sc.

Ottawa Civic Hospital
1053 CarlingAvenue
Ottawa, Ontario KIY 489, Canada
Lilly C. Lee, B.A.Sc., S.M., M.D.

Chief Resident

55 LakeAvenue North
Worcester, Massachus etts
65
Associate Professor
I Medical Center Boulevard
I4/inston-Salem, North Carolina 2 7 I 5 7 - I 089
William J. Levin, M.D.

Assistant Professor of Emergency Medicine
Department of Otolaryngology / Head and Neck
Surgery
Nau York Eye and Ear Infirmary;
Deparfment of Emergency Medicine
Metropolitan Hospital
l90l FirstAvenue, Room 2Al8
I 608
Judith K. Lucas, M.D.

Department of Pediatric Emergency
Maricopa Medical Center
2601 East Roosevelt
Phoenix, Arizona 8 5 008 - 49 7 3
Andr6s M. Lugo, M.D., C.S.P.I.

School of Pharmacy
Tbxas Tech University
El Paso Campus
University of Houston;
School of Pharmacy
University of Nan Mexico;
Thomason Hospital
West Texas
Ralph B. Leonard, M.D., Ph.D.
Regional Poison Center
4815 Alameda
El Paso, Texas 79905
A. Roy Magnusson, M.S., M.D.

Associate Professor
3I8l Southwest Sam Jaclson Park Road
Jacalyn S. Maller, M.D.

Clinical Assistant Professor of Pediatrics
University of Pennsylvania School of Medicine;
Department of Primary Care Pediatrics
Children b Hospital of Philadelphia
34th Street and Civic Center Boulevard
CoNrrusuroRs
Richard Malley, M.D.
Instructor of Pediatrics
Division of Infectious Diseases
Childrenb Hospital
300 Longwood Avenue
John B. McCabe, M.D.

Professor of Emergency Medicine
Vice President and Vice Dean for Clinical Affairs
Health Science Center-Syracus e
Syracuse, Nau York I 3 2 I 0
Robert F. McCormack, M.D.

Clinic al As
RitaA. Manfredi, M.D.

Assistant Professor
Georgetown University School of Medicine
3700 Resevoir Road
Washington, D.C. 20007
sis tant
Profes
or

School of Medicine and Biomedical Sciences,'
B uffa I o G en era
I H o sp it a I
100 High Street
N. Ctay Mann, Ph.D.
Paul F. McGuire, M.D.
Assistant Professor
3 I 8 I Southwest Sam Jackson Park Road
Portland, Oregon 9 7 2 0 I - 3 098
Resident Physician
Health Science Center-Syracus e
750 EastAdams Street
Mariann M. Manno, M.D.

Assistant Professor
Departnt en t of Pediatrics
55 LakeAvenue North
Worcesteti Massachus etts 0 I 60 5
Assistant Professor
JuanA. March, M.D.
Hartford Hospital
CharlesA. McKay, M.D.

Department of Traumatology and Emergency
Medicine
University of Connecticut School of Medicine
Assistant Professor and Director
80 Seymour Street
Division of Emergency Medical Services

Greenville, North Carolina 27 8 5 8-4 3 5 4
Hartfurd, Connecticut
Christopher J. Markus, D.O.

A s s is t ant C linical Profes s or
Mercy Hospital and Medical Center
2525 South Michigan Avenue
Chicago, Illinois 606 I 6-2 47 7
PeterW. Maxwell, M.D.

Attending Physician
St. Peterb Hospital
Albany, New York 12054
06
02 - 5 03 7

Boston University School of Medicine;
Attending Physician
818 HarrisonAvenue, Dowling
South
Boston, Massachusetts 02 I 18
Neil S. Meehan, D.O.

Clinical Instructor
55 LakeAvenue North
0
65
William J. Meggs, M.D., Ph.D.

Brigham and Women's Hospital
75 Francis Street
Boston, Massachusetts 02 I
Ron Medzon, M.D.

Instructor
Andrew T. McAfee, M.D.

Instructor
/ xxxiii
Associate Professor
University Medical Center of Easlern Carolina
600 Moye Boulevard
xxxiv /
CoNrrusuroRs
M.D.
Professor
John T. Meredith,
Douglas S. Nelson, M.D.
Assistant
Assistant Professor ofPediatrics

Department of Pediatric Emergency Medicine
University of Utah / Primary Children s Medical
Center
Salt Lake City, Utah 84113

Brady Medical Sciences Building 4W54
Greenville, North Carolina 27858-4354
Chris J. Michalakes, D.O.

Assistant Professor of Clinical
Medicine
Emergency

Buffalo General Hospital

100 High Street
Buffalo' New York 14203
constance G. Nichots, M.D.
Douglas R. Migden, D.O., J.D.

Clinical Assistant Professor of Emergency
Medicine

Buffalo, New York I 4 2 0 3 ;
Emergency Physician
Group Hearth cooperative oJ puget sound
seattle, washington 981t I
John c. Moorhead, M.D.,
ProJbssor
M.s.
Department oJ Emergency Medicine

3l8I Southwest Sam Jacl<son Park Road,
Portland, Oregon 97201-3098
UHN52

State University of New York at Buffalo /
Center
462 Grider Street
Erie
County Medical
Richard J. Mullins,
Thomas Nowicki, M.D.

CrockerAvenue
West Hartford, Connecticut 06110
Armida Nufrez-Finley, M.D.
9 7 2 0 I - 3 09
Portland Adventist Medical Center

10123 Southeast Market
L223A
Portland, Oregon 97216-2599
M.D.
Instructor of Medicine
Associate Residency Director
Brigham and Womenb Hospital
75 Francis Street
S. Nadel,
Robert L. Norton, M.D.
Emergency Physician
3181 Southwest Sam Jackson Park Road,
Eric
Schevon Nicoletti' M'D'

Assistant Professor
Department of Emergencl' Medicine
university of Massachusetts Medical Center
worcester' Massachusetts 01655-0228
:?-I-lt
Clinical
Resident in Emergency Medicine
M.D.
Portland, Oregon
Assistant Professor
Worcester, Massachusetts 01655-0228

3l8l southwest sam Jaclcson Park Road
Portland' oregon 97201-3098
Ronald M. Moscati, M.D.

clinicalAssistant ProJbsso,
Professor
Lewis S. Nelson, M.D.

Yale University School of Medicine
464 Congress Avenue
New Haven, Connecticut 06519
Daniel Warne Ochsenschlager, M.D.

Professor of Pediatrics
Emergency Medical Trauma Center
George Washington University School
Medicine;
Childrenb National Medical Center
I I I Michigan Avenue Northwest
of
CoNrRrsuroRS
Kristen J. Paddon, M.D.
Tamara Ingrid Pottker, M.D.
Assistant Professor
Department of Pediatr i cs
Medic al Co I I ege of Wis cons in

Childrenb Hospital Medical Center of Cincinnati
3333 BurnetAvenue
Cincinnati, Ohio 45229
9000 West Wisconsin Avenue, Mail Stop 677

Milwaukee, LTisconsin 5 3 2 2 6
Edward A. Panacek, M.D.

University of Califtrnia at Davis Medical Center
I 5 Stockton Boulevard
23
Sacramento, Califtrnia
958
Michael A. Pellegrino, D.O.

tant Clinic al Ins truc tor
N. Heramba Prasad, M.D.

Associate Professor
Physician's Quadrangle, Building M

Greenville, North Carolina 2 78 5 8-43 5 4

Paul T. Preisz, M.B., B.S.

Lecturer in Emergency Medicine
University of New South Wales;
Department of Medicine
St. Vincentb
Victoria Road Darlinghurst

Sydney, Na'v South Wales 2092, Australia
A s s is
Catherine E. Perron, M.D.

Clinical Instructor in Pediatrics
Departntent of Medicine

Children s Hospital
330 Longwood Avenue

Laura Peterson, M.D.

Assistant Professor
Universiht of Massachusetts Medical Center
55 LakeAvenue North
Heidi M. Pinkert, M.D.

Assistant Profess or of Clinical Pediatrics
St. Luke's-Roosevelt Hospital Center
llll
AmsterdamAvenue
Lauren Pipas, M.D.

Assistant Professor
Syracuse, Neyv York I 3 2 0 I
Hospital
LouiseA. Prince, M.D.

Assistant Professor
750 East Adqms Street
Mark R. Pundt, M.D.

C linical As
istant Profe
ss
or

6167 Bridlewood Drive South
East Amherst, New York I 405
Kathleen A. Raftery, M.D.

Assistan Residency Director

Brigham and Women's Hospital
75 Francis Street
Rama B. Rao, M.D.
George Podgorny, M.D.
Fellow, Medical Tbxicology

Attending Physician, B ellevue Hospital ;
New York University Hospital
462 FirstAvenue
Associate Profes s or of Clinical Surgery

Michael Rapp, M.D.

Arlington Hospital
Forest University
GeorgiaAvenue
Winston-Salem, North Carolina 27 I 04- I 9 I 7

I70l North George Mason Drive
Arlington, Virginia 2 2 2 0 5 - 3 67 I
Wake
2II5
xxxvi /
CoNrnrsuroRs
Robert F. Reardon, M.D.

Clinical Instructor
Jimmy Quirriz Rodriguez, M.D.

100 High Street
Robert C. Reiser, M.D.

Assistant Professor
University of Virginia School of Medicine
University of Virginia Health System
PO. Box 10014
Charlottesville, Virginia 2 2906-00 I 4
Francis P. Renzi, M.D.

Director of Residency Training Program
55 LakeAvenue North
I 65 5
Marc C. Restuccia, M.D.

Assistant Professor
55 LakeAvenue North
WorcesteL Mass achus etts 0 I 6 5 5 -0 2 2 8
Jefatura, Servicio de Urgencias

Hospital Mexico
La Uruca
PO. Box 10105
San Jose, Costa Rica
Patricia B. Rosen, M.D., M.P.H.

Assistant Professor
Tbxas A&M University College of Medicine;
Scott and White Memorial Hospital
2401 South 3lst Street
Temple, Texas 76508
Paul B. Roth, M.D.

Dean
University of New Mexico School of Medicine
BMSB, Room 177
Albuquerque, New Mexico 87131
Todd C. Rothenhaus, M.D.

Clinical Insh'uctor
John R. Richards, M.D.

Emergency Medicine Division
University of California at Davis Medical Center
2315 Stockton Boulevard, Building 2100
Sacramento, Califurnia 9 5 8 I 7

Boston, Massachusetts
Mark G. Roback, M.D.
3M Medical Department;
Associqte Professor
Tbxicology Graduate Program
Univ ersity of Minn es o t a
Minneapolis, Minnesota 5 5 4 5 5
Robert Roy, Ph.D.

Toxicology Services
Assistant Professor of Pediatrics

Section of Pediatric Emergency Medicine
The Childrenb Hospital / University of Colorado
1056 East |9thAvenue, Box 251
Denve4 Colorado 80218
Walter C. Robey III, M.D.

Assistant Professor and Associate Residency
Director
East Carolina University School of Medicine;
Richard M. Ruddy, M.D.

Professor of Clinical Pediatrics
University of Cincinnati College of Medicine
Childrenb Hospital Medical Center
3333 BurnetAvenue, OSB 4
Cincinnati, Ohio 45229
Greenville, North Carolina 2 7 8 5 8-4 3 5 4
Robert Rodriguez, M.D.

University ofTexas Southwest Medical Center
Dallas, Texas
75 2
3 7-8 5 79
Maria I. Rudis, Pharm.D.

North Texas Poison Center
PO. Box 35926
Dallas, Texas 75235
CoNrnrsuroRs
EricW. Schmidt, M.D.
Assistant Professor
55 LakeAvenueNorth
Worcesteti Mas sqchusetts
I 65 5
Shu Shum, M.D.

Northwest Texas Hospital;
B aptist-St. Anthony Hospital ;
Texqs kch University
1400 Wallace Boulevard
Amarillo, Texas 79106

Sandra M. Schneider, M.D.
Professor and Chair
University of Rochester S*ong Memorial Hospital
601 ElmwoodAvenue, Box 49200
Rochester, New York 14627
Kim Sing, M.D.

3I8I Southwest Sam Jacl$on Park Road
Paula J. Schweich, M.D.

Connecticut Children b Medical Center
282 Washington Street
Hartftrd, Connecticut 06 I 0 6
Andrew Singer, M.D.

Douglas Scudder, M.D.
Clinical Instructor
Ajeet J. Singh, M.D.

Clinical Instructor

55 LakeAvenue North

55 LakeAvenue North
Worcesteri Massachus etts
65
Michael B. Seim, M.D.

Emergency Physician Professional Associqtion
Methodist Hospital
7900 Xerxes Avenue South, Suite 740
Bloomington, Minnesota
Gary
S. Setnik,
54 3
M.D.
Sydney Adventist Hospital

185
FoxValley Road
Wahroonga, New South Wales 2076, Ausfralia
Worceste4 Massachusetts
65
Eunice M. Singletary, M.D.

Associate Professor
Eastern Virginia Medical School
600 Gresham Drive
Norfolk, Virginia
2 3 50
Instructor in Medicine
330 Mount Auburn Street
Cambridge, Massachusetts 02 I 38
Michael Shannon, M.D., M.P.H.

Associate Chief of Emergency Services
Director of the Lead & Toxicology Clinic
Children's Hospital;
StaffToxicologist
Massachusetts Poison Control System
300 Longwood Avenue
Boston, Massachusetts 02I I 5
Mahesh Shrestha, M.D.

Adjunct Professor of Medicine and Surgery
E mergency M edi cin e D iv i s ion
University of kxas Southwestern Medical Center
Dallas, Texas 75235
Steven F. Siraco, M.D.
Clinical Instructor
55 LakeAvenue North
Worcesteri Mass achusetts
65
Marco L.A. Sivilotti, M.D., M.Sc.

Assistant Professor
University of Ottawa
Ottawa, Ontario KIY 489, Canada
Robert Skoglund, Ph.D.

Senior Tbxicologist
Pois on C enter ;
Assistant Professor
Toxicology Graduate Program
International
Univ ers ity of Minn
es
ot a
Minneapolis, Minnesota
5545
/ xxxvii
xxxviii /
CoNrrunuroRs
Frank C. Smeeks, M.D., M.S.

Wright State University School of Medicine
3 5 2 5 Southern B oulevard
Kettering, Ohio 45429
Martin J. Smilkstein, M.D.

Associate Professor
3I8I Southwest Sam Jacl<son Park Road
Jackson Smood, M.D.

3I8I Southwest Sam Jaclaon Park Road
Portland, Oregon 97 20 I -3 098
Stacey Sperling, M.D.
Assistant Professor
One Boston Medical Center Place
Boston, Massachusetts 021 l8
RaffiV. Terzian, M.D., M.P.H.

Chief Resident


600 Moye Boulevard
Jerry D. Thomas, M.D.

Assistant Professor
Emory University School of Medicine
68 Armstrong Street Southeast
Atlanta, Georgia 3 03 0 3 - 3 2 I 9
Katherine Thomas, M.D.
artment of P sy chiatry
D ep

3181 Sam Jacl<son Park Road
Portland, Oregon 97 2 0 I -3098
Stephen H. Thomas, M.D.
Instructor
Eustacia Su, M.D.
Assistant Professor of Emergency Medicine and

Mass achusetts General Hospital
Pediatrics
Oregon Health Sciences University Hospital
3l8I Southwest Sam Jacl<son Park Road, UHN 52
Jeffrey R. Suchard, M.D.

Fellow
Department of Medical Tbxicology
Good Sqmaritan Regional Medical Center
925 East McDowell Road, Second Floor
Todd B.Taylor, M.D.
Attending Em ergency P hy s i c i an
Good Samaritan Regional Medical Center and
Phoenix Children b Hospital
Phoenix, Arizona;
Affi liate As s i s t ant P rofe s s or
Division of Clinical Education

Arizona College of Osteopathic Medicine;
1323 East El Parque Drive
Tbmpe, Arizona 8 5 2 8 2-2 649
Stephen J. Teach, M.D., M.P.H.
Assistant Professor of Pediatrics and Emergency
Medicine
George Washington University School of Medicine;
Childrenb National Medical Center
I1 I Michigan Avenue Northwest
32 Fruit Street
Clinics Building, Room I 16

Susan B. Torrey, M.D.

tor of Pe di atric s
Ins truc
Children's Hospital
300 Longwood Avenue
I5
Jeffrey Tucker, M.D.

Children's Hospital at Buffalo
219 Bryant Street
CherylVance, M.D.
University of Caffirnia at Davis
Medical Center
2315 Stochon Boulevard
Sacramento, Califurnia 9 5 8 I 6
Kristi Vaughn, R.N., M.S.N.

Instructor
3181 Southwest Sam Jacl*on Park Road
Portland, Oregon 9 7 2 0 I - 3 098
CoNrnreuroRs
GregoryA.Volturo, M.D.
Leslie R. Woff,
Vf .O.,
Associate Professor and Vice Chair

University of Massachusetts Medical School
As sis tant Profes
55 LakeAvenue North
Worcesten Massachusetts
65
Michael Wainscott, M.D.

Associate Professor
University of Tbxas Southwest Medical Center
Dallas, kxas
75 2
3 5-8 5
l.C.Vf .f.
or/Tbxicology Co ordinator
Wright State University
PO. Box 927
Dayton, Ohio 4540 I -0927
RobertW.Wolford, M.D.
Associate Professor
Program in Emergency Medicine
Michigan State University College of Human
Medicine;
Saginaw Cooperative Hospitals
1000 HoughtonAvenue
Craig R.Warden, M.D.

3I8l Southwest Sam Jacl<son Park Road
Portland, Oregon 97 20 I - 3098
Saginaw, Michigan 48 64 2
Alan H. B.Wu, Ph.D.

Director Clinical Chemistry
Debra L.Weiner, M.D.

Ins truc tor of Pe di atri c s
Children s Hospital
Profes sor, Laboratory Medicine
Department of Pathology and Laboratory Medicine
Hartford Hospital
University of Connecticut Health Center
300 Longwood Avenue

Boston, Massqchusetts 02 I I 5
James F.Witey
/ xxxix
80 Seymour Street
Hartford, Connecticut
06 I
02
II, M.D.
Associate Professor
D ep ar tment of Pe di atric s
The School of Medicine of the University
Connecticut Health Sciences Center
of
Connecticut Children s Medical Center

282 Washington Street
Harford, Connecticut 06 I 06
Garry J. Wilkes, M.B.B.S.

Director of Trauma and Retrieval Services
Kevin S.Yeskey, M.D.

Associate Professor
Department of Military and Emergency Medicine
Unifurmed Services University of the Health
Sciences
4301 Jones Bridge Road

Bethesda, Maryland 208 I 4
Gary P.Young, M.D.
Sir Charles Gairdner Hospital
Verdun Street, Nedlands
Medical Director
Perth, Western Australia 6009, Australia
1255 Hilyard Steet

Eugene, Oregon 97401

Sacred Heart Medical Center
Kenneth A. Williams, M.D.

Assistant Professor
55 LakeAvenue North
W. John Zehner, M.D.

al P rofe s s or
University Hospital
As s i s t ant Clinic
Health Science Center;

Lena Williams, R.N., B.S.C.S.P.I.
North Tbxas Poison Center
P.O. Box 35926
Dallas, Texas 75235
Attending Physician
St. Josephb Hospital Medical Center
301 ProspectAvenue
Syracuse NewYork 13203
Preface
Emergency medicine is a young and vibrant speciality with a rapidly expanding body of knowledge.
The specialty is somewhat unique in that the approach to the patient in acute distress focuses on the
rapid identification of lifethreatening conditions, performance of stabilizing procedures based on a
provisional diagnosis, and then further diagnostic assessment to identify a more definite diagnosis.
Once a diagnosis has been determined, it is often up to the emergency physician to arrange access for
the patient to appropriate resources available within the local health care system. In a busy emergency
department, it is sometimes difficult to gather all available clinical data and use that data to construct a
working diagnosis.
In light ofthese practice characteristics, this textbook has been designed to allow emergency physicians to (l) study for the boards using a focused and pertinent guide; (2) rapidly access specific information about illnesses and injuries in a manner that will facilitate the cases of emergency department
patients; (3) provide background information about disease processes and mechanisms or injury that
will assist in the bedside instruction of patients as well as the classroom instruction of medical personnel; and (4) provide a list of key references for those who wish to investigate a clinical entity in greater
depth than in the textbook.
We are now witnessing rnajor changes in health care delivery across the globe. As a new specialty
with a reputation of providing efficient but sometimes costly medical care, emergency medicine has
been subjected to particularly intense scrutiny. Payers would like to see the nonurgent portion ofthe
over 90 million annual visits to the U.S. emergency departments referred to alternate practice settings
in an effort to reduce costs. Despite this pressure, there continues to be a public demand for care through
emergency departments for those who have no other alternative. In addition, emergency departments
have begun observing patients up to 24 hours to complete diagnostic evaluation treatment protocols that
would otherwise have to be performed as part of a hospital admission. Emergency department observation medicine is one innovation in the management of emergency departments that is addressed in
Chapters 2 and20 of this textbook.
The textbook has been designed to present the body of knowledge that forms the basis of emergency
medicine. To achieve this goal it was decided to follow as closely as possible the outline of the Core
Contentfor Emergency Medicine.The table of contents of this textbook parallels the clinical and administrative sections of the current version (June 1997) of the Core Contentfor Emergency Medicine.The
chapters within the 21 sections of the text cover the vast majority of topics identified inthe Core Content.MosI of the headings match those in the Core Content. To facilitate the reader, we have put the
number of the Core Content section in parentheses next to the corresponding section in this text. The
Core Contenl is also found in the Appendix at the back of the book. Not all items that appear in the
Core Contenl are included in the textbook. This is an attempt to avoid the inclusion of redundant materials and subjects that were thought to require little discussion or elaboration.
xli
Acknowledgments
I acknowledge the dedication of the editors E. JacksonAllison, Jr., G. Richard Braen, Gary R. Fleisher,
John B. McCabe, and John C. Moorhea4 as well as the contribution of the associate editors Mary
Christine Bailey, Christopher Keyes, Mariann M. Manno, and Gregory A. Volturo. The chapter authors
are to be commended for their work. Special thanks to Carol Bloom, Jennifer Cederberg, Alicia Galvan, Pat Keith, and Frank Livesay in the Department of Emergency Medicine at the University of Massachusetts.
Thanks to my family, Ann, Emily, and Andrew, who have put up with me despite the fact that I have
been devoted to this scholarly pursuit.
xliii
CFIAPTER 1
Abdominal and Gastrointestinal Disorders

Gury A. Johnson
Abdominal and Gastrointestinal Disorders (1.0)
Wayne J. Farnsworth
Esophagus (1.1); Rectum and Anus (1.8)
David G. Heisig
Liver (1.2); Large Bowel (1.7)
Louise A. Prince
Gallbladder and Biliary Tract (1.3); Pancreas (1.4)
Wesley P. Eilbert
Stomach (1.5)
Paul F. McGuire
Small Bowel (1.6)
ABDOMINAL AND GASTROINTESTINAL

DISORDERS (1.0) (See 13.0)
matory disease, acute pancreatitis, perforated ulcer, and

urolithiasis. Approximately 15% of patients seen for
abdominal pain will require operation and 27o/o wlll
Abdominal pain is one of the emergency physician's

most challenging chief complaints. It is a common emer-
require hospitalization.
Age of patient greatly impacts on the differential diagnosis and incidence ofsevere disease and illness. One out
of five children seeks medical help for abdominal pain by
age 15. Approximately 5% of these require hospitalization.
Presentations often vary by age. Infants commonly present
with colic, gastroenteritis, and constipation. They also may
have intussusception, malrotation, volvulus, renal neoplasm, necrotizing endocarditis, or incarcerated hemia.
Toddlers frequently have belly pain with pneumonia or
pharyngitis, or other serious diagnoses including Meckel's
diverticulum or appendicitis. Chronic illnesses may present acutely with belly pain. These illnesses include diabetes mellitus, sickle cell anemia, Mediterranean fever,
is often not
definitively diagnosed but can represent a life-threatengency department (ED) presentation that
ing disease that requires immediate intervention.
Epidemiology
After ED evaluation, various authors have found that

nonspecific abdominal pain is the preliminary diagnosis
in4lohto
50o/o
ofall patients.
These patients often have a
benign prognosis. In a prospective study of 230 patients

followed-up for 5 years, only 7o/o required hospitalization
after hospital admission for nonspecific abdominal pain.
Many conditions that present as abdominal pain represent severe disease that requires immediate intervention.
These diagnoses include appendicitis, acute cholecystitis,
intestinal obstruction, intestinal ischemia, pelvic inflam-
Porphyria, and cystic fibrosis. Adolescents may have other

abdominal disorders such as pelvic inflammatory disease,
urinary tract disease, and inflammatory bowel disease.
Both school-age and adolescent children may have a psychosocial cause of abdominal pain.
2 /
EunnctNcn MtuctNn: THn Conn Cunnrculurr,r
Acute abdominal pain in the elderly is frequently a lifethreatening illness. Nonspecific abdominal pain as a discharge diagnosis is relatively unusual (9% to lgo total).
Acute cholecystitis (26
to
4loh), malignant
disease
(l3o/o), incarcerated hernia (5 to l0%), and pancreatitis (4

to 5%o) are common diagnoses. Mortality in this patient
population is significant
to l4oh) and one-third of
patients required operations.
(ll
Pathophysiology
Most abdominal pain is transmitted through visceral

components. Possible neural mechanisms include stretch
fibers, which are in the wall of all organs, as well as the
capsules ofsolid organs. Input from these organs is conducted along afferent nerve fibers that return to the spinal
cord at various levels. Visceral pain is often poorly localized, and, is characteristically vague. Somatic pain often
originates in the peritoneum and is much better localized
than visceral pain. Once the peritoneum is irritated, pain
is well localized and more easily described.
Referred pain is frequently involved with evaluation of
abdominal pain. Common referral patterns include pain
in the chest (e.g., pneumonia), giving an upper quadrant
tenderness, and pain near the diaphragm, giving a sensation in the neck or the back of the shoulder blade (C-3, -4,
and -5 distribution).
Emergency Department Evaluation
Historical Factors
For patients who present with abdominal pain, the history should include location of pain, movement of pain,
time of onset, and duration of pain. Also, aggravating or
palliative factors should be queried. Pain often moves
and in many cases its final destination is more predictive
of the final diagnosis than its original location (e.g.,
appendicitis). Episodic sharp pain that comes in increasing waves is often considered "colicky." Such pain may
increase the suspicion of a hollow viscus as the origin
(e.g., bowel or ureter). Pain that has been present for a
long period of time is less likely to be emergent; however, many patients with acute appendicitis or cholecystitis present more than 12 to 24 hours after the onset of
pain.
Associated symptoms provide important clues to the

diagnosis. The presence of nausea or vomiting may help
indicate the presence of hollow viscus disease. Appendicitis pain classically occurs before vomiting; however,
this rule is violated in many appendicitis patients. Bowel
symptoms including frequency and nature of stools, and
presence or absence of bright red blood or melena must
be queried. Dysuria, urinary frequency, or discoloration

of urine should be noted as should menstrual history in
female patients. Symptoms referable
to
organs
in the
chest or retroperitoneum must also be queried.

A past history that reveals prior abdominal pathology
or prior surgical procedures may provide important clues
as to the final diagnosis. Multiple medical diseases (cardiac orpulmonary disease, atherosclerotic risk factors, or
diabetes) will impact and complicate the presentations of
abdominal pain.
Physical Examination
Abnormal vital signs including tachycardia and elevation of temperature can be important clues to patients
who have truly emergent or surgical presentation of disease. Often the general appearance ofthe patient can be
helpful. Patients with peritonitis will lie very still to avoid
any movement that may irritate their inflamed peritoneum. Patients with colic will frequently be very
mobile and be difficult to examine because of their
unwillingness to lie still.
Infrequently, simple inspection
will reveal a localized
mass or general distention of the abdomen. It is important
to note location of prior surgical
scars. The abdomen
should be auscultated for presence or absence of bowel

sounds. Very active bowel sounds may be a clue that the
patient has increased gastrointestinal motility. The

abdomen should be palpated for the point of maximal
tenderness and total area oftenderness. It is wise to begin
palpation away from the most tender area since the
patient may provide more voluntary guarding after a tender area is elicited. Masses and organomegaly must
specifically be searched for. Hepatic or sphenic enlargement should be noted.
Rebound tendemess may indicate peritonitis. A traditional method of testing for rebound tenderness is to palpate the abdomen somewhat deeply and then rapidly
remove the hand. This withdrawal will move abdominal
contents including the peritoneum and may indicate peri-
tonitis. The test must be interpreted with care since

abrupt movements startle patients and their response to
the test may be difficult to interpret. Simply moving the
stretcher, tapping the patient's heel in order to move the
abdomen, or lightly percussing the abdomen may provide
alternative tests for detecting peritonitis.
Many additional physical tests have been described.
Murphy's sign tests for possible cholecystitis. While palpating the right upper quadrant the patient is asked to take
a deep breath. If the patient suddenly halts inspiration in
response to pain in the right upper quadrant, it is considered a positive Murphy sign. Rovsing's sign is positive
when pain in the right lower quadrant occurs with palpation in the left lower quadrant. This may be indicative of
appendicitis. The iliopsoas sign also looks for possible
appendicitis. A positive iliopsoas sign is pain that occurs
when the patient has right lower quadrant pain when he
AloourNer eNo
GIsTRoTNTESTTNAL
DrsonoBns
tries to flex at the hip against resistance. The obturator

sign also looks for appendicitis. With the right leg flexed
at the hip, the hip is rotated internally. The test is positive
if pain is elicited with this motion. Rectal examination
should be performed for presence or absence of mass,
pain, and quality of stool. Testing for occult blood may be
helpful as well.
Pelvic examination should be performed on all women
with lower quadrant pain. Similarly, examination of male
genitalia is mandatory in all patients with lower quadrant
pain. Palpation ofthe back and percussion over costovertebral angles should always be performed. All patients
should have pulmonary and cardiac examinations as well.
emergent cause of pain. Pyuria may occur with disease

that is not intrinsically renal such as appendicitis. Hematuria may indicate renal disease (e.g., urolithiasis) or a
more systemic insult (e.g., endocarditis).
A pregnancy test should be performed in all female
patients of child-bearing age who have not had a hysterectomy. It is clear that patients who have tubal ligation
are still at risk for pregnancy-related diseases.
Abdominal radiography may be performed to look for
evidence of bowel obstruction, calcifications, foreign
bodies, or free intraperitoneal air. Special radiographic
Laboratory Evaluation
phv.
Laboratory evaluations of patients with abdomen pain

may be very specifically or very broadly undertaken.
Disposition
Certainly some patients may have a clear diagnosis based

on history and examination alone and laboratory analysis
is not required. Other patients may have a life-threatening
presentation or a confusing presentation that requires
multiple lab tests to be performed.
Complete blood count is often ordered to examine the
patient's hematocrit and white blood cell count. Acute
bleeding will have a normal hematocrit, but chronic
blood loss with an insufficient compensatory mechanism
may reveal a low hematocrit. Total white count and white
count differential may imply presence of bacterial or surgical disease. The literature has addressed the place of
leukocye and neutrophil counts in the evaluation of

appendicitis. Patients with appendicitis do tend to have
higher white counts and higher percentage of neutrophilia. However, there is extensive crossover with
other diagnoses including benign diagnoses (e.g., gastroenteritis).
Testing for hepatic enzymes may be very helpful in the
setting of acute hepatitis, biliary tract disease, or pancreatitis. Hepatocellular enzymes (aspartate transaminase,
alanine transaminase) will be elevated with hepatic
inflammation or masses. This will also be more modestly
elevated with biliary tract disease. Alkaline phosphatase
will also be elevated in hepatic disease but more severely
elevated in most cases of biliary tract disease. Bilirubin,
both total and direct, should be measured to screen for
hepatobiliary disease.
Amylase and/or lipase may be measured to look for
pancreatic inflammation. These will also be elevated in
other disease states. Amylase is present in nearly all hollow organs and therefore may be elevated with salivary
gland
or
gastrointestinal disease. Lipase has been
reported to have higher predictive value than serum amylase. However, it may be elevated in a large number of
non-pancreatic disease states also.

Urinalysis should be performed on all patients with
suspicion of urinary tract infection (UTI) or a suspected
tests may be considered in patients after initial evaluation.
These tests would include intravenous pyelogram,

angiography, computed tomography, and ultrasonogra-
Since many patients have a potentially serious disease

that is difficult to diagnose, a low threshold should be
present for obtaining consultation and admitting patients
with acute abdominal pain. Diseases that are notoriously

difficult to diagnose include ischemic bowel disease,
appendicitis, and pancreatic disease.
Treatment
Emergent treatment of these patients include hemodynamic management, antibiotic therapy, and analgesia. A
hemodynamically unstable patient should have large-bore
IV catheters placed and isotonic fluid administered. Control of emesis with antiemetics or nasogastric suction may
aid in maintaining appropriate fluid hydration.
Administration of analgesics has always been controversial in the setting of acute abdominal pain. Despite traditional concerns, it is clear that judicious use of anal-
gesics
in
closely observed patients may be safely
performed.
Patients
with
suspected urinary tract infections or
bowel perforations require antibiotic administration. Adequate gram-negative coverage is required in either case.
bowel perforation is suspected anaerobic and enterococcal coverage should also be provided.
If
SELECTED READING
Analgesia and the acute abdomen. Br Med J 1979;2:1093.
Bower RI, Bell MJ, Temberg JL. Diagnostic value of the white blood count
and neutrophil percentage in the evaluation of abdominal pain in children. Surg Gynecol Obstet 198l;152:424426.
Brewer RI, Golden GT, Hitch DC. Abdominal pain an analysis of 1,000
consecutive cases in a university hospital emergency room. Am J Surg
1976;131:219-223.
Buchert GS. Abdominal pain in children: an emergency practitioner's
gide. Emerg Med Clin North Am 1989;7(3):497-517.
Campbell JPM, Gunn AA. Plain abdominal radiographs and acute abdominal pain. Br J Surg 1 988;75:554-556.
4/
ErunncrNcv MnucrNr: Tnn Conn CunrucuLUM
Fenyo G. Acute abdominal disease in the elderly experience from two series
in Stockholm. Am J Surg 1982;143:751-754.

Jess P, Bjerregaard, Brynitz S, et al. Prognosis ofacute nonspecific abdominal pain: a prospective sfidy. Am J Surg 1982;144:338-340.
LinXZ,Wang SS, TsaiYT, et al. Serum amylase, isoamylase, and lipase in
the acute abdomen. J Clin Gastroenterol 1989;ll(1)47-52.

Staniland JR, Ditchbum J, DeDombal FT. Clinical presentation
abdomen: study of 600 patients. Br Med J 7972;3:393-398.
of
acute
will present with a history that may

difficult and./or painful swallowing.
Progressive esophageal dilation may result in prolonged
retention or regurgitation of undigested food. Trapped
fluid may slosh or splash. The patient is consequently
The typical patient
span several years of
more prone to aspiration and pneumonia. The diagnosis is

typically not made in the ED; however, patients with dis-
covered
ESOPHAGUS (1.1)
or
suspected achalasia should be referred to
ensure appropriate follow-up.
The esophagus is the proximal poftion of the upper

gastrointestinal (GD tract originating just distal to the
epiglottis and ending at the entry to the stomach. It is the
most posterior component of the mediastinum. It is supplied from short branches off of the thoracic aorta and
from the inferior thyroid and left gastric arteries, and has
venous drainage through both portal and systemic distributions. It is innervated by the spinal accessory and vagus
nerves and the autonomic nervous system. It serves as the
portal of entry to the GI tract.
(1, 1. 1,
1)
Esophageal spasm results from synchronous contrac-
tion of esophageal smooth muscle along the length of the

esophagus and is typically associated with pain. The contraction and consequently the pain may last just a few
seconds or many hours. Spasm may be precipitated by
reflux, swallowed materials at extremes of temperature,
large food boluses, or exercise.
The patient will typically complain of substernal chest
pain, which may radiate to the arm, jaw, or neck. During
the course of the spasmodic episode the patient will also
have difficulty in further attempts at swallowing. Symptomatic relief from esophageal spasm may be accom-
plished with nitrates, calcium channel blockers, anticholinergics, and cyclic antidepressants. The emergency
physician must attempt to differentiate this pain from that
of cardiac etiologies, but on occasion this
cannot be
accomplished in the ED.
Achulasia (1.1.1.2)
Achalasia is a syndrome manifested by absent or
abnormal esophageal motility often coupled with a rigid
lower esophageal sphincter. The motility disorder is most
commonly recognized in the lower two-thirds of the
esophagus. It is a common cause of esophageal chest
pain, but is less common than reflux and spasm; many
patients with noncardiac chest pain suffer from unrecog-
nized achalasia. The true incidence and etiology are

unclear, but several authors report an association with a
decrease in the numbers of ganglion cells in the esophageal mesenteric plexus.
Scleroderma, also known as progressive systemic sclerosis, along with other etiologies that impair contraction
of
esophageal smooth muscle,
is
associated with
decreased and absent esophageal peristalsis. The lower

esophageal sphincter also may become incompetent in
scleroderma. Histologically there
deposition, leading
is increased collagen
to fibrosis and a thinning of the
mucosal elements. Air, or gas, visualized in the esophagus on posteroanterior (PA) and lateral chest films may
suggest the diagnosis of scleroderma as an etiology for
dysphasia. In the evaluation of the patient presenting to
the ED for dysphagia, the presence of sclerodactyly,
Motor Abnormalities (1.1.1)

Esophageal Spasm
Scleroderma
or Raynaud's phenomenon
should prompt a rheumatologic referral for further evaluation. The diagnosis is made by lack of motility on barium swallow and confirmed by esophageal motility stud-
telangiectasia, calcinosis,
ies.
Structural Disorders (1.1.2)

Varices (1.1.2.1)
Esophageal varices are dilated veins that occur as the
result of portal hypertension. Portal hypertension is most
commonly seen with hepatocellular disorders such as cirrhosis or cancer and should be suspected in patients with
these disorders. Portal vein thrombosis may occur in
patients with normal hepatic function. The occurrence of
a variceal bleed portends severe disease with high mortality. Nearly one-third of deaths from upper GI bleeds
occur in these patients.
A patient with a variceal bleed will typically present
with massive hemorrhage and shock. Fluid and blood
resuscitation is mandatory. Nasogastric tube placement
evaluation of continued bleeding should be performed. Underlying hepatocellular disease may also
induce coagulopathy and other metabolic disorders.
There is presently no evidence to suggest that careful
for
instrumentation causes variceal trauma or induces bleeds.

Persistent bleeding requires urgent gastroenterology consultation. Emergency therapy consists of vasopressin
infusion at 0.2 to 0.5 units per minute with emergency
embolization, sclerotherapy, or Sengstaken-Blakemore
AgooN,IINAL AND GASTRoINTESTINAL DIsonoEns
intubation reserved for massive persistent bleeding in
most commonly are the result of recurrent or violent
patients who are vasopressin failures.
regurgitation. These esophageal rents are usually superficial and mild with minimal self-limited bleeding. In some
cases the amount of bleeding may be massive and require
fluid and blood therapy.
The patient will typically present with a complaint of
bright red blood in the vomitus after series of regurgitations. Hemodynamically stable patients without evidence
of active upper GI bleeding on nasogastric evaluation
may be managed conservatively as outpatients. In cases
with ongoing or massive blood loss, the patient should be
admitted for observation and therapy.
Rupture
(B
oerhaave's Syndrome)
(1. 1. 2,
2)
Boerhaave's syndrome, esophageal rupture, typically

results from a force applied against a weakened esophageal wall as occurs with repeated emesis or prolonged
gastric intubation. Vomiting, hiccuping, or performing
the Valsalva maneuver against a closed glottis can also
result
in esophageal
detected
rupture. Mediastinal gas may be
by auscultating Hamman's crunch, which is
characteristic crunching sound accompanying systole.

The presence of mediastinal emphysema, a mediastinalbased left pleural effirsion, or left-sided empyema are all
suggestive of Boerhaave's syndrome.
The treatment of Boerhaave's syndrome is surgical.

Fluid resuscitation and rapid broad-spectrum antibiotics
are important ED interventions. The evacuation of a
pneumothorax may be indicated in the ED as well. The
combination of chemical and bacterial insult produces a
rapidly progressive and profound mediastinitis. In the
absence of treatment, cases of mediastinitis from Boerhaave's syndrome are usually fatal in 2 to 3 days.
Perforation (1.1.2.3)
Perforation may occur from penetrating trauma, foreign body ingestion, or iatrogenic causes. The most common offending instrument is the rigid endoscope. A case
report of perforation following the Heimlich maneuver

has been documented. Perforation also may also occur
following erosion or ulceration, or as a complication of
carcinomatous spread. Perforation of the cervical esophagus in the setting of flexion-hyperextension trauma has
been reported.
is best demonstrated radiis less likely to

Gastrografin
Although
ographically.
Esophageal perforation
an esophageal perforation, it should be

first, as it is better tolerated in the mediastinal
demonstrate
employed
space and does not obscure visualization for endoscopy.
If a Gastrografin swallow does not demonstrate a perforation when one is suspected" especially with penetrating
trauma, then a barium esophagram should follow.
Perforation when unrecognized and untreated can lead
to indolent and eventually overwhelming infection simi-
lar to that with rupture. Historically the treatment has

been that of surgical repair. A small percentage of small
perforations have been shown to heal spontaneously
without infectious sequelae.
Tears (Mallory-Weiss Syndrome)
(1. 1.
2.4)
Mallory-Weiss tears are longitudinal disruptions in the

esophageal mucosa at the gastroesophageal junction that
Hemstoma (1.1.2.5)
Esophageal hematomas are a relatively rare finding
and can be either traumatic or nontraumatic. Intramural
esophageal hematomas are of two main types: those with
and those without a communication with the intraluminal
space. Those with a communication radiographically

appear with the "double-barrel" lumen sign and are
thought to represent a paftial rupture of the esophagus.
Regardless of type, esophageal hematomas may be associated with bleeding or mass effect resulting in dysphasia
or chest pain. Etiologies include hemophilia, coagulopathy, erosion from aortic aneurysm, foreign-body ingestion, vomiting, uremic hypocoagulation, carbon tetrachloride inhalation, and blunt trauma (including minor
head injury). Iatrogenic precipitants are as follows: upper
endoscopy; sclerotherapy of esophageal varices; and
emetic administration, surgical vagotomy, and esophageal dilation, either direct or pneumatic. There are scattered case reports of spontaneous intramural esophageal
hematoma.
Foreign Body (1.1.2.6)
Aside from children and those with cognitive impairment, increased risk for esophageal foreign body occurs
in alcoholics and those with dentures. Inmates in correctional institutions are a special risk, in that they will
attempt concealment of restricted material or will ingest
material to seek a temporary medical release from the
facility. (Body packing for the smuggling of illicit drugs
may also cause obstruction.) Foreign-body ingestion
occurs most commonly in toddlers.
Small objects (i.e., <2 to 5 cm) are more likely to pass
through the esophagus. Objects that lodge do so at the
predictable narrowings located at the cricopharyngeus,
aortic arch, left mainstem bronchus, and the lower esophageal sphincter. In pediatric patients, in addition to the
above, objects may lodge at the thoracic inlet' Patients
with esophageal canceq esophageal webs and rings,
esophageal strictures, and motility problems may present
with obstructing objects at other levels. More that 95% of
6 /
EN,rsncsNcy MrorcrNr: THn Conn CunrucuLUM
patients with a piece of meat impacted in the esophagus

will have predisposing pathology. The typical adult may
present with a history of ingestion or may have the sen-
sation that something is lodged. Children are often not

observed to have ingested anything and may manifest
with drooling, retching, gagging, coughing, stridor, or an
inability or unwillingness to eat or drink.
In the evaluation and management of the patient with
an ingested foreign body, it is important to maintain and
protect the airway. The clinical situation may require intubation to protect the airway. In some cases, particularly in
children, the emergency physician may need to determine
the nature ofthe foreign body and confirm that it lies in
the esophagus and not in the respiratory tree. Indirect
laryngoscopy may be of benefit in some cases. In the case
of ingested coins, plain radiographs can make this determination. Esophageal coins will be viewed in the anteroposterior (AP) or head-on projection on standard AP or
PA radiographs. Tracheal coins will be viewed in the lateral or edge-on projection on standard AP or PA radiographs. An easy way for the clinician to remember this
may be to consider that the coin must turn sideways to
pass through the vocal cords into the trachea. The failure
to identify a foreign body by plain radiographs does not
exclude its presence.
Localization can be aided by the use of contrast radiographs. A barium-soaked cotton ball may be employed.
Water-soluble contrast is preferred in the evaluation of
esophageal foreign body for two reasons: first, the presence of barium makes endoscopic removal difficult, if not
impossible, because of impairment of visualization; and
second, if a perforation has occurred, water-soluble contrast is much better tolerated in the mediastinum. If a perforation is suspected but cannot be demonstrated with
water-soluble contrast, barium must be employed before
perforation can be excluded.
In the ED small esophageal foreign bodies, other than
sharp objects and batteries, can be observed for up to 12
hours to allow for spontaneous passage. Healthy patients
with small objects in the distal third of the esophagus can
be considered for glucagon administration (0.5 to 1.0 mg
IV) to relax the esophagus and enhance the likelihood of
passage. Both nifedipine and nitroglycerin also relax the
smooth muscle of the esophagus and may be utilized.
Any object that does not pass out of the esophagus

requires endoscopic removal. On occasion lodged
digestible material can be advanced into the stomach
under endoscopic guidance rather than retrieved, thereby
eliminating the risk of aspiration. Attempts at esophageal
foreign body removal with Foley or other balloon
catheters should be discouraged" even under fluoroscopic
guidance, because of the risk of pulmonary aspiration and
respiratory compromise. Although the use of enzymatic
degradation of impacted food has been advocated in the
past, such agents can also degrade the esophagus, resulting in perforation, and should not be employed.
Button-battery ingestion represents a special circumstance ofesophageal foreign body. The battery may cause
esophageal electrical burns in as soon as 4 hours after

ingestion and esophageal perforation in as soon as 6
hours. It is thus imperative that an esophageal battery be
removed or advanced into the stomach immediately.
Nondigestible objects that pass through the esophagus
into the stomach require surveillance to ensure successful
passage. Objects have been known to lodge in diverticula
or in the appendix with infectious and obstructive consequences.
Diaphragmatic Hernia
(1. 1.
2.7)
Diaphragmatic hernias can be either congenital or

traumatic. The neonate born with congenital diaphragmatic hernia typically has early severe respiratory distress, small lung volumes, and a sunken abdomen. Ninety
percent of congenital diaphragmatic hernias occur on the
left and correspondingly displace the apical impulse of
the heart to the right. The diagnosis, if not made in utero,
is made when abdominal viscera are seen above the
diaphragm on the chest roentgenogram. Treatment is sup-
portive initially with endotracheal and gastric intubation

for respiratory distress with eventual surgical repair.
Traumatic diaphragmatic herniation can occur as a
result of blunt trauma or with penetrating injury . The
majority of cases are on the left and recognized by early
migration of abdominal contents into the thorax. With
right diaphragmatic hernia the diagnosis may be delayed
as the presence of the liver slows this translocation.
Patients who do not have an early radiographic diagnosis
made will present with respiration compromise and dysp-
nea. The treatment
of
diaphragmatic hernias, whether
penetrating or blunt in etiology, is operative. patients with

severe respiratory distress may require endotracheal and
gastric intubation with more urgent repair.
Diverticula (1.1.2.8)
Zenker's diverticulum is the most common esophageal
diverticulum and is a pharyngoesophageal pouch that

may result from incomplete relaxation of the cricopharyngeus. The patient may complain of regurgitation of
food ingested several days earlier. The aspiration offood
contents from this cavity may occur when the patient lies
down. This chamber may be seen on neck radiographs.
Treatment is surgical.
Hiatal Hernia
(1.
1.2.9)
A hiatal hernia is defined
as the herniation
ofpart of
the stomach into the thoracic cavity. If the gastroesophageal junction and part of the fundus of the stomach her-
Aroouner
niate, this is known as a sliding hiatal hernia;
if the gas-
troesophageal junction remains in the abdomen and

another portion of the stomach herniates alongside the
esophagus through the esophageal foramen, this is known
ur i putuetophageal hernia. The majority of cases of
hiatal hernia are asymptomatic, and by age 70 as many as
600/o of patients may have a hiatal hernia. The presence of
a hiatal hernia may enhance reflux or may strangulate or
incarcerate, producing pain or obstruction. A hiatal hernia may require repair if it is large or symptomatic.
Webs and
Strictures (1.1.2.10)
AND GASTRoINTESTINAL
DIsorueRS
estrogens, and anticholinergics. Also of concern are precipitants ofincreased gastric acidity such as spicy foods,
smoking, stress, and anxiety. Decreased esophageal

motility and diabetes can also worsen reflux and potentiate esophagitis. Most of the damage due to reflux occurs
at night while the patient sleeps. During this and other
periods of recumbency, there is no gravitational resistance to proximal migration of gastric contents. Exercise,
forward bending, lifting, or straining may also precipitate
reflux. The patient with reflux esophagitis will often

complain of severe heartburn, sometimes awaking from
sleep. A sour taste or exacerbation by leaning forward or
with the Valsalva maneuver is a common historical feature.
Esophageal Webs
Esophageal webs are outpouchings in the squamous
epitheiium of the esophageal mucosa that may be cir-
cumferential. When they occur in the distal esophagus

they are also referred to as esophageal rings or Schatzki's
ring. A hiatal hernia is often present with a ring. Plum-"t-Vittto.t syndrome is the combination of iron-defi-
ciency anemia
As rings beco
The mainstay of treatment for reflux esophagitis is

behavior modification to reduce the risk factors. The
obese and marginally overweight should be counseled
that weight loss will also reduce reflux. The head of the
bed can be elevated 4 to 6 inches to reduce nocturnal
reflux. Hz blockers can increase gastric pH. Short courses
of proton pump inhibitors such as omeprazole have been
reported
to be of benefit. More recently
promotility
agents such as metoclopramide have been associated
with
success. Sucralfate, particularly when given as a slurry,

will provide both symptomatic relief and reduce the dam-
monly present
decade. Ifnot
ies, webs present with obstruction.
Esophageal Strictures
Esophageal stricture formation is a complication of

esophigeal inflammation or injury such as with chronic
insult from reflux esophagitis or with single wounding as
in the case of caustic ingestion (see Inflammatory Disorders, below). Stricture-complicating reflux may be more
age from gastric acid. Patients with severe reflux

esophagitis require elective endoscopic evaluation to
exclude the precancerous lesion of Barrett's esophagus
and to evaluate for stricture formation. Recalcitrant cases
can be referred for surgical therapy such as by Nissen
fundoplication.
Cuustic
Injury
(1. 1.3.
2)
Caustic esophageal injuries result from ingestion

either acidic or alkaline substances.
of
Caustic Alkali
tis is also reported.
Inflammatory Disorders (1.1.3)

Reflux Esophagitis
(1.1.3. 1)
Reflux esophagitis is the erosive or digestive injury to

the esophagus that occurs with the retrograde propulsion
or leakage of acidic gastric, or rarely alkaline bilious,
materials proximal to the lower esophageal sphincter
(LES). It is often associated with decreased LES tone and
hiatal hernia.
Reflux esophagitis is worsened by agents or activities
that decrease the tone of the LES. These include smoking
(nicotine), caffeine, chocolate, alcohol, fatty foods, preg-
nancy, nitrates, calcium channel blockers, progestins,
The degree of injury with alkaline caustics is related

to several factors. The physical state of the material is
important. Liquid caustics are associated with more
rapid and diffuse injury than are solids that stick on a
particular site and burn until they are removed or
washed off. Alkaline caustics will continue to burn until
they are removed or diluted by the liquefaction that they
induce. Thus, the duration of contact and time until dilution portend the degree and depth ofinjury' Ifthe caustic induces laryngeal or esophageal spasm, it can be
held and its effect concentrated in a local area. The
stomach contents also play a role, particularly in the
dilution rate, but not to the same degree as that with
acidic ingestions. Pyloric sphincter tone also plays a

role in the duration of contact. Aspiration of caustic
alkali may produce glottic and subglottic edema, aspira-
B/
EnarncoNcy MeucrNr: Tun Conr CunrucuLUM
tion pneumonitis, adult respiratory distress syndrome,

and respiratory-enteric fistulae.
The predominant site of injury for caustic alkali is the
esophagus where rapid liquefaction necrosis has already
taken place by the time the patient presents to the ED.
Full+hickness esophageal burns are complete in I second
of contact with 30% sodium hydroxide, but require a full
10 seconds
with22.5% sodium hydroxide.
Alkaline esophageal burns evolve through four distinct

phases. Phase 0 is the immediate liquefactive necrosis.
Phase 1 occurs in the first 5 days and is marked by an
acute inflammatory process with mucosal hemorrhage,
increasing vascular thrombosis and the sloughing of
necrotic layers. During this time translocation of bacteria
from the esophagus to the mediastinum may occur without perforation and lead to mediastinitis. phase 2 is a
repair phase during which granulation tissue develops,
collagen deposition begins, and mucosal reepithelialization begins. Phase 2 is often complete by l5 days postinjury, but may last up to 6 weeks. Phase 3, beginning anytime after 2 weeks postinjury, is manifested by scar
retraction. It is this progressive contraction of collagen
fibers that reduces both circumference and length of the
esophagus and that
strictures in 7oh
will eventually give rise to esophageal
to 15% of patients.
In the evaluation and treatment of caustic ingestion,

particular attention must be paid to the airway. Rapid pro-
esophagus are somewhat spared. The stomach is most

commonly involved and the preingestion stomach con_
tents determine the location of maximal injury. In the
case of an empty stomach, the injury is located in the
antral and midgastric regions, unlike the distal injury
found with solid-filled stomachs. A liquid-filled stomacl
will
demonstrate diffrrse injury.
Caustic acids produce
a coagulation necrosis with
also
gas, and
The evaluation of acid ingestion by clinical means is

corre-
injury
rophaof correlate well with gastric injury.
The treatment of acid ingestion also requires careful

rapid assessment for
tion. The treatment is
ticular attention to t
acid-base disorders. Dilution and neutralization of acid is
contraindicated secondary to the strong exothermic reac_
tion that ensues.
these patients. Authors differ on whether or not
milk (or,
less preferably, water) dilution should be employed.
Opponents cite the increased risk of vomiting, given the
likelihood that all the damage may have already been
done. Immediate gastroenterologic consultation for careful early endoscopy should be employed. The prudent
endoscopist will advance the endoscope only to the point
of the first circumferential (phase 3) burn and withdraw
This will minimize the risk of iatrogenic perforation and
confirm that the burn is basically as bad as it can be. Any
patient with a phase 3 burn should have bronchoscopy to
evaluate the degree of pulmonary injury, and these
patients may do better with early surgery.
Esophageal rest for the first 6 to 8 weeks is universal
therapy. These patients require close follow-up and
should receive dilation or replacement for stricture
depending on the severity. Lifelong surveillance is mandated, given the increased risk of squamous esophageal
carcinoma following alkaline caustic injury.
Caustic Acids
With acidic compounds, greater contact time is

required for injury. Consequently, the oropharynx and
Infectious Disorders (1.1.4)

Herpetic Esophagitis
(1. 1.4.
l)
Herpetic esophagitis is the upper GI manifestation of

herpes simplex virus infection. It can occur in nonimmunocompromised individuals. It typically follows an
upper respiratory infection, and the majority of immuno_
onset. As with other viral lesions, it is self-limited in the

immunocompetent, and complications are more common
in the immunocompromised, who should receive acy-
clovir therapy.
Monilial Esophagitis
(1.
1.4.2)
Monilial or yeast esophagitis is manifested by mild

odynophagia. Should severe pain with swallowing be
manifest, another or coexistent process is suspect. In the
immunocompromised patient, monilial esophagitis may
occur in the absence of any oropharyngeal findings.
AsloN{rNAL AND GASTRoTNTESTTNAL
of choice for Candida

esophagitis, but some patients, particularly the immunocompromise4 may require higher doses or fluconazole.
Patients who progress to systemic infection should
receive a course of amphotericin B.
Ketoconazole remains the drug
Tumors (1.1.5)
Esophageal carcinoma is a devastating disease. It typically presents in advanced and/or inoperable stages, usually (70%) after metastasis has occurred. This results in a
lower 5-year survival than that seen with many other
malignancies. Patients at greater risk for esophageal can-
cer include smokers, drinkers, tobacco chewers, those

with Barrett's esophagitis, and those with esophageal
stricture following caustic ingestion.
The typical patient will present after a period of progressive dysphagia and tumor growth. Unfortunately, the
esophagus must be narrowed by 50% to 670/o before the
patient experiences difficulty swallowing solids; thus, the
tumor is advanced and has had the opportunity to metastasize before the symptoms have become severe enough
to cause the patient to seek medical attention. The patient
may not have difficulty with liquids until the lumen has
been narrowed to a diameter of 4 mm by the growth of the
cancer. The occurrence of odynophagia is a poor prognostic indicator. Depending on the location of tumor and
its pattern of spread, the patient may also present with
voice changes; phrenic nerve dysfunction; gastroparesis;
neck mass from tumor, infection,
or
subcutaneous
emphysema; bleeding; or pulmonary infection secondary

to either aspiration or perforation.
Any patient with suspected or possible esophageal
malignancy should have an urgent outpatient evaluation
and staging. If such cannot be arranged" admission may
be necessary.
SELECTED READING
Anderson KD, Rouse TM, Randolph JG. A controlled trial of corticosteroid
in children with corrosive injury ofthe esophagus. N Engl J Med 1990;
323(10):637440.
Davies HA. Anginal pain ofesophageai origin: clinical presentation, prevalence, and prognosis. Am J Med 1992;92(suppl 5A):5s-10s.
DeVault KR, Castell DO. Current diagnosis and treatment of gastroesophageal reflux disease. Mayo Clin Proc 1994;69:867-876.
Gumaste V! Dave PB. Ingestion ofcorrosive substances by adtrJts. Am J
Gastroenterol 1992;87( I ): l-5.
Kerr WF. Spontaneous intramural rupture and intramural haematoma of the
oesophagus. Thorax 1980;35:890-897.
MatloffDS. Treatrnent of acute variceal bleeding. Gastroenterol Clin North
Am 1992;21 (l): 103-1 18.

Ott DJ. Motility disorders of the esophagus. Radiol Clin North Am 1994;
32(6):1117-1134.
Peters JH, DeMeester TR. Gastroesophageal reflux. Surg Clin North Am
1 993 ;7 3 (6): I t t9-t | 44.
Webb WA. Management of foreign bodies of the upper gastrointestinal
tract: update. Gas tro intes t E ndosc 1 995 ;4 I ( I ) :39-5 1.
Wilcox MC. Esophageal disease in acquired immunodeficiency syndrome:
etiology, diagnosis, and management. Am J Med 1992;92:412421.
LrvER
Dnononns / I
(1.2)
The liver is the largest solid organ in the body. It is

located in the right upper quadrant of the abdomen lust
beneath the diaphragm, protected by the rib cage. It is
supplied with blood by the portal vein, which drains the
splenic, intestinal, and colonic fields, and is a rich source
of nutrients and substances absorbed from the gut. It is
also supplied by the hepatic artery, a branch ofthe celiac
artery, which provides the majority of the liver's oxygenated blood. Blood flow within the hepatic microvasculature passes from the portal tria4 which contains the
terminal venules of the portal vein and the arterioles of
the hepatic arter1y, through the sinusoidal capillaries, a
low pressure capillary system relatively permeable to
serum filtrate, to the central venules that eventually coalesce to form the hepatic vein, which drains the liver.
Within the portal triad are the canaliculi of the biliary
tree, which form the bile ducts and carry bile formed
within the liver to the gallbladder and the intestine. The
parenchyma of the liver is devoid of pain fibers but the
organ is encapsulated by Glisson's capsule, which is
heavily innervated. Puncturing or distorting Glisson's

capsule results in the clinical sensation of pain.
The liver stores glucose in the form of glycogen and
through gluconeogenesis it is able to supply this source of
energy during fasting. It detoxifies drugs and metabolites
that pass through it via the portal vasculature. The liver
and the kidney provide the two most common pathways
by which foreign substances are excreted from the body.
The liver is the sole site of albumin production and is the
source of many other proteins in the body including coag-
ulation factors, complement factors, and transport moieties. Bile, which solubilizes fat during digestion and
contains substances excreted from the body via the liver,
is made in the liver at the canalicular membranes.
Approximately 20% of the cardiac output passes through
this vital organ, which serves as a complex biochemical
"factory."
Evaluating liver function clinically involves obtaining
a thorough history, performing an appropriate physical
examination, reviewing the liver function tests (LFTs),
and utilizing avariety of radiologic and nuclear medicine
procedures when appropriate. Liver biopsy to obtain tissue for histologic review is often mandatory for precise
diagnosis and the determination of the extent of liver disease. Some of these methods of assessment are commonly employed in the ED. The history, physical examination, LFTs, and certain less invasive radiologic studies
such as ultrasound are readily available tools. Other
modalities used to detect liver disease, such as the
liver/spleen scan or biopsy, are rarely employed in this
setting. Although the precise diagnosis of liver disorders
is rarely confirmed during a single ED visit, a careful and
focused approach will allow the clinician to determine if
management requires an inpatient versus an outpatient
10 /
ErrasncnNcy MnorcrNn:
Tnr
Conn CunrucuLUM
setting and facilitates all aspects of subsequent clinical
Excess unconjugated hyperbilirubinemia may result from
care.
hemolysis, which can cause excess bilirubin formation

and overwhelm the normal liver. Elevations in conjugated
bilirubin denote pathology in the liver or biliary system.
Other LFTs such as the 5'-nucleotidase or 1-glutamyl
transpeptidase are useful in determining if alkaline phosphatase abnormalities are liver related, but they take several days to process and are rarely useful for decision
making in the ED setting.
A cost-effective approach to liver disease requires a
working knowledge of the tools available to the practitioner for evaluating liver disease, as well as a basic
understanding of the pathophysiologic processes involving the liver. Hepatitis, cirrhosis with or without synthetic
failure, portal hypertension and its complications, hepatic
neoplasia, and hepatic abscesses are diseases ofthe liver
encountered in emergency medicine. Practitioners in this
setting should be familiar with the basic evaluation and
management of these entities.
Historical information regarding liver diseases should

include the standard detailed "history ofpresent illness"
as is usually obtained. Specific points such as drug/toxin
exposures, risk factors for viral hepatitis (sexual practices, blood transfusions, intravenous drug abuse, etc.),
family history of liver disease orjaundice, alcohol usage,
and prior liver dysfunction must receive extra attention
during questioning. The presence or absence ofjaundice,
hepatomegaly, splenomegaly, "spider" telangiectasias,
pahner erythema, caput medusae, gynecomastia and tes-
ticular atrophy in males, Dupuytren's contractures, and

ascites must be carefully recorded whenever liver disease
is suspected. A carefully obtained history and physical
examination may not disclose serious liver disease. Indolent chronic hepatitis, for example, may be present for
decades without being noticed unless more invasive
rneans of evaluation are employed.
Liver function tests (LFTs) are chemical and hematologic studies grouped together due to their potential relevance to hepatic abnormalities. Strictly speaking, these
assays do not measure actual hepatic function and they
may be "abnormal" as a result of the dysfunction of organ
systerns other than the liver and biliary tree. The prothrombin time (PT) and the serum albumin come closest
to being actual tests of liver "function." Failure of liver
synthetic function will lead to an elevated PT and bleeding as well as hypoalbuminemia and its consequences.
However, vitamin K deficiency (broad-spectrum antibiotic use, fat malabsorption, malnutrition) can lead to an
abnormal PT despite an intact liver, and malnutrition or
excess albumin wastage, such as occurs in the nephrotic
syndrome, can lead to hypoalbuminemia despite a functioning liver. The aminotranferases, alanine aminotransferase (ALT) and aspartate aminotransferase (AST), are
commonly part of a "liver function" panel. They are routinely elevated with hepatocellular damage or death such
as would occur in hepatitis. Although ALT is far more
specific for liver tissue than AST, neither is exclusive to
liver tissue and must be interpreted in this light. Alkaline
phosphatase actually represents a family of enzymes that
are associated with cholestasis, the blockage of the production and flow of bile. Alkaline phosphatase levels may
be significantly elevated as a result of a biochemical
defect in bile production at the canalicular level or secondary to a gross mechanical obstruction to bile flow
such as an impacted gallstone. This family of enzymes is
not exclusive to the liver. Bone tissue has active alkaline
phosphatases, and bone growth or repair leads to elevated
serum levels of alkaline phosphatase. Bilirubin, the
metabolite of hemoglobin, is conjugated to make it lipid
soluble within the hepatocyte. It is subsequently excreted
via bile. Obstruction to the creation of bile or its flow
through the biliary ductal system, as well as failure in the
conjugation process, will cause hyperbilirubinemia.
Hepatitis (1.2.1)
^ Hepatitis
literally means inflammation of the liver.

Somewhat arbitrarily hepatitis has been divided into
"acute" (less than 6 months in duration) and "chronic"
(greater than 6 months) categories. There are many
causes for hepatitis. Viruses, drugs and toxins (especially
alcohol), and hypoxemia are the most cornmon etiologies.
Wilson's disease and crr-antitrypsin deficiency are two
rare inherited metabolic diseases that cause chronic
hepatitis. Various types of autoimmune hepatitis exist and
these diseases almost invariably become chronic in
nature. Acute self-limited hepatitis usually heals without
sequelae. Rarely, the etiologic agent (a virus or drug for
example) will cause fulminant destruction of the liver
parenchyma with lifethreatening results. Although liver
transplantation has significantly improved the overall

outcome in many of these patients, the syndrome of fulminant hepatic failure still has mortality rates of between
30To and7}o/o. Chronic hepatitis may remain indolent and
subclinical for decades until the patient succumbs to an
unrelated problem. It may also lead to liver destruction
with synthetic failure and portal hypertension within
months. The multiplicities of etiology and natural course
make hepatitis a challenging medical condition.
Viral (1.2.1.1)
Viral hepatitis is common. Hepatotrophic viruses A

through E and G (HAV HBV and so on), Epstein-Barr
virus (EBV), cytomegalovirus (CMV), and herpes simplex virus (HSV) are the major viruses responsible for
hepatitis. Many other viral agents can cause liver inflammation as part of their clinical presentations as well. HAV
and HEV are selflimited diseases. The other hepa-
AsooNrrNAL eNo GISTRoTNTESTTNAL
totrophic viruses may lead to chronic hepatitis. The natural course of the newly discovered HGV infection is not
well characterized as yet. EBV (mononucleosis), CMV
("heterophile-negative" mononucleosis), and HSV infections generally cause systemic infections that may or may
not have hepatitis as a major clinical component.
Hepatitis A and E are RNA viruses spread via the
fecal-oral route through contaminated water or foods.
Less than 0.01% of the HAV infections lead to fulminant
hepatic failure, but l0% or more HEV infections (especially in pregnant women) cause death. HAV is very common in the United States but there has not been an outbreak of HEV in the United States yet. Gamma globulin
reduces the clinical manifestations of HAV after exposure. A vaccine is available for the prevention of HAV
Prophylaxis against HEV is not yet available. Pregnant
women are advised against travel to endemic areas. HB!
HCY and HDV may all be self-limited or lead to chronic
infection. HBV (a DNA virus) leads to chronic hepatitis
in approximately l0% of cases. Chronicity usually occurs
in babies who acquire the virus from their infected moth-
ers at birth. HDV is an incomplete RNA virus that

requires coinfection with HBV Like HBV HDV is transmitted via the parenteral route and will become a chronic
infection if the HBV does so. Hepatitis B immune globulin (HBIG) is used to prevent clinical disease after exposure to HBV A successful vaccine against HBV has been
in use for over l0 years. Successful prophylaxis against
HBV prevents HDV Unexpected births in the ED to
mothers infected with HBV should prompt inoculation
with HBIG and the first of the three doses of the HBV
vaccination series. HCV is an RNA virus spread by parenteral means. Sexual contacts and maternal-fetal infections are recognized" but the rates ofthese routes oftransmission are less than for HBV Intravenous drug abuse
with shared needles is a common source of infection for
both HBV and HCV Forty percent of HCV infections are
considered to be of unknown origin. In HCV over 5070 of
those infected become chronically infected, although the
subsequent clinical courses are variable from patient to
patient. Some people are never clinically compromised
while others encounter cirrhosis, liver failure, and/or
hepatocellular carcinoma. Gamma globulin has not provided prophylaxis and there is no vaccine.
Patients with viral hepatitis commonly present with
flulike symptoms (malaise, low-grade fever, myalgias,
anorexia) and may not be recognized as having hepatitis
without concomitant jaundice. Other liver and biliary
tract disorders can present similarly especially in the
elderly and immunosuppressed population. Serologies
are needed to confirm the diagnosis (although routine
serologic testing is not yet available for HEV and HGV).
The following serologies should be sent when the diagnosis ofviral hepatitis is suspected: hepatitis A antibody
(HAV Ab); hepatitis B surface antigen (HBV sAg), surface antibody (HBVsAb), and core antibody (HBV cAb);
DrsoRorns
11
and an antibody to hepatitis C (HCV Ab). Further serologic testing may be warranted after these studies have
been reviewed. The results of these initial serologic tests
will be available hours to days after the initial visit, but
obtaining them promptly in the ED facilitates further
workup.
In the ED it is also necessary to determine whether

admission to the hospital is required or outpatient followup is adequate. The latter is appropriate in the vast majority of cases. Patients require admission for intravenous
hydration if severe nausea and vomiting have led to dehydration. They also require urgent admission if there are
any manifestations of liver failure (as discussed below).
Outpatient follow-up with a primary care physician is
acceptable for the routine presentations ofviral hepatitis,
but follow-up should be timely and include repeat LFTs
to ascertain stability, deterioration, or improvement. In
addition, follow-up review of the pending serologic tests
should be arranged. If infectious hepatitis is confirmed,
all potentially exposed contacts should be sought and
prophylaxis, if available, should be offered. Viral hepatitis may be reportable to local public health officials.
Hepatotoxins such as alcohol and excess acetaminophen
should be avoided during recovery. Relatively small

doses of acetaminophen (greater than 2 g per day) can be
dangerous when there is existing liver damage. Patients

with hepatitis should be counseled to avoid behaviors that
might spread infection, such as food handling, sexual
contact, blood donation, etc., until the risk of infection
can be clarified and more specific recommendations can
be made.
Drug and Toxin (1.2;1.4)

Drugs and toxins are another very common cause of
both acute and chronic hepatitis. Drugs may cause idiosyncratic liver disease or they may do so in predictable
dose-related fashion. Virtually all drugs and many environmental chemicals may result in liver disease. When a
patient presents with symptoms and signs of liver disease,
a thorough drug and toxin exposure history must be
obtained. Over-the-counter preparations can be just as
dangerous and the review should include them as well as
prescribed medications and illicit "recreational" drugs.
All unnecessary drugs should be stopped until the precise

diagnosis of the liver disorder can be ascertained. Alterations in drug dosing might need to be considered when
there is the possibility of altered hepatic drug metabolism. Substitutions should be considered for any necessary drugs suspected of being the cause of the hepatitis.
Liver disease may manifest as hepatocellular dysfunc-
tion, cholestasis, or both. Treatment generally
centers
around the removal of the offending agent and supportive

care. Fulminant hepatic failure as a result of drug toxicity often requires transplantation. Chronic autoimmune
12 /
ElmncnNcy MnorcrNn:
TABLE
1-1.
Tnr Conr
CunrucuLUM
Common drugs known to induce liver disease
Nitrofurantoin
Sulfonamides
Acetaminophen
Aspirin
lsoniazid
Phenytoin
Diltiazam
Methotrexate (hepatopathy often without abnormal LFTs)
Note: All drugs should be suspected until another etiology
for liver dysfunction is found. Drug doses may need to be
altered in patients with liver dysfunction.
hepatitis may result from drug-related hepatitis and

require corticosteroids and/or immunosuppressive therapy. Table l-l lists several drugs well known to induce
liver disease. Virtually any drug may be responsible and
all should be considered when the diagnosis of liver disease remains obscure.
Alcoholic (1.2.1.5)
Alcohol causes fatty liver, which is reversible upon disof consumption. Cirrhosis, the fibrosis of
hepatic parenchyma, can result from the consumption of
alcohol in large quantities for long periods of time.
Hepatitis is a rarer, but very serious, result of ethanolism
with a 50% mortality rate. Controversy exists as to
continuation
whether alcoholic hepatitis is an acute or a chronic hepatitis. Clinically, the disorder manifests fully within weeks
of the onset of symptoms. However, this potentially lethal
form ofhepatitis requires lengthy and heavy exposure to
ethanol. Unlike the relatively subtler presentations of viral
hepatitis and drug-induced hepatitis (excepting fulminant
hepatic failure as a presentation), alcoholic hepatitis often
presents with fever, significant leukocytosis, prostration,
and profoundly deranged liver function. Because of the
need for pyridoxine as a coefficient of ALT and ASI and
the fact that pyridoxine deficiency is common in alcohol
abuse, the peak levels of these two enzymes are generally
much lower than those seen in viral, drug-induced, or
ischemic hepatitis. This should not be interpreted as suggesting that alcoholic hepatitis is less serious. Alcoholic
hepatitis should prompt admission to the hospital and
immediate cessation of all alcohol consumption. Aceta-
minophen should be avoided if at all possible, or dosed

sparingly (no more than2 gtotal per day) as patients with
alcoholic liver disease are very susceptible to acetaminophen toxicity. Good, balanced nutrition is important
to the recovery of these patients and corticosteroids may
be helpful in serious cases that are not complicated by
gastrointestinal bleeding.
Autoimmune hepatitis is uncommon. Drugs may trigger autoimmune hepatitis, but many cases are idiopathic.
Treatment is aimed at removing possible culprit drugs
and initiating immunosuppressive therapy with pred-
nisone (or an equivalent) and azathioprine under the
direction of a gastroenterologist/hepatologist. Most

patients can be handled as outpatients unless complications such as portal hypertension or liver failure intervene. If autoimmune hepatitis is suspected, an antinuclear
antibody and an anti-smooth muscle antibody should be
obtained.
Ischemia, both acute and chronic, can cause hepatitis

andlor hepatopathy. Similar to acute tubular necrosis, or
adult respiratory distress syndrome (ARDS), ',shock
liver," with significant elevations in transaminases and
concomitant synthetic and metabolic dysfunction, can
result from systemic hypotension or an acute restriction
of hepatic blood flow. The liver's dual blood supply
lessens the likelihood of this occurring at the local level.
The ED may treat patients with this entity
if
they have
been transported from outlying areas or for some other

reason have had time elapsement since the initial hypoxic
insult. Maintenance of hemodynamic stability and tissue
oxygenation and the avoidance ofpotential hepatotoxins
during recovery are the mainstays of therapy.
Wilson's disease is a congenital disease involving inappropriate copper metabolism with subsequent copper
overload in the liver and brain. It may present emergently
as fulminant hepatic failure with or without a hemolytic
crisis, or as a complication of cirrhosis and portal hypertension. Often it is recognized as chronic hepatitis due to
abnormal LFTs or when family members of known

probands are screened. Emergent treatment focuses on
the management of the portal hlrpertensive complications
or emergency orthotopic liver transplantation in the case
of fulminant hepatic failure.
Cirrhosis (1.2.2)
Liver failure refers to the condition in which enough
hepatic parenchyma has been damaged or destroyed that
synthetic and metabolic function are compromised.
Clearly, at some point in time, liver failure can lead to the
death
of the individual. Fulminant hepatic failure
has
been arbitrarily defined as the clinical syndrome in which

a patient with no known prior liver disease suffers deteri-
orating liver function and resultant hepatic encephalopathy within 8 weeks. If this process evolves between 8
weeks and 6 months it is considered subacute or subfulminant hepatic failure. Chronic liver or hepatic failure
takes 6 months or longer to transpire. These definitions
are clinical. In the case of fulminant hepatic failure the
hepatocytes are generally destroyed in large numbers
leading to metabolic failure. In the ED setting the complications of liver failure and portal hypertension are
commonly encountered and the practitioner needs to be
aware of them. Liver biopsy can be quite dangerous in the
face of severe coagulopathy and is rarely performed in

this setting.
AenoN,rrNAr eNo GIsTRoTNTESTTNAL DrsoRnnns
Synthetic failure can lead to hypoalbuminemia as well

as other protein deficiencies causing defects in immunity,
coagulation, and carrier mechanisms. Deficient gluconeogenesis leads to hypoglycemia. Portal hypertension is
the condition in which the blood pressure in the portal
venous system exceeds ll to 12 mm Hg and blood is
shunted back to the systemic venous circulation via ves-
tigial venous systems. The hepatic sinusoids are

bypassed. This creates two basic problems. First, metabolites and drugs from the gut bypass the liver, which would
normally process them. This leads to an increased levels
of a variety of substances in the systemic circulation,
some of which have untoward biologic effects. Additionally the liver is deprived of its metabolic needs, which
may worsen the liver disease. Second, portal hypertension
presents a mechanical problem. Thin-walled vestigial
veins may not be able to tolerate the increased blood pres-
sure and rupture. Massive hemorrhage and death can

result. Although cirrhosis with sinusoidal fibrosis is by
far the most common cause of portal hypertension in the
United States, other lesions can cause this condition. Portal vein thrombosis (or the thrombosis of a major component of the portal venous system, such as the splenic
vein) can result in shunting. Schistosomiasis infection is
the most common cause of portal hypertension worldwide. This parasite's eggs are deposited in the small
venules of the portal venous system within the liver
parenchyma but before the sinusoidal capillaries. Since
the hepatic parenchyma and synthetic function are preserved in patients with schistosomiasis, they tend to do
better even if their elevated portal pressure causes massive bleeding. The Budd-Chiari syndrome refers to the
obstruction of the hepatic vein. In this setting pressure is
transmitted back through the sinusoidal system and into
the portal system. This compromises blood flow into the
liver from both the portal vein and the hepatic artery.
Ischemia and metabolic failure can ensue and the liver
can become severely compromised. Chemotherapy and
bone marrow transplantation can cause clotting at the
level of the initial hepatic venules. The resultant condition mimics the Budd-Chiari syndrome in many ways.
The three main complications of portal hypertension
are hepatic encephalopathy, variceal bleeding, and
ascites. Hepatic encephalopathy (HE) is a clinical condition in which blood, shunted away from the liver and into
the systemic circulation, carries substances that would
normally be metabolized in the liver in greater concentration to the central nervous system (CNS). This results
in neuroinhibition of the CNS. Ammonia, benzodiazepine-like substances, y-aminobutyric acid, mercaptans, short chain fatty acids, and other substances have
been implicated in the pathogenesis of this condition, but
conclusive proof of the precise etiologic agent or agents
remains elusive. Clinically patients manifest with

increasing CNS inhibition such that they initially become
irritable and subsequently become more confused and
13
TABLE 1-2. Stages of hepatic encephalopathy

Stage 1: Restless, disruption of sleep/wake cycle, subtle
cognitive impairment, agitation
Stage 2: Cognitive impairment obvious, drowsy, asterixis
present, disorientation
Stage 3: Disorientation, slurred speech, somnolent but
arousable to stimuli
Stage 4: Deep coma, unarousable, potential airway
compromise
somnolent until frank coma ensues. The stages of "coma"

in hepatic encephalopathy are noted inTable 11. Hepatic
encephalopathy can be treated with bowel purging and
the acidification of the luminal contents, which lessens

the uptake of urea. Lactulose is commonly employed to
create an osmotic diarrhea and to acidify the gut contents.
Hypoglycemia, acid,/base disorders, electrolyte abnormalities, intercurrent illnesses and infections, hypoxia,
and sedating medications (especially benzodiazepines
and barbiturates) can induce clinical HE or exacerbate it.
These entities should be sought out and aggressively corrected when a patient presents with HE. Management of
variceal bleeding via shunting procedures as described
below will often exacerbate HE as more blood bypasses
the hepatic sinusoids.
Variceal bleeding is the most dramatic and imminently
life-threatening complication of portal hypertension, with
an overall mortality rate of 30o/o to 50%. It should be suspected in anyone with a massive bleed who has known
liver disease where it is often further exacerbated by concurrent coagulopathy. Gastroesophageal varices may
bleed massively, compromising the patient's airway and
causing hypotension and shock. Urgent intubation and
volume expanders, including blood, are required as a gastroenterologist is summoned to attempt variceal sclerosis
or banding. Direct variceal tamponade is one of two basic
ways in which the bleeding can be controlled. The other
method is the reduction of the portal venous system pressure. Massive bleeds naturally decompress the system but
may cause death before the bleeding stops. Vasoactive
substance such as vasopressin, nitroglycerin, octreotide,
and propranolol have been used to decrease portal blood
flow and pressure. Varices that are known to be present
but have not bled should be managed with nonselective
beta-blockade using propranolol, while every effort is
may to avoid hepatotoxins and mitigate any ongoing

hepatitis. Variceal bleeding should be managed directly
with endoscopic sclerotherapy or banding while additional pharmacologic agents such as octreotide or vasopressin with nitroglycerin are used to lessen portal pressure. Refractory bleeding may be controlled by placing
one of several specially designed intraluminal balloons
into the stomach and esophageal lumina. The balloons are
then inflated to tamponade the variceal bleeding. Radiologic shunts, known as transjugular intrahepatic portosystemic shunt stents (TIPSS), can be placed under fluoro-
14 /
EnrnncnNcy MnorcmE: TFrn Conr CunnrculuM
of diuretic
scopic control to decompress the portal system. Emergent
sists
referral for liver transplantation should be considered
adding loop diuretics as needed subsequently, in conjunction with strict dietary sodium restriction (as low as 500
mglday). Tense ascites, refractory ascites, and SBP can be
managed by therapeutic large-volume paracentesis with
the goal of removing as much fluid as possible. Salt-poor
when appropriate.
The third major complication of portal hypertension is
ascites. The increased pressure ofthe portal system forces
filtration of the blood plasma through the sinusoidal capillary walls, which have loose junctions and tend to be
"leaky." Cirrhotic livers have been observed to be "weeping" during laparoscopic visualization. This phenomenon
can occur in the gut venous system, as well,
if
portal
hypertension is presinusoidal. This fluid is relatively protein-poor and can serve as culture media for bacteria.
Spontaneous bacterial peritonitis (SBP) is likely due to
hematogenous contamination of this fluid although transmural contamination from the gut has been postulated.
Escherichia coli is the most common bacterial isolate,
and Streptococcus pneumoniqe is the second most often
identified. Antibiotics and the drainage of the ascites are
the means by which this infection is managed. Appropriate antibiotics include cephalosporins and penicillinase-
resistant penicillins, but isolate sensitivities should dictate specific therapy. Aminoglycosides may precipitate
renal insufficiency in cirrhotic patients and should be
avoided. Ascites causes restrictive lung disease at greater
volumes. It may lead to early satiety and anorexia due to
gastric compression. Rarely, tense ascites will lead to
umbilical rupture and rapid decompression. Ascites is an
example of "third-spacing" in which fluid collects within
the body but outside the vascular and interstitial compartments. It can have profound effects on the hemodynamics of the body and self-perpetuate. Ascites is not
exclusively the result of portal hypertension. Chronic
pancreatic fistulae, malignancies, and intestinal lymphatic obstruction are readily identifiable causes of free
intraperitoneal fl uid collection.
The first step in managing ascites is to ascertain the
cause. The history and physical examination are often
useful in suggesting the underlying etiology. A diagnostic
paracentesis should be performed to distinguish the cause
and rule out infection. The fluid should be sent for a cell
count and differential, an albumin level, and for culture
using direct inoculation of 10 ml of ascitic fluid into each
blood culture bottle directly at bedside. This technique
increases the culture sensitivity by approximately 40o/o to
50%.If the fluid is milky, it should be assayed for cholesterol. Ifthe patient has a history ofpancreatitis and/or
pseudocysts, an amylase level should be checked. Simultaneously a serum albumin level should be checked. An
albumin gradient is calculated by subtracting the ascites
albumin level from the serum albumin level. A gradient
of 1.1 or greater suggests a portal hypertensive etiology,
whereas a gradient less than 1.1 cautions the practitioner
to look for another cause. A low gradient and a history of
malignancy, especially
the fluid is cloudy, should
if
prompt the clinician to send the fluid for cytologic

review Management of portal hypertensive ascites con-
therapy using aldosterone first, and
albumin should be infused intravenously (25 g) at the

time of the paracentesis. Studies have shown improvement in response to paracentesis and the resultant hemodynamics with the use of salt-poor albumin. TIPS stents
may lessen portal pressure and reduce refractory ascites.
Transplantation provides definitive care for cirrhotic
ascites.
Hepatic/Hepatorenal Failure (1.2.3)

The hepatorenal syndrome (HRS) is another common,
and often deadly complication of end-stage liver disease.
Oliguria accompanied by azotemia and a rising BUN and
creatinine, a low urine sodium, and a "bland" urinalysis
in the face of serious liver disease suggest this syndrome.
HRS appears to result from disordered hemodynamics
and the decrease in effective arterial volume seen in cirrhosis. It can be triggered by therapeutic attempts at managing the ascites and edema resulting from portal hypertension. It may be triggered by the use of nephrotoxins as
well. Several theories exist to explain various aspects of
the HRS. Intrarenal prostaglandin inhibition has been

shown to decrease intrarenal blood flow, which is a final
pathway in the development of the qmdrome. Prostaglandin inhibition can cause or seriously exacerbate the hepatorenal syndrome and should be avoided. The use ofnon-
steroidal antiinflammatory drugs (NSAIDs) should be

avoided in all patients with cirrhosis or ascites of uncertain etiology. All patients with suspected HRS should be
admitted and a urine sodium should be checked. A low
urine sodium, especially if the patient had been receiving
diuretics, is suggestive. There are reports of improvement
in patients suffering from HRS with the placement of

TIPS stents, but the only routinely (albeit not always) successful intervention is orthotopic liver transplantation.
Kidneys from patients dying of HRS will function upon
transplantation if acute tubular necrosis has not supervened.
Tlrmors (1.2.4)
The liver is a common site for benign and malignant
neoplasia (both primary and metastatic). Asymptomatic
neoplasia is noted when radiologic studies such as ultrasounds or CAT scans are performed for abnormal LFTs
(detected as part of screening surveys if the patients are
asymptomatic) or for unrelated reasons that include a
liver window. Hepatocellular carcinoma (HCC) is a common malignant tumor worldwide. It results from cirrhosis
AsroN4rNrAL eNn GesrnorNTESTrNAr
of all kinds, chronic hepatitis (especially viral), the ingestion of aflatoxins and exposure to hepatotoxins, and
hepatopathies such as hemochromatosis. The diagnosis
requires histologic confirmation, although an elevated
alpha-fetoprotein is suggestive. Surgical resection of limited HCC affords the only real hope for cure. Segmental
resection or orthotopic transplantation may help unless
the patient already has symptoms, metastases, or bulky
disease. HCC is resistant to radiotherapy and chemotherapy. Liver tumors present with pain if they grow big
enough to distort Glisson's capsule. They can trigger
fever and this may prompt the patient to seek medical
attention. Malignant tumors often produce anorexia and
wasting, even if small. Strategic placement within the
parenchyma may cause biliary obstruction with jaundice.
Massive infiltration by primary or metastatic disease

might lead to frank liver failure, and very large tumors
(both benign and malignant) can rupture leading to pain,
hypotension from hemorrhage, and shock. Referral for
further evaluation and management is the general rule for
liver masses detected in the ED unless a related complication provokes a crisis.
DsonnnRs
75
droemetine are effective against E. histolytica. Pyogenic

abscesses have a 25Yo or greater mortality rate, while
uncomplicated amebic abscesses are lethal approximately
l%o of the time. If the amebic abscess ruptures and contaminates the pericardium (extension from the left lobe of
the liver), the mortality rate may exceed 25oh.
SELECTED READING
Chiou SS, Changchien CS. Management ofend-stage liver disease. Tiznsp I ant P ro
993 ;25 (5):29 48-29 52.
Fregia A, Jensen DM. Evaluation

1994;20(r):50-54
of abnormal liver tests Contp
Ther
Hirschman SZ. Current therapeutic approaches to vital hepatitis. Clin Inf

Dis 1995;20:741-746.
Kirsh BM, Lam N, Layden TJ, Wiley TE. Diagnosis and managetnent of
fulminant hepatic failure. Comp Ther 1995;21(4):166 17l.
Morgan MY The treatment of chronic hepatic encephalopathy. Hepatctgctstroe ntero I ogv1'99
:38:37 7 -387
Laffi G, La Villa G, Gentilini P Pathogenesis and management of the hepatorenal syndrome Semin Liver Dis 1994;14(1):71-tll
Nizam R, Buniak B, Carson JL. Drug-induced hepatic disorders Pharmucol Ther 1996;2 I (I): 16, 23 26, 29-33.
Pagliaro L, D'Amico G, Luca A, et al. Portal hypertension: diagnosis and
treatment. J Hepatol 1995;23(suppl l):3644.
Reddy KR, Schiff ER. Approach to a liver tnass. Sarrtln Liver Dis 1993;
13(4):42343s
Runyon B. Care of patients with ascites. N Engl J Med 1994;330(5):
33'7-342.
Abscess (1.2.5)
Hepatic abscesses are rare; they are seen in up to

0.54% of autopsies and at half that rate in hospitalized
patients. Fever, leukocytosis, abdominal pain (if there is
distortion of Glisson's capsule), or concomitant biliary
obstruction, associated with abnormal LFTs, suggests the
diagnosis. Cholangitis, cholecystitis, and tumors, however, are far more common causes for these presenting
symptoms and signs. Ultrasound and CAI scan will suggest a space-occupying lesion, and tissue densities might
suggest that it is not solid. Percutaneous or open drainage
will confirm the microbiology and is very important in
the management. Smaller or multifocal abscesses may be
treated with pharmacotherapy alone, but larger abscesses
will require drainage. Pyogenic abscesses account for
90% of cases and often are caused by E. coli, various
staphylococcal species, various streptococcal species,

and anaerobes. Biliary tract infections, intraabdominal
infections (e.g., appendicitis), and hematogenous spread
are cofilmon sources, although approximately one in five
cases remains cryptogenic. Broad-spectrum antibiotics
should be employed in addition to drainage. Amebic
abscesses occur in approximately l0% of patients with
liver abscesses. Entamoeba histolytica causes abscesses
with less symptoms of inflammation than pyogenic types.
They are more common in areas where human feces is
used as fertilizer or in institutions such as facilities for the
mentally handicapped. Drainage of an amebic abscess
should be undertaken ifit appears large enough to rupture
or when a pyogenic abscess is suspected. Antiamebic
therapy with metronidazole, chloroquine, and dyhy-
Vukmir RB. Pyogenic liver abscesses. Am Fam Physician 1993;47(6):

1435-1441.
GALLBLADDER AND BILIARY TRACT (1.3)

Acute diseases of the biliary tract, most notably the
gallbladder and biliary tree, are responsible for a large
number of ED visits. The majority of patients present
with acute cholecystitis, which is inflammation or infection of the gallbladder, or biliary colic, which is pain due
to temporary obstruction of the common bile duct. Both
of these entities are most commonly caused by cholelithiasis. It is estimated that approximalely 20 million Americans have gallstones. Less common, but serious diseases
of the biliary tract include ascending cholangitis and gallstone ileus. Carcinomas of the biliary tract are much less
common, with poor 5-year survival rates ranging from
2oh to 5o/o.
Cholecystitis (1.3.1)
Acute inflammation of the gallbladder is one of the
most frequent causes of abdominal emergencies. Approx-
imately 900/o of cases of acute cholecystitis are caused by

gallstone disease. Acalculous cholecystitis accounts for
only approximately 5Yo of cases. Risk factors include
increasing age, female gender, fertility, and obesity.
Pathophysiology
In most cases of acute cholecystitis, the initiating factor is obstruction ofthe neck ofthe gallbladder or the cys-
16 /
ElrnRcrNcy MrucrNr:
Tsr Conr Cunnlculuu
tic duct by a gallstone. This obstruction causes increased

secretion of fluid from the glands within the gallbladder
wall. The resultant increase in hydrostatic pressure causes
distention of the gallbladder and subsequent ischemia of
the wall. Inflammation may also be attributed to the cytotoxic constituents of the bile itself including phospholipase and its conversion to lysolecithin. Ensuing infection
can serve
to
worsen the preexisting inflammatory
process. Bacteria are only isolated in 50Yo to 75oh of

cases, most commonly gram-negative rods (E. coli) and
anaerobes (Bacteroides fragilis). This bacterial invasion
occurs either from blood-borne organisms or from bacteria ascending from the duodenum via the common bile
duct.
The pathogenesis of acalculous cholecystitis remains a
mystery. Patients most at risk include those with diabetes,
chronic diseases, sepsis, burns, or multiple system failure. Contributory factors implicated in acalculous cholecystitis include fasting, dehydration, fevers, and possibly
antibiotic therapy.
Presentation
The most common presenting complaint in patients

with acute cholecystitis is right upper quadrant or epigastric pain. This pain may initially be colicky in nature, but
eventually becomes constant. Patients may report a prior
history of similar pain that had resolved spontaneously.
These episodes of pain may be in direct relationship to
the consumption of a heavy meal or fatty foods, or to the
use of a narcotic analgesia, which causes spasm of the
sphincter of Oddi. Radiation of the pain is usually to the
tip of the right scapula. Nausea, vomiting, and fevers may
be present. Significant physical findings include temperature elevation, tachycardia, tenderness in the right upper
quadrant most notably with deep inspiration (Murphy's
sign), and possibly evidence
of peritonitis
including
guarding and rebound tenderness. The dilated gallbladder

may be palpable in30% of patients. Evidence ofjaundice
may indicate a stone in the common bile duct, but may be
present in acute cholecystitis as well.
Ancillary
Tests
There are no truly diagnostic laboratory studies for

cholecystitis. Leukocytosis with evidence of a left shift
on differential is common. However, extremely high
white blood cell counts may be due to complications such
as cholangitis, gangrene, or perforation. Liver function
tests such as serum aminotransferase and alkaline phosphatase may be within normal limits or slightly elevated.
Higher elevations of aminotransferases and total bilirubin
are suggestive of a common bile duct stone. An elevated
amylase is indicative of acute pancreatitis as a diagnosis
either solely or concurrent with acute cholecystitis.
Abdominal x-ray may show evidence of localized small

bowel loop dilatation in the area of the gallbladder (a sentinel loop) indicating an ileus.
Ultrasonography has now become the radiologic test of
choice for acute cholecystitis in the emergency setting.
The accuracy of ultrasonography approaches 100% for

the detection of gallstones. The presence of gallstones,
thickened gallbladder wall, and pericholecystic fluid has
positive predictive value of 90%. Evidence of other disofthe liver, kidney, and pancreas,
may be detected as well.
Nuclear scintigraphy with 99m-technetium-labeled
hepatobiliary inodiacetic acid (HIDA) is generally now
considered the gold standard for the detection of acute
cholecystitis. The isotope is taken up by hepatocytes and
is secreted into the bile canaliculi allowing outline of the
gallbladder within approximately t hour. Failure to visualize the gallbladder indicates cystic duct obstruction and
is considered a positive test. Visualization of both the
gallbladder and common duct is a negative test with a
negative predictive value of 98%. False positives may
occur in certain conditions such as fasting for extensive
periods, total parenteral nutrition, chronic cholecystitis,
narcotic use, and congenital absence of the gallbladder.
For patients with total bilirubins greater than 5 to 7 when
the sensitivity of HIDA scanning declines, the use of
diisopropyl IDA (DISIDA) allows the visualization of the
a
eases, including those
biliary tree.
Dffirential Diagnosis
Other considerations in patients suspicious for acute
cholecystitis include appendicitis, hepatitis, hepatic
abscess, right lower lobe pneumonia, myocardial
ischemia, peptic ulcer disease, pancreatitis, pyelonephridiseases, ovarian disease, and
ectopic pregnancy. The literature suggests a misdiagnosis
rate of approximately 20%o when relying on clinical diagnosis alone.
tis or other acute renal
Management
Most treatment in the ED is supportive and symptom

oriented. Aggressive treatment of dehydration with crystalloids is warranted. Nausea and vomiting usually can be
relieved with antiemetics and possibly nasogastric suctioning. It is possible that keeping the stomach empty
with nasogastric suctioning may diminish gallbladder
stimulation. Prompt surgical consult is warranted. Narcotics are effective in controlling the pain of acute cholecystitis. Meperidine is preferred over morphine since the
latter may cause spasm of the sphincter of Oddi. Even
though their use is questionable, antimicrobials are still
recommended. Coverage with a broad-spectrum second-
or third-generation
cephalosporin
is usually
adequate
Asno\arNAr AND GASTRoINTESTINAT DtsoRor,Rs

unless sepsis is suspected. In the face ofsepsis, the use
of
a triple antibiotic regimen such as ampicillin, clindamycin, and gentamicin is warranted. Approximately
of patients with acute calculous cholecystitis will

of symptoms with medical management
alone. The timing of surgery for acute cholecystitis
remains somewhat controversial. The onset of laparoscopic cholecystectomy has made it advantageous to put
off surgery until the gallbladder inflammation has
75o/o
have resolution
decreased or resolved, making laparoscopic surgery pos-
sible. Allowing a "cooling off" period also provides for

the medical stabilization of acutely ill patients. Immediate
cholecystectomy is usually reserved for patients with evidence ofcomplications such as gangrene or perforation.
Disposition
Patients who are afebrile with relatively normal liver
function tests and white blood cell counts and whose pain
subsides with narcotic analgesia are thought to have biliary colic. These patients can be discharged with pain
medication and scheduled for outpatient workup. They
should be advised to return immediately for persistent
pain or fevers. All patients diagnosed with acute cholecystitis should be admitted and have inpatient evaluations
and treatment. If there is evidence of complications such
as gangrene or perforation, the patient should have early
surgical intervention.
Cholangitis (1.3.2)
Infection within the biliary duct system, cholangitis,
has been reported to occur in 8% of patients admitted for
biliary tract disease. Cholangitis was first described by

Charcot in 1877. Most often it is associated with choledocholithiasis, which is the obstruction of the common
bile duct by gallstones. This process is almost exclusively
a disease
ofthe elderly.
Pathophysiology
The obstruction of the common bile duct either by gallstones, malignancy, or benign stricture leads to increased
intraluminal pressure and bacterial infection. Often,
incomplete obstruction is seen in cholangitis. Bacteria
may seed the common duct either by retrograde invasion
from the duodenum, via blood from the portal vein, or
through the lymphatic system. The most common
pathogens include E. coli, Klebsiella, Bacteroides, and
Enterococcus.
Presentation
Right upper quadrant abdominal pain, fever, and jaundice (Charcot's triad) characterize cholangitis. More
17
severe cases may be associated with altered sensorium

and hypotension, completing Reynold's pentad. Other
symptoms and signs include nausea, vomiting, tachycardia, and tachypnea. Sepsis is a common complication of
cholangitis. When evaluating these patients, one must
always keep in mind cholecystitis and hepatitis since their
presentations are similar.
Ancillary
Tests
Leukocytosis with a left shift is common; however,

depression of the white blood cell count may be seen in
cases with evidence of sepsis. Hyperbilirubinemia and an
elevation of alkaline phosphatase are often seen. Serum
aminotransferases may be mildly elevated. If acidosis is a
concern, arterial blood gases should be obtained. On
occasion, abdominal x-rays may reveal air in the biliary
tree. Ultrasonography may demonstrate stones within the
gallbladder or biliary tree and possibly common bile ductal or intrahepatic ductal dilatation. Lack ofvisualization
during nuclear scintigraphy may be useful to diagnose
common duct obstruction early on in the disease process.
Diagnostic alternatives include computed tomography
(CT) scanning, percutaneous transhepatic cholangiography (THC), and endoscopic retrograde cholangiopancreatography (ERCP).
Management
Stabilization of the patient from
hemodynamic stand-
point should take priority. Fluid resuscitation with crystalloid is usually necessary to stabilize blood pressure. In
the septic patient, vasopressors may be necessary. Blood
cultures should be obtained immediately and coverage
with broad-spectrum antibiotics should be initiated as
soon as possible. Usual antibiotic recommendations
include ampicillin, aminoglycosides, and clindamycin or
metronidazole. An alternative to triple antibiotic coverage
is mezlocillin since it is actively secreted into the biliary
tree and covers gram-positive cocci, gram negatives, and
anaerobes. Early surgical consultation is a must. Definitive therapy is to drain the biliary tree either by surgical,
transcutaneous, or endoscopic means. In patients in
whom the diagnosis is missed initially, mortality rates are
as high as 30oh to 40o/o.
Cholelithiasis and Choledocholithiasis (1.3.3)

Gallstones are present in approximately 20o/o of
women and 8oh of men. The presence of common duct
stones (choledocholithiasis) in acute and chronic cholecystitis is approximately 7o/o to l5o/o. Approximately
500,000 operations are performed per year due to the
complications of cholelithiasis. The most common presentation of cholelithiasis is biliary colic. The differential
18 /
Err,rnncnNcv
MsnrcrNl: Tns CoRE Cunnrculurr,r
diagnosis of biliary colic includes acute cholecystitis,

peptic ulcer disease, pancreatitis, hepatitis, appendicitis,
pneumonia, pyelonephritis, renal colic, diseases of the
ovary fallopian tubes, and myocardial ischemia.
differentiation of acute cholecystitis from biliary colic.

Serum aminotransferases are important to obtain to help
differentiate between this disease entity and hepatitis.
Bilirubin levels will help detect the presence of a common bile duct stone. Elevations of amylase and lipase
Pathophysiology
may indicate pancreatitis.

Plain radiographs provide little clinical information
since only l0%o of stones are radiopaque. Ultrasonography is highly accurate for the detection of gallstones
(approaching 100%). Detection of dilated intrahepatic
and common bile ducts is also possible with ultrasonography. Oral cholecystography is used to detect gallstones
in the highly clinically suspicious patient when ultra-
There are two types of gallstones-cholesterol and

pigmented. Cholesterol stones are formed when there is a
high concentration ofcholesterol in the bile. Cholesterol
crystals precipitate when the cholesterol concentration
exceeds the ability of the bile acids and lecithin to reduce
it. These crystals form a nidus for stone formation. Risk
factors include increased age, female gender, obesity,
multiparity, rapid weight loss, cystic fibrosis, family history, and drugs such as clofibrate and oral contraceptives.
There are two types of pigmented stones-black and
brown. Black stones are formed in the gallbladder and
of calcium bilirubinate. These

are most often seen in the elderly and those
contain a large amount

stones
patients with sickle cell anemia and hereditary spherocytosis. Brown stones can form either in the gallbladder or
the intra- or extrahepatic biliary system and are frequently associated with infection. Bacterial as well as
parasitic infections such as Ascaris lumbricoides and
Clonorchis sinensis have been incriminated.
Both black and brown stones contain calcium bilirubinate. Approximately 4Yo calcium by weight is needed for
a stone to be radiopaque.
sonography is negative.
Management
The management of biliary colic is symptomatic relief.
Dehydration is treated using IV crystalloids. Nausea and
vomiting can be relieved by antiemetics and nasogastric
suctioning if necessary. Pain is best treated with narcotic
analgesia, especially meperidine, to limit the effects on
the sphincter of Oddi. When patients are asymptomatic,
they may be discharged with a prescription of narcotics to
be used for similar episodes. Surgical evaluation or referral is necessary for further workup and intervention.
Removal of the gallbladder by surgical means is the most
common intervention; however, extracorporeal shock

wave lithotripsy and oral administration of bile acids have
been proven to have some effectiveness.
Presentation
Gallstone Ileus (1.3.4)
Episodes of biliary colic are frequent in the ED. These
episodes may be caused by either the migration of small
stones through the common bile duct or temporary lodging oflarger stones in the neck ofthe gallbladder during
gallbladder contraction. The pain may be colicky or constant, located anywhere in the upper abdomen but most
commonly in the right upper quadrant or epigastrium.
This pain may radiate to the tip of the right scapula. Nausea and vomiting may accompany the pain and may
become severe enough to cause dehydration. The patient
may relate a history of previous episodeg of similar pain
and an association to the consumption of fatty foods or
heavy meals. If a patient has had previous cholecystectomy, retained common duct stones should be suspected.
Physical findings are often limited. Right upper quadrant or epigastric tenderness is often present. There may
be evidence of dehydration such as tachycardia and
orthostasis. Rarely, jaundice may be encountered.
Ancillary
Tests
Most commonly obtained laboratory evaluations
are
usually within normal limits. Leukocytosis may aid in the
An unusual complication of cholelithiasis is gallstone

ileus. This entity is most frequently seen in older women
(average age 64). There is a past history of known
cholelithiasis in 50Yo to 75Yo. Gallstone ileus is responsible for lYoto2%o ofmechanical small bowel obstructions.
Commonly associated diseases include major cardiovascular disorders and diabetes mellitus.
Pathophysiology
Adhesions between the gallbladder and intestinal

tract, usually the duodenum, begin the process. Eventually a fistula forms, allowing the passage of gallstones
from the gallbladder into the intestinal tract.
These
stones are usually single and larger than 2 to 3 cm. This

stone then can form an intraluminal obstruction. Fistulous formation may be the result of prior surgery. The
formation of a fistula is not always to the duodenum but
may be to any organ of the gastrointestinal tract from the
stomach to the colon, pleural or pericardial cavities, tracheobronchial tree, pregnant uterus, ovarian cyst, renal
pelvis, or ureter.
AsooN4rNAL AND GASTRoTNTESTTNAL
Presentstion
Symptoms of acute cholecystitis immediately prior to
gallstone ileus occur
in
one-fourth
to
one-third of
patients. These symptoms are then followed by symptoms
of
intestinal obstruction, nausea, vomiting, crampy
abdominal pain, and distention.
Ancillary
Tests
The most helpful test in this disease is a radiologic

examination of the abdomen. Plain abdominal x-ray may
demonstrate evidence of small bowel obstruction or air
within the biliary tree. A stone within the gastrointestinal
tract may also be seen. Electrolytes and renal function
tests are helpful in evaluation of fluid and electrolyte
losses from vomiting and accumulation of fluid in and
around inflamed tissues.
Management
Resuscitation of the patient is the first priority. The
administration of IV crystalloid to replace fluid losses
and relieve dehydration is necessary. Nasogastric suction-
ing to relieve gastric distention and vomiting will be

required. Antibiotic therapy is usually recommended with
the choices being similar to those in cholangitis. Early
surgical consultation should be obtained. Surgical
removal of the stone and a search for other stones in the
gastrointestinal tract will be necessary. The differential
diagnosis is that of other causes of small bowel obstruc-
tion including adhesions, hernias, and cancer.
DsoRlans
19
of jaundice, weight loss, pruritis, and abdominal pain.

Palpation of the gallbladder may be possible in one-third
of cases. Serum bilirubin is often elevated to extremely
high levels. Ultrasonography may show dilated intrahepatic ducts. ERCP may be diagnostic, but THC is associated with the highest yield. Surgical intervention may be
curative or palliative. Five-year survival rates are approximately 2o/o to 5oh.
Carcinoma of the ampulla of Vater may be the result of
tumor extension from elsewhere or a primary site of sarcomas, carcinoid tumors, or adenocarcinomas. Obstruc-
tive jaundice is the usual presenting symptom and

appears early in the course of the disease. Ultrasonography may be helpful, but ERCP is usually necessary for
diagnosis. Wide surgical excision is required for cure.
Due to early detection, 5-year survival rates range from
32o/o to 620/o.
The emergency physician should be highly suspicious
of carcinoma in any patient who presents with rapid onset
ofjaundice, weight loss, and abdominal pain. Beginning
the workup of these patients with an ultrasound or CT
scan is reasonable. However, for definitive diagnosis,

urgent referral to a gastroenterologist for ERCP or THC
will be required.
SELECTED READING
Babb R. Acute acalculous cholecystitis.
J Clin Gastroenterol 1992;15(3):
238,24t.
Harwood-Nuss AL, Linden CH, Luten RC, et al. Acute diseases of the gallbladder. In: The clinical practice of emergency medicine. Philadelphia:
Lippincott, 1991;131-132.
Kadakia SC. Biliary tract emergencies. Med Clin North Am 1993;77(5):
101 5-1 036.
T! et al. Nuclear hepatobiliary imaging. Radiol Clin North

Am 1 993 ;3 I (4):923-933.
May HL, Aghababian RY Fleisher GR, et al. Acute cholecystitis. In'. Emergency medicine,2nd ed. Boston: Little, Brown, 1992;1477-1479'
Rosen P, Barkin R, et al. Disorders ofthe liver, biliary tract, and pancreas.
In: Emergency medicine: concepts and clinical practice, 3rd ed. 1992;
Kim EE, Moon
Tumors (1.3.5)
Gallbladder carcinoma is the fifth most common type
of carcinoma involving the gastrointestinal tract and
accounts for 5Yo of cancers found at autopsy. It affects
predominantly older women. Approximately 90oh of

patients have had a history of cholelithiasis. Adenocarcinoma accounts for 80% of the tumors while the remainder are undifferentiated or squamous cell. The presenting
symptoms are usually indistinguishable from those of
cholecystitis and cholelithiasis. About one-half of the

patients appear jaundiced and there is a palpable right
upper quadrant mass in two-thirds. Both ultrasound and
CT scanning can be used to image the tumor; however,
they fail to identifu tumor in 49olo of cases. Surgical intervention offers only a small hope for cure. Five-year survival rates are only 2o/owith9}Yo of patients dying before
the end
of
1 year.
Carcinoma of the extrahepatic ducts has an average

autopsy incidence of 0.3Yo. This tumor type occurs more
frequently in men. All lesions are histologically adenocarcinomas. Characteristic symptoms include rapid onset
t60l-1626.
Schwartz S, Shires G, Spencer F, et al. Gallbladder and extrahepatic biliary
system. In: Principles ofsurgery,5th ed. 1989;1381-1412.
Tintinalli J, Ruiz E, Krome R, et al. Cholecystitis and biliary colic. In:
Emergency medicine: a comprehensive study guide,4th ed. New York:
McGraw-Hill, 1996;495497.
Wilson J, et al. Diseases of the gallbladder and bile ducts' In: Harrison's
principles of internal medicine, 12th ed. NewYork: McGraw-Hill, 1988;
1358-1368.
PANCREAS (1.4)
Lying in the retroperitoneum, the pancreas is an organ
that serves multiple functions. As an endocrine organ, the
pancreas supplies insulin, glucagon, and somatostatin.
These products are required to maintain glucose homeostasis. Amylase, lipase, bicarbonate, trypsin, chymotrypsin, elastase, carboxypeptidase, and phospholipase
are some of the exocrine products provided by the pancreas. These products'primary role is to neutralize gastric
20 /
EuencrNcv MnlrcrNr,: Trtn Conr Cunnrculurrr
acid and aid in digestion of proteins, fats, and carbohydrates. The pancreas can be afflicted with multiple disease
processes. Acute and chronic pancreatitis are responsible
for many ED visits. Complications of those disease entities include pseudocysts and abscesses, which are occasionally seen in the emergency setting. Pancreatic insufficiency is one of the most common causes of maldigestion
and can be caused by chronic pancreatitis, cystic fibrosis,
TABLE
1-4.
Drugs associated with

pancreatitis
Proven
Azathioprine
Cisplatin
Estrogens
Furemide
Tetracycline
Thiazides
Possible
Acetominophen
Carbamazepine
Clonidine
Cimetidine
Enalopril
lndomethacin
lsoniazid
Metronidazole
Opiates
Procainamide
Rifampin
Salicylates
Valproic acid
and pancreatic carcinoma, or may be seen after pancreatic
or gastric resection. Pancreatic carcinoma often makes its
initial presentation in the ED with weight loss and pain. It

is important for the emergency physician to recognize the
signs and symptoms of pancreatic disease and provide
appropriate intervention and referral.
Inflammatory (1.4.1)
Acute Pancreutitis (1.4. 1. 1)
Pancreatitis can be of two types, acute and chronic.
Acute pancreatitis is present in approximately 0.5Yo of
the population of the United States. The incidence of pan-
creatitis is increasing in certain areas over the last 20

years. This may be partly due to improved diagnosis or
perhaps to the prevalence of alcohol abuse. Usually a
mild self-limiting disease, acute pancreatitis can also
become a life-threatening illness when it deteriorates into
hemorrhagic
or necrotic pancreatitis. Mortality
rates
reach as high as 5%.
Pathophysiology
There are many theories as to the mechanism for the
development of acute pancreatitis. The principal concept
TABLE 1-3. Etiologies of acute pancreatitis
Alcohol
Biliary tract disease (choledocholithiasis, carcinomas)
Hyperlipoproteinemias
Hypercalcemia
Drugs
Posttraumatic
Postoperative
Post-ERCP
Pregnancy
Penetrating peptic ulcer
Carcinoma of the pancreas
Scorpion bites
Vasculitis
lnfectious
Mumps
Coxsackie virus
Mycoplasma
Legionella
Campylobacter
Hepatitis B virus
Ascariasis
of these theories is the autodigestion of the pancreas by

inappropriate activation of proteolytic enzymes such as
trypsin. This autodigestion results in coagulation necrosis
and vascular injury causing hemorrhage, edema, and
pain. Other mechanistic theories include the reflux of bile
or
duodenal contents, bacterial infection, and ductal
hypertension.
The list of possible or known etiologies of acute pancreatitis is quite extensive (Table 1-3). In the United
States, acute pancreatitis can be attributed to cholelithiasis and alcohol in 80% to90Yo of patients. Usually 5 to 10
years ofchronic alcohol ingestion is necessary to develop
acute pancreatitis. However, there have been cases of
acute pancreatitis after sudden binge drinking in the nonalcoholic.
Drugs are also well-known contributors to pancreatitis
(Table l-4). Tetracycline, thiazides, furosemide, cisplatin, and azathioprine arejust a few ofthe drugs definitively shown to cause acute pancreatitis.
Presentation
Constant midepigastric pain accompanied by nausea

and vomiting is the usual presentation of acute pancreati-
tis. Radiation of pain to the midback is common. A history ofgallstones in the past, alcohol use, or pancreatitis-
is also helpful in making the

diagnosis.
Patients with acute pancreatitis often present in acute
distress. Tachypnea and tachycardia may be present as
well as signs of orthostasis. Abdominal tenderness in the
epigastrium is the norm; guarding is commonly present.
Loss of bowel sounds is indicative of an associated ileus.
related drug therapy
AsnoNarNAL AND GASTRoTNTESTTNAT DrsoRonns

Grey Tirrner's sign, ecchymosis around the umbilicus, and
Cullen's sign, ecchymosis in the flanks, are indicative of
retroperitoneal blood consistent with hemorrhagic pancreatitis.
Ancillary
Tests
Elevations of serum amylase and lipase are usually

indicative of acute pancreatitis. Elevation of serum amylase carries a reported sensitivity of 95%. Amylase levels may remain elevated for up to 72 hours after the
onset of acute pancreatitis. However, other conditions
such as perforated ulcer, acute cholecystitis, intestinal
obstruction, mesenteric veinous thrombosis, ruptured
ectopic pregnancy, renal insufficiency, or the use ofopiates can also cause hyperamylasemia. This myriad of
conditions associated with hyperamylasemia creates
poor specificity for this test. Conversely, up to one-third
of patients with clinical pancreatitis may have normal
amylase levels.
The advent of the ability to test serum lipase has

increased diagnostic accuracy for pancreatitis. Lipase is
found primarily in the pancreas. It has a sensitivity similar to amylase but a specificity nearing 100%. The degree
of elevation of amylase and lipase has no clinical correlation to the severity of disease. The highest elevations are
commonly seen in gallstone pancreatitis.
Ranson has outlined criteria for predicting the severity
and mortality of acute pancreatitis (Table l-5). Mortality
using the Ranson criteria is based on the number of prog-
nostic signs present: 0-2, l%o;34, l5Vo; 5-6,40%o; and

greater than 6, 100%.
There are a variety of radiologic imaging modalities
available for diagnostic purposes, but most have a lim-
ited role in acute pancreatitis. Plain abdominal x-ray

may show calcifications in the epigastric area suggestive of chronic pancreatitis or localized evidence of
ileus (sentinel loop) suggestive of acute pancreatitis.
Upright chest x-ray may show free intraperitoneal air
associated with perforated viscous. Ultrasonography in
the majority of patients will either be normal or show
an enlarged hypoechoic gland. Pseudocysts, abscesses,
TABLE
1-5.
Ranson criteria for prognosis in acute
pancreatitis
On admission
ln 48 hours
Age > 55 years

Serum glucose > 2O0 mg7"
WBC > 16,000/mm3
LDH > 350 IU/L
SGOT > 250 SF units
Fluid sequestration > 6 L

pOz < 60 mm Hg
Hematocrit fall > 10%
Calcium < 8 mg%
Basedeficit>4mEq/L
BUNrise>5mg/dl
Adapted from Ranson JHC. Etiologic and prognostic factors in human acute pancreatitis: a review. Am J Gastroenterol 1982:77:663.
27
phlegmons, and hemorrhages may be identified by

ultrasound. Gallstones are also reliably detected by
ultrasonography. CT scanning can identify the pancreas
easily but has a limited role in acute pancreatitis. Its
most useful application is for the demonstration of
inflammation in the surrounding tissues, phlegmons,
abscesses, pseudocysts, and hemorrhage. Ideally CT
scanning should be performed only when complications
are suspected.
The differential diagnosis in acute pancreatitis is that
of the acute abdomen, including cardiopulmonary diseases, intraabdominal processes, and renal or gynecologic disease. Differentiation is possible using good hisancillary
tory and examination skills as well as

evaluations.
Management
The management of acute pancreatitis is both supportive and expectant. Most patients present with dehydration and will require fluid resuscitation. Pancreatitis
is a burn to the retroperitoneal space and can go on to
large amounts of fluid sequestration. Therefore, the
insertion of multiple large-bore IV catheters or a central
line may become necessary. Nasogastric aspiration is
controversial. It may be helpful in limiting emesis, providing gastric and proximal duodenal decompression,
and decreasing stimulation of the pancreas by gastric
contents. Pain control with narcotics such as meperidine
is usually necessary. Meperidine is preferred over morphine to limit induced spasm of the sphincter of Oddi.
An antiemetic may be helpful as well for symptomatic

relief. Insertion of a Foley catheter is helpful in monitoring urine output.
Vigilance in the search for complications can help
decrease morbidity. Electrolytes including calcium and
magnesium should be closely monitored. Arterial blood
gases should be monitored to detect decreasing PaO2.
Hypoglycemia needs to be detected and rapidly
addressed. Hypotension either due to fluid sequestration
or hemorrhage needs to be aggressively managed. A
falling hematocrit andlor Grey Tirrner's and Cullen's
signs indicate the possibility of hemorrhage. Transfusion may become necessary. Broad-spectrum antibiotics
should be reserved for those patients with evidence of

biliary sepsis. Peritoneal lavage has not been proven to
be effective.
Surgical intervention is usually reserved for those

patients with acute decompensation. Potentially surgically correctable problems include abscess, secondary
infection of necrotic material requiring debridement, or
hemorrhage into a pseudocyst.
22 /
EunncrNcy MslrcrNn: Tun Conr CunnrculuM
Disposition
The vast majority of patients with acute pancreatitis
require admission to the hospital for management, some
even to the intensive care unit. Only a select few with
very mild disease and the ability to tolerate oral intake
may be discharged, with close follow-up arranged. The
majority of these patients have evidence of chronic pancreatitis. These patients should be kept on clear liquids
initially and advised to return immediately for worsening
pain, fevers, and the inability to take liquids orally.
C hronic
Pancreatitis (1.4. 1. 2)
Pathophysiology
Chronic pancreatitis is a result ofrepeated episodes
acute pancreatitis.
of
Initially the diagnosis may be on clin-
ical grounds rather than through pathologic changes. His-
tologically, lobules of functional acinar tissue and islet

cells are surrounded by fibrous tissue. In the late stages,
calcifications may occur. The primary cause of chronic
pancreatitis appears to be alcoholism; however, other
entities such as pancreas divisum, protein malnutrition,
and hyperparathyroidism have been incriminated. Gallstones on occasion do lead to recurrent pancreatitis but
do not seem to contribute to chronic pancreatitis.
The typical presentation is that of an alcoholic patient

who has had repeated presentations with acute pancreatitis. This patient generally complains of severe epigastric
abdominal pain that may be similar to that experienced
during acute disease. Pain may radiate to the back and
may be partially relieved by sitting forward. The pain may
change from intermittent to constant. There may be evi-
of
pancreatic insufficiency, steatorrhea, weight

loss, and polyuria. Nausea and vomiting may occur if this
attack has been precipitated by a bout of alcoholism.
Physical examination generally reveals a chronically
ill-appearing patient who is malnourished and in moderate to severe distress due to pain. Tenderness in the midepigastric arca may be elicited; however, pain out of pro-
portion
to the exam is
pathognomonic. Pancreatic ductal dilitation may be

seen on CT or ultrasound. In general, endoscopic retrograde cholangiopancreatography (ERCP) is the most
reliable diagnostic tool. This readily detects abnormalities on the pancreatic ductal system. Testing for pancreatic secretory insufficiency does not have a role in the
ED setting.
Management
Treatment of chronic pancreatitis is generally symptomatic and supportive. Hydration with crystalloids is often
necessary, especially in the patient with evidence of dehydration or orthostasis. Vomiting should be treated with
antiemetics. Pain control is generally achieved with narcotics such as meperidine; however, ketorolac may be
useful in the patient with no evidence of gastrointestinal
hemorrhage or history of peptic ulcer disease. Malabsorption can be treated with pancreatic extracts. Alcohol
should be discontinued in order to facilitate recovery. The
clinically stable, chronic patient who is able to achieve
pain control with oral medications and take oral hydration may be discharged. For patients with severe bouts
similar to acute attacks, admission may be necessary.
Pseudocyst and Abscess (1.4.
Presentation
dence
Plain abdominal x-rays may reveal calcifications in

the epigastric area. These calcifications are considered
usual. Other evidence of
dehydration such as dry mucous membranes, tachycardia,

and orthostasis may be present.
Ancillary ksts
Laboratory evaluation may be unrevealing. Elevations
ofamylase and lipase are unusual as the pancreas has virtually stopped their production due to scarring. Hyperglycemia is a late finding and is usually accompanied by
other evidence of pancreatic insufficiency.
1,
3)
Pseudocyst
Pathophysiology. Approximately three-fourths of all

cystic lesions of the pancreas are pseudocysts. Pseudocysts are so named because they are composed of a
fibrous cavity filled with pancreatic debris and secretory
products. Most are a result of destruction of the pancreatic ductal system and are unilocular. They do not have an
epithelial lining as do true cysts. They are a common

complication of acute pancreatitis but have been encountered in l}Yo of patients with chronic pancreatitis.
Trauma accounts for 25%o. Pseudocysts usually appear in
the fourth or fifth decade and are more common in men.
Many pseudocysts will resorb spontaneously, while a few
will persist and grow to large dimensions, causing complications to surrounding organs.
Presentation The typical presentation of patients with
pseudocysts is pain, fever, and ileus 2 to 3 weeks after
acute pancreatitis or trauma. The pain is usually in the
epigastrium or left upper quadrant and may radiate to the
back. Nausea and vomiting may also be present. The

patient has often had frequent attacks ofacute pancreatitis. The patient may also present due to symptoms related
to complications such as intestinal obstruction, infection,
or hemorrhage into the pseudocyst,

migration of the pseudocyst.
or
intrathoracic
AsooNdrNAL AND GASTRoTNTESTTNAL DrsononRs
Physical examination reveals a nontender or slightly

tender abdominal mass in 75%o of patients. The mass may
fluctuate in size over time as well as completely disappear. The mass may occasionally be confused with an
abdominal aortic aneurysm. Hemodynamic instability

may be present in the case of hemorrhage into the
pseudocyst.
Ancillary Tbsts. Laboratory tests may reveal persistent

elevation of amylase in 50Vo of patients. Pseudocyst
should be suspected in the patient who shows persistent
elevation of amylase levels. Abdominal plain films may
reveal mass effect on adjacent organs but are otherwise
unhelpful. Ultrasound and CT (picture) are the preferred
diagnostic modalities. ERCP is not generally required.
Managemenf. A patient who develops an abdominal

mass during an attack of acute pancreatitis should be
observed for several weeks. If the patient remains relatively stable and the mass persists, he or she may be
referred for elective surgical decompression of the
pseudocyst. Pseudocysts that persist for longer than 4 to
6 weeks are unlikely to resolve spontaneously. If the
mass enlarges or the patient develops complications
such as infection or hemorrhage, immediate surgical
intervention will be required. Catastrophic hemorrhage
may be amenable to angiographic embolization. Drainage may also be achieved using CT:guided percutaneous
Pancreatic Insufficiency
(1. 4.
23
1.4)
Pancreatic insufficiency is a result of chronic pancreatitis, cystic fibrosis, carcinoma of the pancreas, or extensive pancreatic resection. The foremost cause is chronic
pancreatitis. These patients frequently present as diabetics who have a history ofrepeated attacks ofpancreatitis
and now have intestinal malabsorption. Patients with cystic fibrosis become clinically apparent in childhood and
occasionally in young adulthood. Profound weight loss
due to pancreatic insufficiency is often seen in pancreatic
carcinoma.
The loss of pancreatic enzymes leads to failure to

digest carbohydrates, lipids, and proteins. The typical
presentation of pancreatic insufficiency is with steatorrhea, weight loss, and other evidence of nutritional insufficiencies. There may also be evidence of Brz or fat-soluble vitamin insufficiency. Diagnosis is generally made by
quantitating fecal fat excretion.
The primary goals in the treatment of pancreatic insufficiency are to maintain an adequate nutritional status and
restore and maintain body weight. There
is also
an
Abscess
attempt to reduce the symptomatic complaints associated

with steatorrhea such as diarrhea and abdominal discomfort. Treatment is composed of drug therapy and dietary
counseling. Drug therapy consists of pancreatic enzyme
replacement. This is usually a lifelong endeavor and is
quite expensive. There are a variety ofpreparations available to replace pancreatic enzymes. These preparations
Two infectious processes may result in pancreatic

abscess, infection ofnecrotic pancreatic tissue a few days
into the course of acute pancreatitis, and the bacterial
have differing efficacies and should be tailored to the

patient's level of malabsorption. Pancreatic insufficiency
is rarely an ED diagnosis; however, if it is suspected,
appropriate referral for evaluation and treatment should
catheters.
seeding
of pancreatic pseudocysts 4 to 6 weeks
later.
to l0% of patients with acute panthe

abscess usually reveals multiple
creatitis. Culture of
E.
coli, gram-negative anaerobes,
organisms including
Abscess occurs in
be made.
2%o
and aerobic hemolytic streptococci.

These patients may present with clinical deterioration
as their episode ofacute pancreatitis should be resolving.
Fever, abdominal pain, nausea, vomiting, and evidence of
ileus are the hallmark symptoms. Physical examination
reveals fever, tachycardia, abdominal tenderness, and
possibly signs of septic shock.
Ancillary evaluation will reveal a leukocytosis with a
left shift. Serum amylase will be normal or slightly elevated. Radiologic diagnosis is achieved using either ultrasound or CT scanning.
Pancreatic abscesses are not amenable to antibiotic
therapy alone (mortality reaches 100%). Open surgical
drainage and debridement with removal of all necrotic

material is necessary. The technique of pancreatic resec-
Tirmors and Carcinoma (1.4.2)

Carcinoma of the pancreas is the fourth leading cause
of cancer-related death in the United States and its incidence is on the rise. The 3-year survival rate is approximately 2Yo.This disease rarely presents prior to the age of
50 and seems to have a prevalence in males over females
and blacks over whites. Heavy cigarette smoking is the
most common risk factor, it increases the risk of the disease by two- to threefold. Other associated risk factors
include diabetes mellitus and the consumption of highfat, high-protein diets. Chronic pancreatitis does not
appear to carry an increased risk.
Pathophysiology
tion with packing of the abscess cavity seems to have

reduced overall mortality. Percutaneous drainage has met
with only limited success. In general, mortality still
remains quite high.
is either endocrine or nonendocrine. The most common form of endocrine carcinoma is ductal adenocarcinoma (90%). Exocrine
Pancreatic carcinoma
24 /
Err,mncrNcv MnorcINe: THB Conn Cunxrculurr,l
tumors include insulinoma (the most common),

glucagonoma, gastrinoma, VIPoma (secretes high levels
of vasoactive intestinal peptide), somatostatinoma, and
carcinoid.
Presentation and Diagnosis
Carcinomas are generally present for longer than 2
months prior to their diagnosis. Weight loss is the earliest
symptom in 70o/o to 90o/o of patients with endocrine carcinomas. This weight loss is initially from anorexia but
may also be caused by pancreatic insufficiency as the
tumor grows. Pain is found in 50o/o to 80% of patients.
The pain is usually described as gnawing and may radiate
from the epigastric area to the back. Partial relief may be
obtained by sitting forward. Pain is usually a more severe
problem in those patients with tumors in the body or tail
of the pancreas since they may grow quite large prior to
presentation. Jaundice is found in 80% of patients with
tumors in the head of the pancreas. Physical findings on
presentation include a possible abdominal mass, abdominal tenderness, hepatomegaly, and jaundice.
The diagnosis of endocrine tumors is made by CT
scanning in over 80% of patients. CT scanning allows
good visualization of the hea4 body, and tail of the pancreas. Ultrasonography is less accurate. ERCP may be
used to clarif, ambiguous CT or ultrasound findings.
As for the nonendocrine tumors, insulinomas are usu-
present with hypoglycemia. These

patients manifest high insulin levels despite low glucose
levels. Glucose intolerance and a necrotizing migratory
ally benign and
erythematous rash are typical of glucagonoma. This

tumor metastasizes quickly and has done so in 60oh of
patients by the time of presentation. Elevated glucagon
levels usually make the diagnosis. Gastrinoma is the
cause of Zollinger-Ellison syndrome. This tumor usually
results in multiple peptic ulcers. The pancreas may be the
site of VIPomas. These tumors generally present with
watery diarrhea, hypokalemia, and achlorhydria. The

diarrhea associated with this tumor is usually profuse
(over 20 L a day). It may become necessary in these
patients to replace fluids and lost electrolytes.
Trestment and Prognosis
A high index of suspicion in the ED when encountering patients with vague abdominal discomfort, back pain,
and weight loss may help in leading to earlier diagnosis
of pancreatic carcinoma. For endocrine carcinomas, the
only cure is with complete surgical resection. This is only
possible in l0o/o to 15% of patients. Practically, these
resections are done in patients with tumors in the head of
the pancreas who present with jaundice while the tumor
is still small. Five-year survival rates following pancreatic
resection are only 10%.
In patients with unresectable tumors, median survival

is 5 months. Treatment of these patients is directed by
symptomatology. Pain should be controlled using opiate
analgesics. In patients with jaundice and pancreatic head
tumors, surgical diversion of bile may be necessary. For
ill patients, decompression of the biliary tree can be
achieved by endoscopic or percutaneous means. Radiation and chemotherapy with 5-fluorouracil appears to
prolong survival and increase the cure rate in comparison
to complete resection. For patients with widely metastatic disease, chemotherapy has not been shown to provide
any benefit.
SELECTED READING
Bruno MJ, Haverkort EB, Tytgat G, van Leeuwen D. Maldigestion associated with exocrine pancreatic insufficiency: implications of gastrointestinal physiology and properties of enzyme preparations for a causerelated and patient-tailored treatment. Am J Gastroenterol 1995;90(9):
1 3 83-l 393.
Harwood-Nuss AL, Linden CH, Luten RC, et al. Pancreatitis. ln: The clinical practice of emergency medicine Philadelphia: Lippincoft, 1991;
949-951.
Kadakia SC. Biliary tract emergencies. Med Clin North Am 1993l.77(5):
1015-1037.
May HL, Aghababian R! Fleisher GR, et al. Acute pancreatitis.In: Emergency medicine,2nd ed. Boston: Little, Brown, 1992;1480-1482.
Ranson JHC. Etiologic and progrrostic factors in human acute pancreatitis:
a review AmJ Gastroenterol 1982;77:633.
Rosen P, Barkin R, et a[. Disorders ofthe liver, biliary tract, and pancreas.
ln'. Emergenq, medicine: concepts and clinical praclice, 3rd ed'. 1992;
1601-1626.
Schwartz S, et al. Pancreas. In: Principles of surgery-, 5th ed. 1989;

1413-t440.
Schwartz S, Shires G, Spencer F, et al. Peritonitis and intraabdominal
abscesses. In: P rin c ip le s of surgery, 5th ed. 989; 459-1490.
Tintinalli J, Ruiz E, Krome R, et al. Acute pancreatitis. In: Emergency medicine: a comprehensive study guide, 4th ed. New York: McGraw-Hill,
1996;507 509.
Wilson J, et a[. Acute and chronic pancreatitis. ln Harrison s principles of
internal medicine, 12th ed. New York: McGraw-Hill, I 988; I 3 72-1 3 82.
Wilson J, et al. Pancreatic cancer. In: Harrison\ principles ofinternal medicine, l2th ed. NewYork: McGraw-Hill, 1988;1383-1387.
1
STOMACH (1.s)
Disorders of the stomach are a frequent cause of ED
visits and hospital admissions in the United States. The
use oftobacco and alcohol is undoubtedly a contributing
factor. Fortunately, the diagnosis and treatment of stomach disorders has been greatly advanced in the past two
decades with the development of fiberoptic technology
for endoscopy and histamine (Hz) antagonists.
Any patient complaining of epigastric pain should be
considered for a stomach ailment. This is not a specific
symptom, however, as several other proximally located
organs such as the duodenum, biliary tract, pancreas, and
heart may cause pain here. It should also be noted that

disorders of the stomach, perhaps more than any other
intraabdominal organ, can be aggravated by mood and
emotional stress.
AnnourNer AND
A basic understanding of the anatomy and physiology
of the stomach is a prerequisite to understanding the various pathologic conditions that affect it.
Anatomy and Pathophysiology
The stomach, as well as the pharynx and its derivatives,
the respiratory tract, esophagus, duodenum proximal to
the opening of the common bile duct, liver, pancreas, and
biliary tree all arise from the embryologic foregut. For

this reason, visceral (splanchnic) pain from these organs
is frequently referred to the mid-epigastric area. Visceral
or splanchnic pain is that which results from stretching of
the autonomic nerve fibers surrounding a hollow or solid
viscus, and is frequently described as crampy or gaseous.
The stomach normally lies in the left upper quadrant of
the abdomen with the pylorus at approximately the midline. The stomach is fixed only at the esophagocardiac
junction and the pylorus. The stomach varies in size as
determined by its contents or obstruction.
In the normal individual, the stomach is bordered superiorly and laterally by the diaphragm, medially by the
liver, and inferiorly by the transverse colon and small
bowel. The abdominal wall is anterior to the stomach with
the spleen posterolateral to it. The left kidney, suprarenal
gland, and body of the pancreas lie directly posterior to
the stomach in the retroperitoneal space.
The stomach begins at the esophagocardiac junction
just inferior to the diaphragm. It ends at the pyloric
sphincter, a thick band of muscle separating it from the
duodenum. The stomach is divided into four different
regions: the cardia, fundus, body, and antrum (Fig. l-1).
Each region is characterizedby different types and frequency of cells in the gastric mucosa.
The arterial blood supply to the stomach, as well as the
distal esophagus and proximal duodenum, is through
branches
GASTRoTNTESTTNAL
DrsonorRs
of the celiac axis off the descending
25
aorta.
Parasympathetic innervation of the stomach is provided

by anterior and posterior branches of the vagus nerve.
Sympathetic innervation is through roots 7, 8, and 9

the thoracic spine by way of the celiac ganglia.
of
The gastric mucosa starts at the gastroesophageal junction and ends at the pylorus. The fundus and body are the
acid-producing portions of the stomach, with an abundance ofparietal cells that excrete hydrochloric acid. The
mucosa of the antrum is primarily composed of mucus
secreting cells.
Bleeding from the stomach and proximal portion of the
duodenum is manifested as hematemesis or melena or
both. Since blood exposed to gastric acid changes to a
brown color almost immediately, the vomiting of "coffee
grounds" does not necessarily imply the emesis of "old
blood." Melena, a coal black, sticky or tar-like stool, can
be caused by as little as 60 cc of blood. Its presence
implies bleeding proximal to the ligament of Treitz.

While bleeding from a lower colon source may cause a
very dark stool, these stools usually lack the shiny and
sticky character of true melena. Hematochezia, the passage ofrecognizably red blood from the anus, is usually
caused by lower colon bleeds. However, hematochezia
may be caused by a large proximal duodenal bleed with a
rapid transit time. It is worth noting that some foods and
medications may give stool the false appearance of
melena or hematochezia. Included in this list are iron
salts, bismuth, charcoal, various food dyes, and beets.

Most patients presenting to an ED with a stomach diswill complain of abdominal pain or vomiting or
both. Ideally, all patients with these complaints would be
seen immediately. However, this is not always done.
order
Keeping a few key triage points in mind is helpful in

determining which patients must be seen immediately
and who can wait.
Pain that begins abruptly and is maximally severe in

intensity at the time of onset suggests a serious disorder
(e.g., a perforated viscus). Likewise, any patient who sits
very still or walks cautiously to avoid any agitation of the
peritoneal contents may have peritonitis. Any patient
complaining of vomiting blood should be evaluated

immediately. Finally, any patient with a pale, sweaty
appearance or significant tachycardia or hypotension
should receive immediate attention.
History
FlG. 1-1. Regions of the stomach. (From Eisenberg MM,
Fondacaro PF, Dunn DH. Gastroenterology. Applied anatomy
and anomalies of the stomach. Philadelphia: Saunders,
1995.)
As stated above, the mode of onset of the patients'

symptoms is of utmost importance. The duration of the
symptoms is equally important. More serious conditions
26 /
ElrpncnNcv MnorcrNr: THn Conn CunnrculuM
will
usually cause a patient to seek emergent medical

attention in a shorter period of time.
The presence of any abdominal pain in the epigastrum
would suggest an ailment involving the upper gastrointestinal tract, biliary tree, or pancreas. By and large, the
greater the intensity of the abdominal pain, the more
likely the patient to have a serious condition. It should be
noted that patients'perceptions ofpain intensity are influenced by cultural and socioeconomic factors, level of
education, and age. The patient should be asked about
any exacerbating or relieving factors, and specifically
about any relationship of the pain to eating, medications
taken, or other nonalimentary events (e.g., body position
or emotional stress).
The triad
of
anorexia, nausea, and vomiting is
extremely coflrmon in patients with stomach disorders.

However, the presence of any or all of these symptoms is
nonspecific
for these conditions. All patients
with
abdominal complaints should be asked about any prior

medical conditions, surgeries, and current medications
(specifically steroids, salicylates, NSAIDs, and antibiotics). Patients should be asked about any recent change
in the color of their stool. Any reproductive-age female
should be asked for a sexual, reproductive, and menstrual
history. Finally, any tobacco, alcohol, or recreational drug
used should be noted.
Any patient with an abdominal complaint
should
receive a thorough examination including a lung and cardiac exam. Any reproductive age female should have a
pelvic exam, as even upper abdominal complaints may
have a gynecologic etiology. Furthermore, a rectal exam
with stool guaiac testing should be performed.
The abdominal exam itself is best performed with the
patient as relaxed as possible. Visual inspection for distention and peristaltic waves as well as auscultation for
bowel sounds should be done first. Percussion for

intraabdominal organ size and tympany should be per-
formed. Finally, palpation should begin with the non-
ofthe abdomen and progress to those
embolism.
Patients with symptoms suggestive of gastric vohulus
should have upright chest and abdominal x-rays performed. A supradiaphragmatic vohulus will characteristically appear as one or two large, retrocardiac air-fluid
levels. Subdiaphragmatic vohuli frequently appear as a
markedly distended stomach with an abnormal lie and

one or two air-filled levels. There may be a pauciry of
Physical Examination
tender areas
most tender.
occur in children, usually under I year ofage and associated with congenital diaphragmatic defects. The peak
incidence in adults is in the fifth decade, with men and
women being equally affected. The majority of cases of
gastric volvulus are chronic in nature. Chronic volvulus is
probably underdiagnosed since it is frequently associated
with transient, nonspecific symptoms such as heartburn,
vomiting, or epigastric discomfort with meals.
Acute gastric volvulus frequently presents with sudden
onset ofsevere left upper quadrant pain with retching and
an inability to vomit. Since many gastric volvuli occur
above the diaphragm, a significant number of patients
will complain of anterior chest pain. Physical exam may
reveal a distended upper abdomen with a normal, soft
lower abdomen, or a relatively unremarkable abdominal
exam if the volvulus occurs above the diaphragm.
Differential diagnosis includes perforated peptic ulcer,
acute pancreatitis, pyloric obstruction, peptic ulcer disease, and cholecystitis. Patients with gastric vohulus
above the diaphragm may have symptoms mimicking
myocardial infarction, aortic dissection, or pulmonary
areas
Structural Lesions (1.5.1)

Volvulas (1.5.1.1)
bowel gas in the remainder of the gastrointestinal tract.

The inclusion of oral contrast may aid in the radiographic
diagnosis. The inability to pass a nasogastric tube into the
stomach combined with pain and violent retching completes a clinical triad that is highly suggestive of gastric
vohulus.
ED interventions
for patients suspected of gastric

volr,ulus include IV hydration and nasogastric tube insertion (as discussed above). Occasionally, the stomach can
be decompressed with a nasogastric tube. Immediate surgical consultation is warranted as surgical intervention is
almost always necessary. Emergent surgical decompression is indicated if decompression with a nasogastric tube
is unsuccessful. Endoscopic decompression may be considered in some patients who are poor surgical candidates. After replacement of the stomach into its normal
position in the abdomen, a variety of surgical techniques
may be used to anchor
there. Any diaphragmatic
it
defects contributing
to the volvulus should also
be
repaired.
Gastric volvulus is defined as an abnormal degree of

rotation of one part of the stomach around another. If not
reversed, this may lead to a closed loop obstruction with
ischemia and ultimately necrosis. Abnormal laxity of suspensory ligaments and diaphragmatic abnormalities,
including hiatal hernias, are believed to be the two major
causes. Approximately 15% of cases of gastric volvulus
Foreign Bodies
(1. 5.
1.2)
Foreign-body ingestion is not an uncommon complaint
in the ED. While most ingestions are uneventful, approximately 1,500 people die yearly in the United States as a
AsooN,uNAr eNo GesrnorNTESTrNAr
result of swallowed foreign bodies. Approximately 80%

cases are in the pediatric population. Adults at
increased risk for foreign-body ingestion include denture
wearers, alcoholics, individuals with poor eyesight, psy-
of all
chotic and demented patients, and prisoners. Most

patients with a gastric foreign body will be asymptomatic. This differs significantly from patients with esophageal foreign bodies, the majority of whom complain of
some discomfort. Any patient with a history of foreignbody ingestion should be evaluated for symptoms of
obstruction, perforation, and bleeding. Most gastric foreign bodies will be visible using plain or contrast radiography.
The majority of obstructions caused by foreign bodies

occur in the esophagus. Between 80% and 93o/o of foreign bodies that reach the stomach will pass spontaneously. The main points of obstruction for gastric foreign bodies include the pylorus, duodenum, ileocecal
valve, and anus.
Clear guidelines for the removal of gastric foreign bodies do not exist. By and large, the use of emetics is discouraged, especially in cases involving sharp or pointed
objects and button batteries. Most gastric foreign bodies
can be safely removed with the use of an endoscope.
Endoscopy should be considered for objects more than 2
cm in diameter or 5 cm in length, especially if the object
has irregular as opposed
to rounded edges. Sharp or
pointed objects, such as sewing needles or razor blades,

should be considered for emergent endoscopic removal
since 15% to 35Vo will cause perforation, usually at the
iliocecal valve. Potentially toxic objects should be

retrieved, with exceptions as discussed below Controversy exists as to the timing of endoscopy to remove otherwise innocuous foreign bodies that do not pass through
the stomach. These retained objects pose the risk of pressure necrosis, causing gastritis or ulceration.
Two situations warrant special mention: button battery
ingestion and the ingestion ofpackaged illegal drugs for
the purposes of concealment (i.e., "body packers"). Button batteries can leak highly alkaline substances and
cause severe burns or perforation in the esophagus. For
this reason, emergent endoscopy is indicated if the battery is lodged in the esophagus. Button batteries that
have passed into the stomach do not require emergent
removal in the asymptomatic patient unless the cell has
remained in the stomach for more than 48 hours. Endoscopic retrieval of intentionally ingested packets of ille-
gal drugs should not be attempted due to the risk of

packet rupture. Surgical removal is indicated if the
patient becomes symptomatic. Asymptomatic patients
can receive "whole bowel irrigation" with GoLYTELY to
expedite the movement of the packet through the gastrointestinal tract.
Patients who are to have endoscopy for gastric foreign
bodies should be placed in the left lateral decubitus position in an attempt to prevent the passage of the object
DrsonorRs
27
through the pylorus. Outpatient management of benign

gastric foreign bodies should include close follow-up and
a high-residue diet to promote passage through the gastrointestinal tract. Repeated radiographs to follow the
progression of the object through the gut may be performed. Patients should be advised to seek immediate
medical attention for any abdominal pain, vomiting, or
fever.
Rupture (1.5.1.3)
Gastric rupture refers to a tear in the stomach wall with
release of the gastric contents into the peritoneum. The
stomach is relatively resistant to rupture, with its muscu-
lar wall having the ability to greatly expand with

increases in intragastric contents, a phenomenon known
as receptive relaxation. Furthermore, the stomach can
decompress into the esophagus and duodenum in the nor-
mal individual. Gastric rupture is a rare condition with

both traumatic and nontraumatic causes.
Nontraumatic or "spontaneous" gastric rupture appears
to have a preponderance among women and a mean age
of occurrence of 43 years. Distention and vomiting

together or by themselves can cause gastric rupture.
Obstructing tumors of the esophagus, pylorus, or duodenum may be contributing factors. Distention can occur
because of zealous overeating, mouth-to-mouth or bagto-mouth ventilation during CPR, esophageal intubation,
profuse upper gastrointestinal bleeding, and from rapid
ascension in diving accidents. Sodium bicarbonate, used
as a remedy for indigestion, may cause gastric distention
from the carbon dioxide released during the compound's
reaction with stomach acid.
The stomach's position in the abdomen is well protected by the rib cage and the liver. Gastric rupture secondary to blunt trauma is accordingly rare. One study
found only 0.4o/o of patients with severe blunt trauma to
have gastric perforation. Gastric rupture has
been
described secondary to motor vehicle accidents, forceful

coughing, grand mal seizures, exertion with heavy lifting,
and use of the Heimlich maneuver.
Patients with gastric rupture complain of abrupt onset

of abdominal pain, with some reporting a bursting sensation. There is usually severe pain with signs of peritoneal
irritation. Abdominal distention may be present. A small
percentage of patients may have subcutaneous emphysema caused by the passage of abdominal air through the
mediastinum into the neck. Many patients present in

shock or quickly progress to it.
Diagnostic studies to consider include upright chest xray and lateral decubitus abdominal x-rays looking for air
under the diaphragms and flanks, respectively. CT using
oral and IV contrast may be helpful. Peritoneal lavage is
felt to be the most sensitive indicator of gastric rupture
after blunt trauma. Since most patients with gastric rup-
28 /
ElanncrNcv MnorcrNr: Tnn Conr CunnrculuM
ture present with impressive abdominal findings, there is

often little need for diagnostic workup prior to surgical
exploration. The differential diagnosis includes all forms
of abdominal catastrophe including aortic dissection and
rupture, bowel infarction, rupture of the gallbladder or

spleen, and vohulus. Any perforated viscus, most commonly secondary to peptic ulcer disease or appendicitis,
may present with symptoms similar to stomach rupture.
ED management of gastric rupture should include
aggressive fluid resuscitation and early administration of
parenteral broad-spectrum antibiotics. Nasogastric suction may help reduce the amount of peritoneal soilage.
Emergent surgical intervention is needed for peritoneal
lavage and gastric repair.
Gastric Outlet Obstruction
(1. 5.
1.4)
The complete or near-complete blockage of the flow of

gastric contents out of the stomach results in gastric out-
let obstruction. Chronic duodenal or pyloric channel

ulcers are responsible for approximately 80Yo of all cases
in adults. Other less common causes are listed in Table
l-6. Approximately 2oh of all ulcer patients will develop
gastric outlet obstruction. These patients typically have a
longer than l0-year history of ulcer disease, usually with
a severe clinical course.
Approximately 90% of patients with gastric outlet

obstruction will complain of upper abdominal pain and
vomiting. Early satiety and recent weight loss will be present in up to two-thirds of patients. Many will give a history of emesis after the evening meal with food from earlier in the day being present in the vomitus. Physical
examination may reveal a succussion splash (a splashing
sound elicited by gently rocking the abdomen) and evidence of chronic malnutrition or dehydration.
Diagnostic workup in the ED should include an upright
abdominal film, which often reveals a markedly dilated
stomach shadow with a large air fluid level. There may be
a paucity of gas in the small and large colon. Routine
TABLE
1-6.
Causes of gastric outlet obstruction other than

peptic ulcer disease
Tumors
Benign
Adenomatous
polyp
Malignant
Gastric carcinoma
Carcinoma of
pancreatic head
Lymphoma
lnflammation
Cholecystitis
Acute pancreatitis
Crohn's disease
Eosinophilic gastroenteritis
Miscellaneous causes
Adult hypertrophic pyloric
stenosis
Postsurgical stenosis
Pyloric diaphragm
Duodenal diaphragm
Caustic structure
Annular pancreas
Ectopic pancreas
From Graham DY. Gastrointestinal disease. Ulcer complications and their nonoperative treatment. Philadelphia:
Saunders, '1 993.
blood chemistries may reveal a hypokalemic, hypochloremic metabolic alkalosis secondary to chronic vomiting.
ED interventions should include nasogastric tube insertion, which often results in evacuation of a large amount
offoul fluid. IV hydration is also frequently necessary.
Conditions that may mimic gastric outlet obstruction
include gastroparesis and small bowel obstruction. Gastroparesis often occurs in the setting ofa predisposing illness, such as long-standing diabetes or scleroderma, and
classically causes vomiting with little associated abdominal pain. Small bowel obstruction is often of more acute
onset than gastric outlet obstruction, and has the characteristic small bowel air fluid levels on x-ray.
All patients with suspected gastric outlet obstruction
should be admitted to the hospital. Endoscopy may be
performed for definitive diagnosis and biopsy if malignancy is suspected as the cause of obstruction. Definitive
management may include endoscopic balloon dilatation
or surgical pyloroplasty or partial gastric resection.

Acute Gastritis
(1. 5. 2.
1)
Gastritis is an inflammation of the mucosa of the stomIt is, by definition, a diagnosis that can be made only
by histologic examination. The term is used, however, by
clinicians to describe a variety of disorders, many of
which would be more appropriately called "nonulcer dyspepsia." The World Congress of Gastroenterology in
1990 grouped the many causes of gastritis into three main
classifications: acute gastritis, chronic gastritis, and "special forms" of gastritis. Only acute gastritis will be disach.
cussed here.
Acute gastritis is characterizedby inflammation of the

gastric mucosa with polymorphonuclear cells with some
loss of epithelium. Causes of acute gastritis include radiation, uremia, alcohol, steroids, NSAIDs, aspirin, various
infections, corrosive agents, and stress. It is a transient
process that often resolves without sequelae. The true
incidence of acute gastritis is unknown, due in large part
to the vast majority of cases going undiagnosed, or being
diagnosed on clinical grounds only.
While never substantiated" most believe gastritis presents with vague upper abdominal complaints including
dyspepsia or indigestion. Vomiting may be present. Acute
gastritis can cause a significant amount of bleeding and
patients may complain of hematemesis or have hemepositive stools. Patients with acute gastritis by definition
should not have signs of peritoneal inflammation.
To many emergency physicians, gastritis probably represents a benign diagnosis ofexclusion. To this end, diagnostic workup in the ED should include a diligent search
for other causes of upper abdominal pain and dyspepsia.
A complete blood count, with attention to the hemoglo-
AsooNaNAL AND GASTRoTNTESTTNAL Drsonoens
bin level if there is a history of bleeding, liver function

tests, and, in elderly patients, an EKG should be performed. Nasogastric suction should be performed in any
patient with a history of hematemesis.
Treatment of acute gastritis should begin only after
other causes of the patient's symptoms have been
excluded. Antiemetics, such as prochlorperazine, may be
used to help control vomiting. A bland diet as well as
cessation of smoking should be recommended. Antacids,
such as Maalox, should be tried and initiation of Hzblocker therapy is an option. Above all else, the presumed cause of the gastritis, if known, should be discontinued.
Most patients with presumed gastritis can be discharged from the ED with close follow-up. Indications
for admission include intractable vomiting, hematemesis
with evidence of continued bleeding or significant blood
loss, severe dehydration, or the inability to rule out more
serious causes of the patient's symptoms.
Three subclassifications ofacute gastritis warrant special mention in an emergency medicine text: stressrelated gastritis, corrosive gastritis, and drug-induced
gastritis.
Stres
s-
Re I ate
d Gastritis
In experimental models, stress predisposes to both gastric and duodenal injury. While it is well accepted that
physical stressors lead to gastric mucosal injury, some
debate exists as to whether psychological stress causes
damage. Gastric damage is a significant problem in
TABLE
1-7,
of
Treatment involves primary prophylaxis
Drain cleaners
Sulfuric acid (95-99%)

Metal cleaners and antirust compounds
Phosphoric acid (5-80%)
Oxalic acid (1%)
Hydrochloric acid (5-25%)

Chromic acid (5-20%)
Soldering fluxes
Zinc chloride (10-35%)

Hydrochloric acid (up to 40%)
in at risk
patients. Antacids, Hz blockers, and sucralfate have all
been proven efficacious for this purpose, and debate

continues over which agent is best. Treatment in the ED
is the same as for other causes of acute gastritis. Cold
saline lavage has been advocated by some for this condition; however, the efficacy of this action remains questionable.
Corrosive Gastritis
The ingestion of corrosive substances can cause a

severe form of gastritis with both short- and long-term
gastric sequelae. The stomach is susceptible to damage
from both highly acidic and highly alkaline compounds
(Table l-7). Acidic compounds cause a coagulative
necrosis, theoretically leading to eschar formation and
protection from further mucosal damage. Alkaline compounds, in contrast, cause a liquefaction necrosis leading
Acids
Hydrochloric acid (1 0-25%)

Oxalic acid (2%)
sodium bisulfate (70-1 00%)
29
patients with large (>35% body surface area) burns, sepsis, respiratory failure requiring prolonged mechanical
ventilation, trauma, shock or prolonged hypotension,
renal failure, and multiple system failure. Prolonged ICU
stays are a separate risk factor. Of particular importance
to emergency physicians is the increased risk of patients
in extended care facilities.
The most common presentation
stress-related
mucosal damage is bleeding, manifested as hematemesis,
guaiac-positive stools, or frank melena. Clinically significant bleeding is more likely to be associated with gastric
or duodenal ulcers in this setting, rather than gastritis.
Household agents containing potentially corrosive acids and alkalis
Toilet bowl cleaners

Sulfuric acid (80%)
Drain cleaners
Sodium hydroxide (1 0-1 00%)
Household ammonia
Ammonium hydroxide (3-1 0%)
Automatic dishwasher detergents
Sodium tripolyphosphate
Sodium metasilicate
Sodium silicate
Sodium carbonate
Oven cleaners
Sodium hydroxide
Bleaches
Sodium hypochlorite (3-6%)
Sodium silicate (15-17%)
Sodium carbonate (60%)
Automobile battery fluid

Swimming pool sanitizers
Calcium or sodium hypochloride (70%)
From Hawkey CJ, Hudson N. Gastroenterology. Mucosal iniury caused by drugs,

chemicals and stress. Philadelphia: Saunders, 1995.
30 /
ErrarncnNcy MnucrNr,:
to saponification of
Tnn Conn CunrucuLUM
these tissues and
a deeper burn.
Given its acidic environment, the stomach is less prone to

alkaline injury than is the esophagus.
Swallowing of a corrosive substance with gastric
injury causes severe epigastric pain with retching and
vomiting of blood or necrotic tissue. Patients with any
stridor, hoarseness, or shortness of breath pose an immediate airway risk and should be considered for immediate
intubation. Patients complaining
dysphagia and
odynophagia are likely to have esophageal burns.
of
The immediate complications of a corrosive gastritis

include hypovolemia, as with any severe burn, and gastric
perforation with peritonitis.
metabolic acidosis can
occur with ingestion of a large amount of acid. Patients

who survive a caustic ingestion face the long-term complications of strictures that may become symptomatic
weeks to years postingestion.
ED management of patients with corrosive gastritis
should include IV hydration, chest and abdominal radiographs looking for evidence of perforation, and admission. Insertion ofa nasogastric tube is contraindicated in
alkali ingestions due to the risk of perforation. It is controversial in large acidic ingestions, as some authors feel
it is worth the perforation risk to remove the acid from the
stomach to prevent severe systemic acidosis. Emetic
agents as well as the administration of "neutralizing"
acidic or basic compounds is contraindicated. Corticosteroids, given in the hopes of preventing stricture formation, remain highly controversial. The empiric use of
broad-spectrum antibiotics in the absence of perforation
is of unproven benefit, yet recommended by many
authorities.
Drug-Induced Gastritis
TABLE 1-8. Medications associated with gastric mucosal

tnlury
Antibiotics
Nalidixic acid
Sullonamides and derivatives

Erythromycin
Pivampicillin
Antiinflammatory drugs
NSAIDS
Corticosteroids
Colchicine
Chloroquine
Elements
Fe+
K+
Gold
Hypnotics
Chloral hydrate
Meprobamate
Miscellaneous
Ethacrynic acid
Ethanol
Mebendazole
Mucolytic agents
Prostaglandins
Reserpine
Salicylates
Sulfasalazine
Sulfinpyrazone
Xanthine (and catfeine)
From Eisenberg MM, Fondacaro PF, Dunn DH. Gastroenterology. Applied anatomy and anomalies of the stomach.
Philadelphia: Saunders, 1 995.
in these patients when treating minor pain.

Some authors advocate primary prophylaxis against
NSAID-induced gastropathy with synthetic prostaglanminophen,
dins, such as misoprostol, or H2 antagonists.

Peptic Ulcer Disease (1.5.3)
Peptic ulcers are defects in the gastrointestinal mucosa
extending through the muscularis mucosae, caused by the
action of the acid and pepsin present in gastric juice. An
estimated half million new cases are diagnosed each year
in the United States, with approximately 15,000 deaths per
year attributed to peptic ulcer disease (P[ID).The lifetime
prevalence of PUD in the United States has been estimated
at llo/o to l4Yo for males and 8o/o to llYo for females with
an increased prevalence among lower socioeconomic
classes. These numbers are likely to increase in the future
Several medications have been associated with gastric

mucosal damage leading to gastritis and ulceration (Table
1-8). Medications are believed to cause mucosal injury
either directly, or by reducing the mucosa's resistance to
the damaging effects of stomach acid, or by increasing
hydrochloric acid output. NSAIDs, including aspirin, are
believed to be the most common offending agents in this
increasing use of NSAIDs. Peptic ulcers are found in the

stomach and in the duodenum. Ninety-five percent of peptic ulcers in the duodemrm are located in the first portion.
category.
under the broad heading ofpeptic ulcers.
Little is known about the exact incidence of mucosal

damage caused by NSAIDs. It appears to be most common in elderly women, perhaps because these are the
A breakdown of the usual mucosal defense mechanisms is believed responsible for the majority of peptic
ulcers. Colonization of the gastrointestinal mucosa with
Helicobacter pylori and NSAID use (including aspirin)
are felt to be the two main risk factors. It has been estimated that roughly 25Yo of chronic NSAID users will
develop PUD. Other risk factors include smoking, a history ofchronic renal failure, hepatic cirrhosis and chronic
patients for whom these medications are most commonly

prescribed. NSAIDs may cause acute or chronic mucosal
injury, and their damaging effects appear to be increased
with the addition of corticosteroids.
Patients with presumed drug-induced gastritis should
discontinue the oflending agent if possible. Caution

should be used by emergency physicians when prescribing NSAIDs, especially in elderly patients. It may be wise
to try alternative firstline analgesics, such as aceta-
due to an increasing proportion
of elderly patients
and
While some clinical differences exist between the two

ulcer types (Table l-9), they will both be discussed here
pulmonary disease, Zollinger-Ellison syndrome, and

radiation therapy. Ethanol consumption, diet, corticosteroid use, and psychogenic stress all remain controversial as possible causes of PUD.
AsooNarNAL eNo GesrnoINTESTrNAr

TABLE
1-9.
Symptoms of gastric and duodenal ulcers

Gastric
ulcer
(Y")
Symptoms
Pain/discomfort
Features of the pain
Primary pain
Epigastric
Right hypochondrium
Left hypochondrium
Frequently severe
Within 30 minutes of food
Gnawing pain
lncreased by food
Clusters (episodic)
Relieved by alkali
Food relief
Occurs at night
Not related to food or variable
Radiation to back
lncreased appetite
Anorexia
Weight loss
Nausea
Vomiting
Heartburn
Nondyspeptic symptoms
Fatty food intolerance
Bloating
Belching
100
67
6
6
Duodenal
ulcer
(%\
53
5
16
1
0-40
56
34
20-31
46-57
24-61
25-36
1 9-45
49-59
19
54-70
38-73
19
25-57
27-59
49
59
From Soll AH. Gastrointestinal disease. Gastric, duodenal

and stress ulcer. Philadelphia: Saunders, 1993.
Epigastric pain is the primary complaint in two-thirds

of PUD patients, and the vast majority will give a history
of some dyspepsia. The pain is frequently described as
"burning," nonradiating, and occurring two to three hours
after meals and at night. Relief is frequently obtained
with food or antacid use. Physical examination
may
reveal some epigastric or upper quadrant tenderness and
heme-positive stool. It should be noted, however, that it is
not possible to diagnose PUD with any accuracy by history and physical exam alone.
Definitive diagnosis
of PUD involves the use of
endoscopy or contrast radiography and is beyond the scope
of this text. Diagnostic workup of patients with symptoms

suggestive of PUD in the ED should focus on the potential
complications, as discussed below. The differential diagnosis to consider includes nonulcer dyspepsia, gastroesophageal reflux, neoplasm, mesenteric ischemia, pancreatitis,
hepatobiliary disease, and ischemic heart disease.
Treatment of presumed uncomplicated PUD is contro-
versial. While
all agree any potentially
those patients who must remain on NSAID therapy. If

outpatient antiulcer medication is started in the ED, it is
probably best to consult with the patient's primary care
physician (if possible) or to at least arrange close followup for the patient.
The majority of uncomplicated PUD patients may be
discharged from the ED. Reasons for admission include
suspicion of any of the complications discussed below.
Approximately l0% of PUD patients will present with a
serious complication as the initial manifestation of their
illness. This is especially true for elderly patients.
Hemorrhage
41-72
55
48
logue. Sucralfate and bismuth are less popular "protective
61-86
7-17
3-5
32-43
22-53
2-48
famotidine being the most commonly prescribed), the

H*/K*-adenosine triphosphatase (AIPase) inhibitor
omeprazole, and misoprostol, a prostaglandin E ana-
antacids (Mylanta or Maalox) are relatively inexpensive

antiulcer medications. All of these medications will give
approximately the same ulcer cure rate after 6 to 8 weeks
of use, with misoprostol being especially efficacious in
39-86
20-63
50-88
21-49
36-87
31
100
13
16
barrier"-type ulcer medications available. Finally,
68
20
24
DrsorusRs
ulcer-causing
medications and social habits should be discontinued (if

possible), differences of opinion exist as to whether outpatient antiulcer medications should be started in the ED.
Several medications now exist for treatment of PUD
including the Hz antagonists (cimetidine, ranitidine, and
Hemorrhage
is the most common complication of
PUD, occurring in approximately l5oh of ulcer patients.

Hemorrhage usually occurs in older ulcer patients, with
the peak incidence being in the sixth decade. Endoscopic
studies indicate that approximately 50% of upper gastrointestinal bleeding (UGIB) is from peptic ulcers. PUD
accounts for approximately 560/o of deaths from UGIB.
Major bleeding from peptic ulcers is arterial in origin.
Hematemesis or melena or both will be present in more
than 95o/o of patients. Hematochezia may be present if
bleeding is rapid. Most PUD bleeding is painless. If a significant amount of upper abdominal pain is present, perforation should be considered since a small percentage of
perforations will have a significant amount of UGIB.
Diagnostic workup for any UGIB should include
complete blood count and coagulation profile. It should

be noted that a significant drop in hemoglobin may not be
present in acute bleeds before fluid resuscitation. Insertion of a nasogastric tube and aspiration is routinely performed; however, this has been shown to be of little value
in predicting active bleeding. Nevertheless, return of
"coffee grounds" or frank blood helps establish the bleeding as being of upper intestinal origin.
The differential diagnosis to consider in any patient
with evidence of UGIB includes gastritis, gastric or
esophageal varices, Mallory-Weiss tears, esophagitis, and
duodenitis. History and physical exam alone are usually
not helpful in determining the exact source of UGIB.
ED interventions for any patient with UGIB should
include insertion of at least one large-bore I! two in any
patient with signs of instability. Blood for type and crossmatch for packed red cells should be sent to the blood
32 /
Err,rnncnNcy
Mnorcnn: Trm Conr Cunnrculurvr
bank, while type O negative red cells may be used
if
needed before the cross-matched blood is available. Correction of any existing coagulopathy should be attempted.
Ice water lavage through a nasogastric tube has been
proven unbeneficial. Lavage with tap water is frequently

done in an attempt to "lavage the stomach to clear" thus
indicating a cessation of bleeding. Administration of

intravenous H2 antagonists has been shown to be of some
benefit in preventing rebleeding.
Endoscopy should be considered for all patients with
UGIB. The timing of this procedure remains controversial. In addition to locating the site of the bleed, several
endoscopic therapeutic modalities such as laser therapy,
thermal probes, and injection sclerotherapy are now
available. Surgical intervention is indicated when continued life-threatening hemorrhage cannot be stopped by
endoscopic methods. Angiographically guided embolization may be an option in those patients who are poor surgical candidates.
Any patient with evidence of significant UGIB should
be admitted to the hospital. Those patients with hemodynamic instabiliry repeated episodes of hematemesis or
hematochezia, age over 60, a failure to clear with gastric
lavage, or significant cardiac, pulmonary, or renal disease
should be admitted to an ICU setting. From l0% to 32o/o
of patients admitted to the hospital because of UGIB
caused by peptic ulcers will rebleed.
Perforation (1.5.3.2)
Perforation is the second most common complication
7Vo of ulcer patients. Perforation
occurs when the ulcer extends through the muscle wall
and serosa, establishing a communication between the
lumen and the peritoneal cavity. Pyloroduodenal perforation occurs six to eight times more frequently than gastric
of PUD, occurring in
perforation. Spillage of gastric or duodenal contents

causes a chemical peritonitis that quickly progresses to
bacterial peritonitis.
Patients frequently complain
contrast material through a nasogastric tube may be done
to aid in the diagnosis. Paracentesis may be helpful in
few select cases.

Treatment in the ED consists of fluid resuscitation,
correction of electrolyte abnormalities, nasogastric suction, and administration of broad-spectrum antibiotics
covering both aerobes and anaerobes. Surgical intervention is indicated in almost all cases, though some studies
have shown similar outcome results in young patients
treated nonoperatively. The overall mortality for perforated peptic ulcers is from 60/o to l|oh.
Pyloric Stenosis
Pyloric stenosis with resultant gastric outlet obstruction occurs in approximately 2oh of PUD patients.
Penetration
Penetration describes the erosion of an ulcer through
the entire thickness of the stomach or duodenal wall without leakage of digestive contents into the peritoneal cavity. The ulcerative process is contained by fibrous adhe-
sions to adjacent structures. Penetration occurs most

commonly with posterior wall ulcers.
The pancreas is the most common site for penetration
by both gastric and duodenal ulcers. Other less commonly penetrated organs include the liver, spleen, and
kidney. Duodenal ulcers may penetrate into the inferior
vena cava and aorta, usually with catastrophic results.
Most patients with penetrating ulcers have had longstanding symptoms, and present with a change in the pattern of their usual ulcer pain. They frequently describe a
change in their pain from episodic to constant, with new
referral of the pain to the midback. Ultrasound and CT
may aid in the diagnosis.
Tirmors (1.5.4)
of an abrupt
onset of
severe upper abdominal pain that progresses to symptoms
of diffuse peritoneal inflammation. Posterior wall gastric

ulcers may perforate into the lesser peritoneal space,
which results in a more localized inflammatory reaction
and more obscure symptoms. Physical exam frequently
reveals an acutely ill patient who remains very still to
avoid any peritoneal irritation. There is diffirse abdominal
tenderness, often most pronounced in the epigastric area,
with many patients having a classic "board-like rigidity"
of the abdominal musculature. Bowel sounds are absent
in approximately two-thirds of cases.
Demonstration of free air on an upright chest x-ray or
left decubitus abdominal film is diagnostic. However, this
finding is present in only 650/o Io 80% of cases. Insufflation with 250 cc of air or instillation of a water-soluble
Both benign and malignant tumors may arise in the

stomach. Benign tumors are usually in the form of gastric
polyps. Most remain clinically silent, with a rare few
causing obstructive symptoms. Approximately 95Yo of
malignant gastric tumors are adenocarcinomas arising
from the gastric epithelium with lymphomas, carcinoid
tumors, and sarcomas representing the other 5%.
Metastatic disease to the stomach may be confused with
a primary tumor. Tumors known to metastasize to the
stomach include melanoma, lung, breast, and" most frequently in AIDS patients, Kaposi's sarcoma. For the purposes of this text, the term gastric cancer will be used in
reference only to gastric adenocarcinomas.
Gastric cancer is the second most colnmon cancer in
the worl4 having been surpassed in frequency by lung
AeloN,rrNAr AND GASTRoINTESTINAL DIsonoeRS

cancer in the 1980s. There has been a dramatic and unexplained decline in the incidence of gastric cancer worldwide in the past 30 to 50 years, most noticeably in indus-
trialized nations. The incidence of gastric cancer

decreased by 67% in the United States from 1950 to
1979. The current annual incidence of gastric cancer in
the United States is less than 5 cases per 100,000 popula-
tion, making it one-fifth as prevalent as colon cancer.

Gastric cancer is rare under the age of 30, with the
largest number ofreported cases in the 50- to 7O-year-old
age group. Males are affected at a ratio of approximately
2:1. There is a definite geographic predilection, with immigrants from Japan, China, Chile, and Ireland being at
exceptionally high risk. Of note, the offspring of immigmnts to the United States will have approximately the
same risk as the native population if raised here. Factors
associated with an increased risk ofgastric cancer include
tobacco and alcohol use; a diet high in pickled vegetables,
salted fish and meats, and smoked foods and nitrates; and
occupations involving excessive exposure to dust. Patients
with chronic atrophic gastritis, intestinal metaplasia, pemicious anemia, and prior gastric surgery appear to be at
increased risk. Gastric cancer is more colrunon in lower

socioeconomic classes. Much controversy exists as to
whether gastric ulcers are a risk factor for gastric cancer.
Also controversial is a possible protective effect of a diet
high in fresh vegetables and dairy foods, and the use of
food refrigeration.
Gastnc cancer is usually asymptomatic in its ear$ stages
and even in advanced stages has nonspecific symptoms.
Abdominal pain is frequently the first complaint' Patients
may complain of a vague upper abdominal fullness or a
steady, severe epigastnc pain. Other complaints may
include vomiting, bloating, early satiety, or frank anorexia.
Hematemesis or melena may occasionally occur. Infrequently, patients present with symptoms from metastatic
disease such as ascites from liver involvement or dyspnea
secondary to malignant pleural effirsions. Physical exam
may reveal cachexia, an epigastric mass, hepatomegaly, or
heme-positive stool. Classic signs of distant metastases

such as umbilical nodes (St. Mary Joseph's nodes), supraclavicular nodes (Virchow's nodes), or an ovarian mass
33
is important in all suspected cases. Indications for admission include severe anemia or malnutrition, intractable
pain or vomiting, significant bleeding, or any evidence of
metastatic disease.
SELECTED READING
Boland CR, Scheiman JM. Tumors of the stomach. In: Yamada T, ed. Textbook of gastroenterology. Philadelphia: Lippincott, 1995;1494-1522.
Eisenberg MM, Fondacaro PF, Dunn DH. Applied anatomy and anomalies
of the stomach. In: Haubrich WS, Schaffirer R Berk JE, eds Gastroenterology. Philadelphia: Saunders, 1995;561-581.
Graham DY. Ulcer complications and their nonoperative treatment' In:
Sleisenger MH, Fordtran JS, eds. Gaslrointestinal disease Philadelphia:
Saunders, 1993 ;698J 12.
Harford W! McArthur KE. Diverticula, hernias, vohulus, and rupture. In:
Sleisenger MH, Fordtran JS, eds. Gastrointestinal disease. Philadelphia:
Saunders, 1993;478485.
Hawkey CJ, Hudson N. Mucosal injury caused by drugs, chemicals, and
stress. In: Haubrich WS, Schaffirer F, Berk JE, eds. Gastroenterology.
Philadelphia: Saunders, 199 5 ;656-699.
Spiro HM. Duodenal ulcer. In: Spiro HM, ed. Clinical gastroenterology.
New York: McGraw-Hill, 1993;251-282.
Spiro HM. Gastric ulcer. In: Spiro HM, ed. Clinical gastroenterologlt New
York: McGraw.H lll, 1993 ;28319 4.

Webb WA. Management of foreign bodies of the upper gastrointestinal
ft act. Gastroenterology 1988;94:204.
Wolf SG. Eliciting and interpreting symptoms and signs. In: Haubrich WS,
Schafftrer F, Berk JE, eds. Gastroenterology. Philadelphia: Saunders,
1995;3-10.
SMALL BOWEL (1.6)

Abnormalities of small bowel function will frequently
result in visits to the ED. While the small intestine pathophysiology is complicated there exist some common
signs and symptoms of specific small bowel disorders.
The emergency medicine physician should promptly
recognize these suggestive characteristics and conduct
initial management and further confirmatory analysis.
The physician should consider any alternative diagnosis
and be alert for potential complications of each small
bowel entity. Once the specific small bowel disease is
diagnosed, definitive treatment and final disposition of
the patient is addressed.
(Krukenberg tumor) rarely occur. Unfortunately, most

physical findings are the result ofextensive disease.
A high index of suspicion is needed to detect gastric
cancer in the ED. Diagnostic workup should include eval-
uation for anemia and liver dysfunction. A chest x-ray

looking for pulmonary metastases should be done in all
patients with concomitant respiratory complaints. Doublecontrast radiography is frequently used to evaluate patients
with epigastric complaints. While this is not a frequently

performed study in the ED it can often be ordered as an
outpatient in coordination with follow-up. Definitive diagnosis is usually made with endoscopic tissue biopsy.
The vast majority of patients with gastric cancer will
not be diagnosed in the ED, and therefore close follow-up
Obstruction (1.6.1.1)
Disorders of small bowel motility are classified into
organic and functional obstruction causes. Organic
causes of obstruction are generally treated with surgical
management. Functional causes of obstruction are generally managed medically.
Mechanical
Organic obstruction, also referred to as mechanical

obstruction, usually results from adhesions, hernias,
34 /
EuoncnNcy Mnucwn: Tun Conr CunrucuLUM
tumors, intussusception, or strictures. Adhesive bands are

the most common cause of small bowel obstruction.
Patients present with diffirse colicky abdominal pain,
which becomes more persistent with increasing distention. Distention becomes more evident with the more dis-
tal the level of obstruction, or the longer the duration of

obstruction. Patients often complain of vomiting, obstipation, and weakness. The patient may present
as restless,
tachycardiac, and anxious, frequently attempting to

relieve the pain by changing positions. Visible and audible peristalsis may be observed. Moderate generalized
abdominal tenderness, without signs of peritoneal irritation and borborygmus, may be present.
Electrolyte disturbances or hemoconcentration secondary to persistent vomiting and dehydration is common. Acute abdominal radiologic series will characteristically show gas and fluid-filled loops in the small bowel.
Differential diagnosis should consider any possible
cause of acute abdominal pain, such as perforation,
appendicitis, gallbladder, or renal abnormality.
Surgical intervention
is the definitive treatment of
mechanical obstruction. Management in the ED includes

nasogastric suction to relieve abdominal distention and
vomiting and possibly reduce aspiration, fluid and electrolyte correction, and administration of preoperative sur-
gical prophylactic antibiotics.
Adynamic
Functional obstruction, also referred to as adynamic or
paralytic ileus, has a variety of intraabdominal etiologies.
Adynamic ileus usually results from external trauma,

severe electrolyte imbalances (especially hypokalemia),
exposure of the peritoneum to irritants, intraabdominal
vascular accidents, pancreatitis, severe infections, uremia, or renal colic, or in association with postoperative
abdominal surgery. Anticholingeric medications altering
the sympathetic tone may cause adynamic ileus.
In contrast to mechanical obstruction, the mild abdominal pain of functional obstruction is continuous rather
than colicky. There may be associated vomiting and obstipation. A possible history of an underlying precipitating
medical condition should be investigated.
Physical examination of the abdomen reveals generalized distention and nonlocalized abdominal tenderness.
Peritoneal signs are generally absent. Dehydration may
develop secondary to vomiting or third spacing of fluid in
the bowel.
Similar to mechanical obstruction, functional obstruction patients may show serum electrolyte imbalances and
hemoconcentration on laboratory analysis. Other laboratory abnormalities may reflect the underlying cause of the
ileus, such as an increase in amylase or lipase with pancreatitis.
Abdominal radiography images will detail distended

gas-filled and air-fluid loops in the small bowel.
The treatment of functional obstructions is conservative medical management and treatment of the primary
underlying condition. Supportive care includes nasogastric suction along with replenishment of fluids and electrolytes as necessary. The patient is restricted from any
oral nourishment until normal intestinal function returns.

Aortoenteric Fistula
(1.6.2. 1)
Aortoenteric fistula occur primarily postoperatively

when an abdominal aorta aneurysm erodes into the
enteric tract. The most common site of occurrence is the
distal portion of the duodenum. Predisposing factors
include atherosclerosis, hypertension, smoking, hyperlipemia, diabetes mellitus, and trauma.
Noninvasive imaging studies for an aortoenteric fistula
include plain abdominal roentgenogram, ultrasound, and
CAI
scan. Lateral abdominal x-rays may reveal an
abdominal aortic aneurysm outlined by sclerotic plaques.

Ultrasonography images of the abdominal aorta may
show the aneurysm location in relation to the renal arteries. Computer-aided tomography images give a better
degree of aneurysm diameter size and indicate if any rupture has occurred. Abdominal angiography will greatly
demonstrate the source of the bleeding site in the gastrointestinal tract.
Prompt surgical intervention and resection is the specific treatment for aortoenteric fistula.
Malabsorption
(1. 6. 2.
2)
Malabsorption syndromes of the small intestine are

caused by the malfunctioning of infected intestinal
mucosa, whereas maldigestion conditions are the result
of intraluminal abnormalities in the breakdown of nutrients by the gastrointestinal tract. Examples of maldigestion conditions are pancreatic or biliary secretion deficiencies. There are a variety of small bowel absorption
etiologies including celiac and tropical sprue, along with
disaccharidase deficiencies.
Sprue syndrome is indicated by motor abnormalities

and impaired nutrient absorption, primarily of fats and
fat-soluble vitamins. The signs and symptoms vary
according to the extent of small bowel involvement and
the progression of the disease. The syndrome can also be
the result of Whipple's disease, Crohn's disease, and parasitic infections. The loss of mucosal microvilli results in
disaccharidase deficiency, particularly lactase deficiency.
Sprue syndromes are characterizedby severe weight loss
and nutritional deficiencies.
AsroN,rrNAL AND GAsTRoTNTESTTNAL
Drsonnnns
35
Tropical sprue patients have a history oftravel to tropical climates with symptoms sometimes appearing years
after travel. Tropical sprue appears to behave like an
infectious process; however, the etiology is unclear. The
patient will complain of indigestion, flatulence, abdominal cramps, and weakness. The symptoms are initially
characterized by explosive watery diarrhea. The diarrhea
ply lacks the enzyme lactase, required for the breakdown

of lactose.
Symptoms generally consist of abdominal bloating,
will become fewer, and the stools become more

solid, foul smelling, frothy, and pale in color. The patient
will describe the greasiness of the stool, due to the poor
fat absorption. The diarrhea can be exacerbated by a
high-fat diet. Physical examination may reveal abdominal
distention and mild tenderness.
confirming the diagnosis.
episodes
Vitamin-deficiency symptoms can develop, such as

glossitis, cheilosis, angular stomatitis, cutaneous hyperpigmentation, and dry rough skin.
Laboratory analysis may reveal a mixed picture of
hypochromic anemia (macro or microcytic). There will
be increased fecal fat content. Serum protein, cholesterol,
calcium, phosphorus, and prothrombin will be decreased.
Pancreatic enzymes are normal. Barium radiologic studies of the small intestine demonstrate a dilated lumen
with flattened mucosal lining.
Tropical sprue responds very well to folic acid and
broad-spectrum antibiotics. Vitamin replenishment should
be considered if the tropical sprue is long standing. Folic
acid should be administrated for several weeks to reverse
the deficiency.
Celiac sprue causes absorption abnormalities in the

fat-soluble vitamins, fats, proteins, carbohydrates, iron,
and water. Symptoms generally begin in early childhood.
There may exist a latent phase of remission, with the
symptoms returning later in adulthood. Patients with dermatitis herpetiformis frequently have symptomatic celiac
sprue as well. Laboratory analysis reveals microcytic
hypochromic anemia.
Unlike tropical sprue, celiac sprue responds very well
to a gluten-free diet. This diet lacks the polypeptide linkage, which cannot be broken down
in
celiac sprue
patients. The diet should also be high in calories and proteins and low in fat. Initially, the diet should lack foods
containing lactose, which may aggravate the condition.
Vitamin and electrolyte replenishment should be considered in celiac sprue as needed. Corticosteroids may also
be necessary for severely ill patients, since their use will
increase the patient's appetite and improve the absorption
ofnutrients by the gastrointestinal tract.
A suction biopsy may be indicated to eliminate other
etiologies such as cancer or lymphoma if the patient is
unresponsive to a gluten-free diet. A small percentage of
discomfort, distention, increased flatulence, and diarrhea,

all in response to the breakdown of lactose.
The diagnosis is often made clinically. However, if
required a milk challenge trial or abstinence may assist in
Meckel's Diverticulum
(1.
6. 2.
3)
Meckel's diverticulum is the congenital remnant of the

omphalomesenteric duct. This protruding appendage or
dilation can be located anywhere within 100 cm of the
ileocecal valve along the antimesenteric border of the
small intestine. Meckel's diverticulum are present in 2o/o
to 3% of the population and is the most frequent congenital malformation of the gastrointestinal tract.
Meckel's diverticulum presentation can vary considerably, mimicking peritonitis, intussusception, and intestinal obstruction or volvulus. Frequently the presence of
gastric mucosa in the diverticulum may result in ulceration. If the ulcer perforates the intestinal lining, signs and
symptoms of peritonitis may develop. Meckel's diverticulum inflammation may also trigger intussusception or
simulate acute appendicitis. The hallmark presentation of
Meckel's diverticulum is a persistent, painless, usually
bright red rectal bleeding in a child.
The initial approach to the patient is threefold: first,
assessment of volume loss and fluid resuscitation, if
required; second, localization ofthe source ofthe bleed;
third, early surgical evaluation. Localization ofthe source
of bleeding from the lower intestine involves endoscopic
visualization, arteriography, exploratory surgery or barium contrast x-ray studies of the small intestine. The gold
standard in diagnosing Meckel's diverticulum is with isotopic scanning (red cell scintigraphy). The diagnosis of
Meckel's diverticulum is generally made clinically by
excluding other causes of the symptoms.

Acute App endicitis ( 1. 6. 3. 1)
Appendicitis is the inflammation
of the vermiform
to have a collagenous layer
appendix. The vermiform appendix is a slender blind-end

tubular structue extending from the proximal portion of
the cecum. Appendicitis is one of the more common presentations of an acute surgical abdomen to the ED. Acute
appendicitis is the most common nontraumatic surgical
located between the surface absorptive cells and the lamina propria.
The most common disaccharidase deficiency is lactase
deficiency. The mucosal lining of the small intestine sim-
emergency in children. Acute appendicitis is usually a

result of bacterial infection secondary to appendiceal
lumen obstruction. The highest incidence of occurrence
is in male patients between ages l0 and20.
patients have been found
36 /
ErrrencrNcv MrorcrNn: Tsn Conn CunrucuLUM
The physical examination is the key to diagnosing

appendicitis. The initial abdominal pain is vague and diffuse, generally periumbilical. Anorexia, with nausea and
vomiting,
is a
common associated symptom. With
increased inflammation the abdominal pain will become

more distinct, sharper in nature, and eventually focused
in the right lower quadrant. Rebound tenderness with
guarding will become more pronounced in the right lower
quadrant. Peritoneal signs may later be accompanied by
signs of a low-grade fever and leukocytosis. Abdominal
pain may be evaluated using the psoas sign (extension of
the right hip) or obturator sign (pain on passive rotation
of the flexed right hip). A rectal and pelvic examination
are mandatory in all abdominal patients.
Differential diagnosis of acute appendicitis includes
ectopic pregnancy, ovarian cyst, adnexal torsion, and
pelvic inflammatory disease. In infants, intussusception
must be considered. Bowel obstruction must also be high
on the differential diagnosis in any acute abdominal pain
presentation to the ED.
Laboratory evaluation can aid in ruling out alternative
with tenderness in the right lower quadrant. Periods of

severe diarrhea may be separated by intervals of constipation. Patients' common presentations frequently
involve anorexia, low-grade fever (or rarely spiking with
chills), flatulence, malaise, and weight loss.
Physical examination often reveals tenderness or a
mass in the right lower abdominal quadrant. Bowel
sounds may be hyperactive. Rectal examination may
reveal occult blood in the stool.
Complications of Crohn's disease are commonly
divided between intestinal and extraintestinal manifestations. Intestinal complications of Crohn's disease include
anorectal ulceration, fissures, fistulas, or perirectal
abscesses. These generally have a higher occurrence in
Crohn's disease patients with colonic involvement.
Obstruction, hemorrhage, anemia, or malabsorption may
develop due to the damage of the intestinal wall. Toxic
megacolon or intestinal perforation are rare complications of Crohn's.
Differential diagnosis of Crohn's disease can be challenging given the various presentations of the patient's
diagnosis. Pregnancy test, urinary analysis, and pelvic
signs and symptoms. The Crohn's disease clinical presen-
cultures are required.

Radiologic images of the abdomen may assist in ruling
out bowel obstruction or perforation. The presence of a
fecalith in the right lower quadrant on the plain abdominal film strongly suggests an appendicitis; however, this
finding is infrequent. Prompt surgical intervention is the
tation will often initially mimic inflammatory large bowel

process of ulcerative colitis. Other diagnoses to consider
at initial presentation include appendicitis, infectious
diarrhea, and lymphoma.
There is no cure for Crohn's disease. Medical treatment
is very similar to that of ulcerative colitis and is empirically aimed at relieving and avoiding the exacerbation of
mainstay of ED management of the acute appendicitis

patient. Prophylactic preoperative antibiotic administration may be preformed in the ED. If recognition of appendicitis is delayed, the complications include abscess formation, perforation, and peritonitis. These complications
carry a higher morbidity and mortality.
Regional Enteritis/Crohn's Disease
(1.
6. 3.
2)
Crohn's disease, also referred to as regional enteritis or
terminal ileitis, is a chronic recurrent segmental inflammatory process of the gastrointestinal tract. Crohn's disease can involve any segment of the intestinal tract from
the lips to the anus, but most frequently involves the distal ileum and the right colon. The underlying etiology of
this disease remains unknown. Histologically, the inflam-
matory process is of a nonspecific granulomatous disease. The inflammatory process
will
show discontinuous
involvement of "skip lesions" of the bowel.

These findings are in contrast to the inflammatory
process of ulcerative colitis, which is restricted to the
large intestine (colon and rectosigmoid) and nonsegmental distribution.
Crohn's disease is characterizedby an insidious onset
of exacerbations and remissions of the variable signs and
symptoms. Patients with Crohn's disease generally complain of intermittent colicky abdominal pain, especially
symptoms.
The antibiotic sulfasalazine is structurally related to

sulfur and aspirin drugs. Sulfasalazine is cleaved in the
colon to its active metabolite 5-aminosalicylate acid (5-
ASA). Sulfasalazine, similarly to steroids, does not

enhance the relief from the acute exacerbations of
Crohn's disease. Sulfasalazine has a better effect on the
disease's involvement in the large than in the small intestine. The slow release of 5-ASA enables the primary
effect to occur in the lower gastrointestinal tract. The topical form of 5-ASA is useful for Crohn's disease cutaneous involvement in the distal colon and rectum.
Corticosteroid therapy effectively suppresses the
inflammatory response of the bowel and systemic manifestations. Treatment lasts for 2 to 4 weeks and then is
tapered off as symptoms permit.
Immunosuppressive therapy of 6-mercaptopurine (6MP) and its active metabolite azathioprine is useful for
the closure of enteroenteric or enterocutaneous fistulas
and abscesses. The immunosuppressive drugs also aid in
tapering the dose of long-term steroid therapy. These
medications have significant side effects that limit their
availability.
No current medications are proven effective in reducing reexacerbations of Crohn's. However, administration
of
sulfasalazine and antidiarrheal medications may
relieve mild exacerbations of Crohn's disease.
AsooN,rrNAL AND GAsTRoINTESTINAL
DIsononRs
37
Infectious Disorders (1.6.4)
troenteritis. The most common presenting complaint is

diarrhea with abdominal discomfort, and may include
Viral (1.6.4.1)
associated vomiting, headache, and fever.

The gram-positive S. qureus triggers diarrhea by exotoxin release and is the most common cause of food poisonlng.
E. coli species, commonly associated with travelers'
diarrhea, causes diarrhea by both direct invasion and exotoxin release. The volume of diarrhea can be severe. The
0157:H'7 strain of E. coli has been indicated in contami-
Viral infections of the small intestine generally occur

worldwide and can affect any age range. The degree of
symptoms may vary with any of the viral agents from
acute viral gastroenteritis diarrheal illness to extraintestinal manifestations. The common clinical findings associated with viral infections of the small intestine include
persistent diarrhea, abdominal cramps, malaise, low-
nated meat products, causing hemorrhagic colitis and
grade fever, nausea, and vomiting.

The two most common viral agents causing infectious
diarrheal syndromes are rotavirus and the Norwalk virus.
Diagnosis is made by antibody-specific identifications of
hemolytic uremic syndrome.

V cholerae secretes an exotoxin choleragen. The gramnegative rod has a polar flagellum that facilitates motility.
The cholera infection is described as severe rice watery
stool samples.
stools with no puss.

S. dysenteriae causes large volumes of bloody diarrhea
with mucus. Shigella secretes shiga toxin, a powerful
exotoxin that promotes binding and invasion into the
Signs and symptoms of rotavirus infection include persistent watery diarrhea and vomiting, which usually
begins 2 to 3 days after exposure. The diarrhea can last
from2 to
12 days. Rotavirus has been associated
with the
development of necrotizing enterocolitis in neonates.

Treatment for the rotavirus infections is supportive.
Patients must maintain an adequate level of hydration.
Attempts at specific vaccine protection against all four

pathogenic serotlpes of rotavirus have been unsuccessful.
Illnesses induced by Norwalk and Norwalk-like viral
agents account for up to 35oh of all acute diarrheal outbreaks in the world. Their outbreaks are generally associated with contaminated food and water. Transmission is
commonly via the fecal oral route. Occurrences are more
prevalent during the winter months. The symptoms, while
not as severe as rotavirus, persists for I to 3 days. Vomit
ing is more prevalent with Norwalk than with the Norwalk-like viruses.
Diagnosis is again by enzyme-linked immunoassay
and radioimmunoassay identification in the stool specimen. The treatment is supportive only.
Bucterial (1.6.4.2)
Numerous bacterial agents can infect the small intestine. Common bacterial species affecting the small intestine are Staphylococcus aureus, Escherichia coli, Vibrio
cholerae, Shigella dysenteriae, Salmonella enteritidis,
Campylobacter jejuni, Yersinia enterocolitica, and

Clostridium dfficile. The incubation period from infection to clinical symptoms for Staphylococcus, E. coli, and
Vibrio arc brief. The remaining organisms have a longer
incubation period in the gastrointestinal tract.
These agents cause diarrhea either by direct intestinal
invasion or by exotoxin stimulation of the intestinal tract.
Direct intestinal invasion causes bloody diarrhea. Exo-
toxin release disturbs the electrolyte balance in
the
intestinal lumen and leads to an osmotic diarrhea.

Poor food preparation, a contaminated food source, or
a fecal to oral transmission will often result in a gas-
intestinal mucosa.
S. enteritidis has hundreds of serotypes that may cause
diarrhea. The diarrhea is described as voluminous, watery
with mucus, and frequently lasts less than a week.
C. jejuni is similar in structure to V cholerae. Campy-
lobacter-induced illness usually begins as fever and

headache followed by bloody loose diarrhea and abdominal cramps.
Y. enterocolilica causes bloody diarrhea by enterotoxin. Yersinlc is self-limited diarrhea and requires no
treatment unless it invades the blood.
C. dfficile-induced diarrhea frequently follows broadspectrum antibiotic administration that wipes out the nor-
mal flora of the gastrointestinal tract and causes

pseudomembranous colitis. This allows C. dfficile,
which is also part of the normal intestinal flora, to proliferate and secrete exotoxin.
The history of other household members with similar
symptoms is highly suggestive of common food poisoning
contamination. Recent traveling is frequently associated
with the source of diarrhea. Clarification of the patient's
stool description is very helpful for detecting any signs of
blood or mucus indicative of an invasive agent.
The physical examination is generally unremarkable.
Major complications involve the possibility of
bac-
teremia and dehydration.
is made by isolating the

Gram stain, culexamination,
offending agent in the stool
Confirmatory diagnosis
ture, and sensitivity. Frequently, polymorphonuclear

leukocytes will be seen on the Gram stain with invasive
infl ammatory causing organrsms.
V cholerae is identified by examining the stool specimen under dark field microscopy. If C. dfficile is thought
responsible for the diarrhea, a special request fot C. dfficile toxin is required by the lab.
Other possible differential diagnosis for gastroenteritis
should be considered for viral or parasitic infection' An
38 /
ENmRcsNcy MnorcrNs:
Tur
Conn Cunnrcuruvr
elderly patient with gastrointestinal bleeding and a nor-
mal stool must be investigated fuither to determine the

source ofbleeding.
Bacterial infections of the small intestine are treated
with supportive rehydration and replenishment of electrolytes. Assessment of fluid status is necessary. Solid
foods are restricted and only oral fluids are administered.
Antispasmodic and antibiotic agents are not indicated in
patients unless symptoms are severe and protracted.
Unnecessary treatment with these drugs will often prolong the carrier state of the individual.
Empirical treatment for severe diarrhea of suspected
bacterial etiology includes initiating fluoroquinolones
(Cipro 500 mg po bid x 3 days) while awaiting stool cultures. If C. dfficile toxin is highly suspected" treatment
regimens consist of either metronidazole (Flagyl 500 mg
po tid x 3 days) or vancomycin(125 mg po qid x 3 days).
If comorbidity or bacteremia is suspected, the patient
should be hospitalized for intravenous antibiotic, and
blood cultures should be obtained. If the source of the
possible contamination
is from a public facility,
the
physician should noti$u the local health authorities.
Parasitic (1.6.4.3)
Parasitic infections of the small intestine are classified
parasites
as helminthic and protozoan. Helminthic
include Nemathelminthes (roundworms) and Platyhelminthes, which include trematodes (flukes) and cestodes (tapeworms). Any type of helminthic infection
causes a eosinophilia response from the host. Mature
helminths do not generate a strong immune response and
may live for years in their human host. However, the
helminth eggs will trigger an immune reaction and may
be distributed throughout the body.
Generally, clinical presentation of helminth infections
include diarrhea, abdominal cramping, weight loss, vomiting, and fever. Severe infections result in malabsorption.
Complications include invasion of the liver, gallbladder,
and bile ducts. Diagnosis is accomplished by identification of helminth eggs or larva in the stool.
Treatment for intestinal nematodes is with any one of
the following agents: mebendazole (Vermox 100 mg po
bid x 3 days), thiabendazole (Mintezol 22 mglkg po bid
x 3 days), albendazole (400 mg po single dose), or pyrantel pamoate (11 mg/kg single dose max
The intestinal roundworms
I gram).
(Nematodes) include
Ascaris lumbricoides, Trichuris trichiura (whipworm),

Enterobius vermicularis (pinworm), Nector qmericanis,
Strongyloides stercoralis, and Trichinella spiralis. A.
lumbricoides, T trichiura, and E. vermicularis infections
are caused by ingestion
of
N. americqms and S.
stercoralis infections result from penetrating filariform
eggs.
larvae through unprotected skin.
E. vermiculans (pinworm) and Z trichiura (whipworm)

do not invade the intestinal tract mucosa, and therefore do
not cause a genenlized eosinophilia. The female E. ver-
micularis (pinworm) usually migrates to the perianal area

at night to lay her eggs. This migration causes perianal
irritation and itching. Children frequently have hand-tomouth reinfection. Diagnosis is aided by examination of
perianal tape to detect for the presence ofeggs.
N. americanis and S. stercoralis larva travel directly to
the lung alveoli, where they are coughed up and swallowed. The worms mature in the gastrointestinal tract.
The Z spiralis infection is traced to the ingestion of
encysted larvae in the contaminated pork meat and is
termed trichinosis. Serum analysis reveals
an
eosinophilia and increased creatinine phosphokinase.

The intestinal Platyhelminthes flatworms include
trematodes (flukes) and cestodes (tapeworms). The main
infectious trematode is Schistosoma Qaponicum and,
mansoni). S. japonicum is common to Eastern Asia, while
S. mansoni is found in South America and Africa. The
schistosome penetrates through skin and invades the
venous system. The larvae migrate to the hepatic portal
venous system, and surround the veins of the intestinal
system. The schistosome lays its eggs, which enter the
gastrointestinal tract for excretion. The patient infected
with the schistosome will frequently complain of skin
irritation and itching at the penetrarion site. Complications of this infection may cause blockage of the portal
system. Praziquantel is the commonly used drug for treatment of the helminths.
C
estodes (tapeworms ) are hermaphrodite s. Tapeworms
live and replicate in the digestive tract of their host, and

lack a true digestive tract of their own. Tapeworm infection generally results from ingestion of a contaminated
intermediate host meat. Examples include Thenia solium
(pork), T. saginate (beef), and, Diphyllobothrium latum
(fish). Tapeworms may become quite long (several
meters) in size. The larvae will invade and deposit in
muscle tissue. The tapeworm continuously sheds eggs
into fecal material. The proper preparation of meats

greatly reduces infections by tapeworms. All tapeworm
infections are treated with praziquantel or niclosamide.
There are four medically important protozoans that can
cause diarrhea infection: Entqmoeba histolytica, Giardia
lamblia, Cryptosporidium, and Is ospora belli.
Protozoa are free-living, single-celled eukaryotic cell
organisms with cellular organelles and cytoplasmic membrane. Protozoa replicate by both sexual and asexual
reproduction. In general, protozoa transform between two
structures. Once the cyst form
of the protozoan
is
ingested, it transforms back to a more motile form called
the trophozoite. The trophozoite permits movement by

the cytoplasmic projections called pseudopodia. These
pseudopodia guide direction ofthe protozoa and allow it
to surround and engulf food. The sizes of protozoavary
AsnoNarNAL AND GASTRqTNTESTTNAL
DrsoruBns
39
from 5 pm to 2 mm. Transmission of protozoa is generally by the fecal-oral route, and is more common in
Tumors (1.6.5)
unsanitary conditions and Third World countries.
Tumors of the small intestine are extremely rare;

approximately 2000 cases occur yearly in the United
States. The suspicion of small bowel cancer is increased
with hereditary or preexisting factors such as long-stand-
E.
histolytica generally causes bloody diarrhea.

Approximately 5% of the United States population are
infected by Histolytica. Most individuals are asymptomatic carriers, who may pass the infective form (cyst) of
Histolytica by fecal-oral transmission.
The trophozoite form of E. histolylica ingests the normal bacterial flora of the intestine wall and sometimes
invades the intestinal mucosa. This invasion causes
bloody diarrhea and abdominal pain.
Treatment of E. histolyticq infections is with metronidazole (Flagyl 750 mg po tid x l0 days).
G. lamblia (giardiasis) affects approximately 5% of the
United States population. Generally the infected patients
are asymptomatic. Outbreaks occur as a result of poor
sanitation conditions contaminating community drinking
water. G. lamblia has also been documented in wildlife as
a common cause of camper's diarrhea, the result of drinking from contaminated streams. The flagellated trophozoite adheres to the small intestine wall. The organism
coats the wall of the small intestine and interferes with fat
absorption. Since the G. lamblia do not invade the

mucosa, they do not cause bloody diarrhea. Instead the
diarrhea is described as very foul-smelling greasy stools.
Diagnosis is made by identification of a cyst or trophozoite in the stool specimens. Treatment of G. lqmblia
infection is with metronidazole (Flagyl 250 mgpo tid x 5
days). Improving sanitary conditions is also part of preventatrve treatment.
The protozoa Cryptosporidium parvium
(cryp-
tosporidiosis) and Isospora belli (isosporiasis) are more

common diarrhea infectious causes in immunocompromised individuals.
The symptoms of abdominal cramping and severe diarrhea are generally self:limited in the immunocompetent
patient. The watery diarrhea can be protracted with evacuations up to 15 L a day. This may subside and the
immunocompromised patient may suffer frequent periods
of recurrence.
Currently there is no effective treatment regimen for

Cryptosp oridium-induced diarrhea. Supportive measures
include nutrition, fluid" and electrolyte replenishment.

I. belli infection generally causes abdominal cramps,
low-grade fever, vomiting, and anorexia. Infrequently the
diarrhea may recur or become protracted for months,
causing malabsorption, steatorrhea, and weight loss. The
diagnosis by identification ofan oocyst is often difficult
because of the sparse amount found in stools.
The clinical course in immunocompetent patients is
self-limited. However, in immunocompromised patients
this protozoa organism is treated using trimethoprim with
sulfamethoxazole (Bactrim DS po qid x l0 days then bid
for 3 weeks).
ing Crohn's disease.

There are no direct signs or symptoms to suggest the
presence of small intestine neoplasms. Their discovery is
usually the result of a secondary presenting complaint.
The earliest manifestations of small bowel tumors are
subtle and may vary from persistent peptic ulcer disease
to occult gastrointestinal bloo4 malabsorption, or intestinal obstruction.
Adenocarcinomas are the most common cancer of all
the small intestine tumors, and the prognosis is very poor.
Adenocarcinomas generally occur in the proximal small
bowel. There is an increased incidence in the long-standing Crohn's disease patient.
Carcinoid tumors develop from argentaffin cells and
frequently occur in the distal small bowel. Carcinoid syndrome of the small intestine is rare and usually does not
develop until after metastases. Carcinoid syndrome can
result from carcinoid tumor cells that secrete 5-hydroxytryptophan. These secretions result in the systemic manifestations of paroxysmal flushing, dyspnea and wheez-
ing, recurrent abdominal pain and diarrhea,
and
symptoms of right-sided valvular heart disease.

The diagnosis of carcinoid syndrome is confirmed by
urine levels of 5-hydroxyindoleacetic acid (5-HIAA), an

active final metabolite of 5-hydroxytryptophan. With carcinoid tumors the metastases are generally too widespread for the benefit of surgical resection. The tumor is
slow growing but the prognosis is poor.
If cancer of the small intestine is suspected, several
diagnostic studies may be employed. These include upper
gastrointestinal radiologic series and endoscopy for

biopsy of abnormal pathology. CAT scan imaging of the
abdomen is required for staging of the neoplasm. Abdominal ultrasound may be required for jaundice secondary
to tumor mass obstruction of biliary outflow common
bile duct.
The definitive treatment for either benign or malignant
tumors of the small intestine is surgical resection. Radiation and or chemotherapy is reserved for tumors that have
advanced past the bowel wall.
Vascular Disorders (1.6.6)

Mesenteric Ischemia (1.6.6.1) and
Ischemic Colitis (1.6,6,2)

Gastrointestinal ischemia is more prevalent in elderly
patients with a previous history of vascular disease, such
40 /
EnmncnNcv MporcrNs: Tnr, Conn CunnrculuM
as coronary artery or peripheral vascular disease. The
LARGE BOWEL (1.7)
advanced atherosclerotic lesions compromise blood flow

to the vessels of the intestinal mucosal wall.
The colon, or large intestine, is the hollow tubular
lntestinal ischemia clinical onset is very rapid, with

aching abdominal pain usually occurring shortly after a
meal. The ischemic episodes can resolve very quickly or
may progress to cause bowel necrosis. Patients may give
a history of similar episodes occurring intermittently for
weeks. There may be some associated nausea and vomiting and, with the presence of acute ischemic colitis, gross
structure that constitutes the final portion of the alimentary canal. It begins at the ileocecal junction, extends to
the anus, and averages l 5 m in length. Approximately 12
cm of the distal colon is designated the rectum. The rectum has a separate arterial and venous system and is discussed in the next section. The colon absorbs up to 70%o
of the water from the fecal stream so that liquid stool
bloody diarrhea.
becomes semisolid. The distal colon and rectum also

serve as a fecal reservoir, allowing convenient defecation.
It is important to remember that the colon is not a vital
organ and many individuals lead long and productive
lives after colectomy. Newer surgical techniques allow for
ileocanal anastomoses so that many patients who have
undergone colectomies do not have ileostomies. Bleeding, colitis, diarrhea, constipation, irritable bowel syndrome, diverticulitis, obstruction, and neoplasia are commonly encountered (and often overlapping) colonic
disorders. This section reviews common problems associ-
Patients with acute ischemia may show rebound tenderness and rigidity on physical examination secondary
to irritation of the peritoneum. Patients with
chronic
ischemia have a tendency to have an increased volume of

stool and may develop malabsorption of nutrients and
fat.
Mesenteric angiography may reveal areas of vascular
deficiency to the intestine. Barium contrast images may
demonstrate edema of the mucosal folds, similar to that
seen with Crohn's disease. After the patient's ischemic
episodes subside, the abnormalities seen on barium studies may remain for a few days. A residual stricrure may
develop at the ischemic site.
Initial management consists of supportive measures
including antibiotics. Long-acting vasodilators may be
beneficial. Acute ischemia episodes generally resolve on
their own. Treatment of suspected intestinal ischemia
requires surgical evaluation for possible salvage ofbowel
necrosis and arterial bypass graft. Exploratory abdominal
surgery is indicated for the suspected infrequent complications of extensive infarction or bowel perforation. The
typical ischemic colitis patient is often a poor surgical
candidate.
SELECTED READING
Addiss DG, Shaffer N, Fowler BS, et al. The epidemiology of appendicitis and appendectomy in the United States.,4zr J Epidemiol 1990;132:
9l 0.
Bayless TM, Knox DL. Whipple's disease: a multisystem infection. NEngl
J Med 1979;300:920.
Flewett TH, Woode GN. The rotaviruses. Arch Virol 1978;57:1.
Grendell JH, Gore RM, Ballantyne GH. Vascular diseases of the bowel. In:
Sleisenger MH, Fordtran JS, eds. Gastrointestinal disease, 4th ed.
Philadelphia: Saunders, 1988.
Lambden PR, Caul EO, Ashley CR, et al. Sequence and genome organization of a human small round-structured (Norwalk-like) vints. Science
1993:'259:516.
Michelassi F, et
a'1. Primary and recurrent Crohn's disease: experience with

1379 patients. Ann Surg 1991;214:230.
Silen ML, Tracy TF Jr. The right lower quadrant "revisited." Pediatr Clin
North Am 1993;40:1201.
Thompson JN, Hemingway AP, McPherson GAD, et al. Obscure gastrointestinal haemorrhage of small-bowel origin. .Br Med J 1994;288:
1663.
Trier JS. Celiac sprue (medical progress). N Engl J Med 1991;325:12009.

Webster DP, Schneider CBN, Cheche S, et al. Differentiating acute appendicitis from pelvis inflammatory disease in women of child bearing age.
Am J Emerg Med 1993;11:569.
ated with the colon.
Bleeding
Bleeding is a common presentation of colonic pathology. It can manifest in a variety of ways from guaiac
positive stools with or without anemia to massive acutely
life-threatening hemorrhage. Visible bleeding routinely
prompts patients to seek medical attention. Chronic lowgrade blood loss may portend a worse outcome eventually, but evaluation and diagnosis are frequently delayed
when bleeding is occult. The location of the bleeding
site, the volume of blood lost, and the rapidity with
which it is lost determines the form that the blood takes
as it passes out through the rectum and anus. Melena
(digested blood) often occurs as a result of upper gastrointestinal (UGI) bleeding but can result from a proximal colonic bleed of lesser volume and slower transit
time. Mahogany stools (partially digested blood) or
bright red blood per rectum frequently denote a lower
gastrointestinal (LGI) source of blood loss but can occur
with a brisk and massive UGI bleed as well. Smaller
amounts of bright red blood per rectum in a hemodynamically stable patient suggests a colorectal source.
Hemorrhoidal bleeding is the most common form of
lower gastrointestinal bleeding.
As in all gastrointestinal bleeding, the first step is to
determine whether the patient is hemodynamically stable,
and if not, to resuscitate the patient immediately. An
attempt to quantitate the amount of blood lost should be
made through questioning. If bleeding continues, direct
observation is helpful. Vital signs, including orthostatic
blood pressures, should be obtained immediately. Mental
Asoor\dtNAr- AND GAsTRoTNTESTTNAL
confusion, cool clammy skin, and hypotension suggest

major blood loss. Clinicians should not be misled by
"normal" hematocrit readings during any acute bleeding
episode. The plasma volume can take 24 hours to equilibrate after large blood volume losses. A patient can
exsanguinate acutely despite a "normal" hematocrit.
Fluid resuscitation is indicated with any significant
bleeding. Blood transfusions should be considered in the
settings of significant anemia (significance may vary due
to comorbid conditions), shock, and gross ongoing hemorrhage. If a patient is experiencing melena, mahogany
stools, or bright red blood per rectum, with hypotension
and an UGI bleed suspected, gastric lavage with room
temperature water is recommended. Lavage water
returned with bile staining, but without blood" argues
against an active UGI bleed. The patient's coagulation
status should be assessed and an effort to correct any
coagulopathy detected should be considered.
Once resuscitative measures have been started, an

effort can be made to diagnose the underlying problem,
find the specific bleeding site, and control it directly if
possible. Chronic bleeding in stable patients and small
amounts of bright red blood per rectum associated with
bowel movements can be handled definitively during follow-up after the ED visit. Anoscopy in the ED may confirm hemorrhoids but follow-up should be arranged to
make certain that they stop bleeding and that they represent the sole source of the blood loss. Significant, symp-
tomatic anemia
will
generally require admission for
transfusion. Ongoing larger volume bleeding requires an

aggressive diagnostic approach beginning in the ED.
Colonoscopy may be attempted in the setting of an active
bleed. The unprepped colon with retained stool and blood
can obstruct the endoscopist's view. If possible, the colon
should be prepared using a standard oral lavage solution.
In ongoing massive GI bleeds (both upper and lower)
arteriography is used to localize the site of the bleeding,
and when possible, to stop it via embolization of the culprit artery. Subsequent endoscopy may be required to
determine the precise reason for the hemorrhage, but this
can be arranged subsequently in a controlled seuing.
Luminal contrast studies, such as barium enemas, are not
indicated in the evaluation of acute LGI bleeding. Even
when they detect potential pathology they do not demonstrate that the lesions are actually bleeding. Furthermore,
they obstruct the views of both the angiographer and the
endoscopist. In the case of massive ongoing bleeding in
an unstable patient, surgery may be indicated. Angiogra-
Drsonnnns
/ 4l
tis, radiation colitis, and ischemic colitis. Anorectal

bleeding is discussed elsewhere. Diverticulosis is common in Western societies with refined diets low in fiber.
Diverticula are herniations of the mucosa and submucosa
through the muscularis propria that result in outpouchings of variable size in continuity with the colonic lumen.
Arterioles in the mucosa encounter sharp angles at the
openings ofthese outpouchings. It is thought that bleeding occurs when sheer forces cause arteriolar rupture at
the edges of a diverticulum. Bleeding is often brisk,
bright red or mahogany in color, painless, and self-limited. Arteriovenous malformations are aberrant connections between arterioles and venules that largely bypass
capillary beds. They may be congenital or acquired. Their
pathogenesis remains a matter of speculation. They can
present in a variety of ways including occult bleeding,
massive bleeds with no warning, and at times as recurrent
gastrointestinal bleeding of obscure etiology. Similar to
diverticular bleeds, AVM bleeding is painless and usually
self-limited. Both conditions may be suspected while

definitive proof remains frustratingly out of reach. Arteriography may suggest AVMs, but without active bleeding during the study there is no proof they are actually
for the hemorrhage. Likewise, endoscopy

may reveal that a patient with recent colonic bleeding has
responsible
either diverticula or AVMs, or both, but if no visible

bleeding site or adherent clot is detecte4 the diagnosis
will remain "likely" while not actually confirmed. When
the patient is hemodynamically stable and the bleeding is
self-limited, it is generally advisable to prepare the colon
properly for endoscopic evaluation rather than attempt
emergent or urgent unprepared studies that ultimately
prove to be suboptimal.
When colorectal neoplastic lesions bleed, they usually
do so because they have outgrown their neovascular
blood supply and tissue necrosis with sloughing results.
Occasionally they serve as the lead point for intussusception wherein colonic mucosa is pulled into the more dis-
tal lumen, resulting in ischemia with tissue loss and

bleeding. Bleeding is often occult but can become massive if a major vessel is disrupted. Weight loss, abdominal pain, and symptoms of anemia are common complaints heard from patients suffering from colon cancer.
Such patients may never be aware that they have been
bleeding.
Causes of colonic bleeding include diverticulosis, arteriovenous malformations (AVMs), neoplasia (benign and
A rare cause ofgastrointestinal bleeding that should not

be overlooked is that which results from aortoenteric or
ilioenteric fistulae. These life-threatening conditions may
present with smaller, temporarily self-limited "herald"
bleeds before potential exsanguination. A patient with aortic or iliac artery aneurysms, or who has undergone
repairs of such aneurysms, should be considered at risk
for such an occurrence. The distal duodenum is the most
frequent site for these fistulae, but other portions of the GI
malignant), inflammatory bowel disease, infectious coli-
tract may be involved. Angiography is recommended.
phy and
endoscopy have lessened the need for

exploratory surgery in this setting. Surgery may be the
definitive corrective action once the problem has been
diagnosed.
42 /
EunRcrNcy MnorcrNp: Ttrn Conr Cunxrculurvl

Irrituble Bowel Syndrome
(1.
7.
l. 1)
The irritable bowel syndrome (IBS) may affect as

much as a third of the population, although only about
5%o of the population actually seeks medical care. Oversensitivity to bowel distention and disordered bowel
motility are theorized to be the basis for the syndrome.
Dyspepsia, bloating, abdominal cramping, diarrhea, con-
stipation, and alternating diarrhea and constipation are

common manifestations of IBS. Although IBS prompts
many visits to health care providers, including ED usage,
it is never fatal. It can occur concurrently with other conditions, for example, IBD. Unfortunately, it still remains
largely a diagnosis of exclusion, although investigators
have proposed diagnostic criteria. A single visit to the ED
may prompt suspicion, but is not enough for a definitive
diagnosis. Lack of bleeding, weight loss, and constitutional symptoms, as well as a chronic course, suggest the
diagnosis. It is also common to see the symptoms (especially cramps and diarrhea) resolve when the patient
sleeps. Patients presenting with changes in bowel habits
of recent onset should not be given the diagnosis without
a thorough evaluation for other causes. Although the ED
can offer attempts at short-term symptomatic relief, it can
probably do the most for patients with IBS by ensuring
that they are referred for follow-up to physicians familiar
with IBS who will provide the appropriate long-term
glion cells leads to lack of proper motor function) are

organic causes for the syndrome of constipation. Anticholinergics, opiate narcotics, alumina-based antacids,
and calcium channel blockers are coflrmon offenders. A
careful history and physical examination should seek to
elicit a medication profile, a family history of constipation, and the specific details of the problem per the
patient, as well as looking for signs of systemic illness
and localized disease. Painful defecation should prompt
anoscopy and,/or proctoscopy. Treatment of an anal fissure, for example, could lead to correction of resultant
constipation. A barium enema or colonoscopy will rule
out obstructing lesions. A barium enema would be more
cost-effective unless there is concomitant bleeding. If the
history physical examination, blood glucose, thyroid
function tests, proctoscopy, and barium enema are not
revealing, the patient can usually be managed with
increased dietary bulk and occasional laxatives. Cathartic
laxative use on a regular basis may lead to neuromuscular degeneration in the colon, and is therefore not recommended. If simple measures do not work, referral to a
gastroenterologist for more extensive evaluation is recommended. In the ED constipated patients must be dis-
tinguished from those who are obstructed. The history

and physical examination as well as abdominal x-rays
usually suffice.
Obstruction (1.7.1.4)
cate.
Constiputio n (1. 7. l. 2, 1. 7. 1. 3)
Constipation refers to decreased frequency of bowel

movements, increased stool firmness, or difficulty with
stool passage. Soft but solid stools are generally passed
painlessly and with minimal straining from once every 3
days to three times per day. The variability of patient perception as to what constitutes constipation is impressive.
Some individuals become concerned with mild increases
in stool solidity or after missing a single bowel movement. Others will not defecate for days or even weeks
prior to seeking care. Two or three million United States
medical visits per year result from constipation. Most

such visits are not emergent. Short-term, self-limited
constipation may result from changes in diet (usually
decrease in dietary bulk), travel and immobility, intercurrent illness, certain medications, or even changes in daily
schedules that disrupt stooling habits. Irritable bowel syndrome, which is often considered functional in nature, is
a common cause of chronic or recurrent constipation.
This will be discussed below. Medications, hypothyroidism, diabetes mellitus, colonic inertia, mechanical
obstructions, anorectal disorders, and Hirschsprung's disofcolonic gan-
ease (a congenital disorder in which lack
Obstruction of the lumen of the colon can occur as the

result of an intraluminal mass, kinking of the colon itself
with a pinching off of the lumen, or external mass compression. The last is the rarest. Colony masses are most
often tumors, although bezoars can occasionally obstruct
the ileocecal valve area. Severe constipation, obstipation,
results in generalized obstruction to forward flow of the
fecal stream. Adhesions, hernias, intussusception, and
vohulus (a twisting of the bowel with subsequent luminal compromise) are common causes of colonic obstruction. Rarely extracolonic tumor bulk will be great enough
to obstruct the colon. Some tumors, such as adenocarcinomas of the prostrate, can grow into the colon and either
bleed or obstruct. Colonic obstruction presents with
abdominal pain, bloating, decreased or absent defecation
and passage offlatus, and variable bowel sounds. Hyperactive bowel sounds are often noted earlier in the course
with diminished or absent bowel sounds when ischemia
or infarction ensues. Longer standing cases may be
accompanied by nausea and the vomiting of feculent
material. Urgent abdominal x-rays reveal dilated bowel
loops, air-fluid levels, thumb-printing (with ischemia),
and free intraperitoneal air ifthere has been a perforation.
Intravenous fluid resuscitation should be started promptly
and surgical consultation sought urgently. When there is
perforation broad-spectrum antibiotics are also neces-
Asno\dINAr eNn GesrnorNTESTrNAr DrsoRorRs

sary. Occasionally a volvulus, especially one involving
the sigmoid colon, can be reduced endoscopically or by
the administration of an enema. Most often complete
bowel obstruction requires operative decompression. Partial bowel obstruction can present with paradoxical diarrhea as mentioned above.
D
iverticulitis (1. 7. 2. I )
Diverticulitis is a common inflammatory lesion of the

colon originating from diverticula. Diverticula either
bleed or become infecte4 but not both. In Western societies diverticula are more common in the left colon,
hence so is diverticulitis. The precise reason for localized
inflammatory lesions arising from diverticula is
unknown. Abscesses can develop with luminal obstruction and/or perforation with generalized peritonitis. Muscular hypertrophy of the colonic wall adjacent to diverticula and diverticulitis can cause further luminal
compromise. Abdominal pain (often in the left lower
quadrant) with fever, nausea, and vomiting are common
presenting symptoms. Laboratory analysis must include a
complete blood count with differential. Mild diverticulitis can be evaluated with a water-based contrast enema.
Sicker patients can be evaluated by CAT scan. Endoscopy
suspected diverticulitis
due to the risk of perforation. Bowel rest, hydration, and
broad-spectrum antibiotics (including anaerobic coverage) are the mainstays of therapy for diverticulitis. The
is relatively contraindicated in
microbiology mirrors that of the gut flora. Some

abscesses can be drained percutaneously if they do not
respond to conservative measures. Perforations, strictures
(especially with significant obstruction), and larger,
refractory abscesses require surgical resection. Surgical
management may require more than one step if there has
been significant peritoneal contamination. Increasing
dietary bulk is suggested as a means to forestall the
development of diverticula and their complications.
Colitis (1.7.3)
Colitis refers to inflammation of the colon. In general,
the term implies that the lesion involves the mucosa or
arises from it. Typhlitis is the much rarer condition in
which the inflammation involves the serosa of the colon
principally. The etiologies of colitis include infectious
agents (including C. dfficile), radiation, ischemia, and
idiopathic inflammatory bowel disease (IBD) (Crohn's
colitis and ulcerative colitis). Symptoms of colitis include
diarrhea, which may or may not be bloody secondary to
frank mucosal disruption, abdominal pain and cramps,
fever, nausea, and vomiting. Abdominal tenderness,
fever, and guaiac positive stools are common signs. The
amount
of bleeding that results from colitis
depends on the intensity
generally
of the mucosal disruption and
43
the surface area involved. It can range from occult bleeding to massive life-threatening hemorrhage, although the
latter is much less common. Unlike diverticular or AVM-
related bleeding, prodromes are common with colitic
bleeding. Infectious colitis may occur in outbreaks

involving several people or more. Contaminated food and
water may spread bacterial infections. Parasitic colitis in
the United States generally means E. histolyticc, and is
seen most frequently among homosexually active men,
those institutionalized due to developmental handicaps,
and those who have traveled to areas where parasites are
endemic. Antibiotic-related diarrhea may involve the
overgrowth of bacteria that leads to diarrhea. C. dfficile
is a facultative anaerobe that can cause syndromes ranging from mild diarrhea to toxic megacolon and shock. C.
dfficile should be suspected in patients with colitis who
have received antibiotics up to 8 weeks prior to presentation. Diarrhea, fever, malaise, nausea, and vomiting usually precede bleeding from colitis. Ischemic colitis occurs
most often in individuals with significant atherosclerotic
disease. On occasion, young patients with fibromuscular
dysplasia of the major intestinal arteries will suffer
ischemic events. The onset of severe abdominal pain fol-
lowed by bloody diarrhea is the classic presentation.

Radiation enterocolitis requires prior exposure to radiation, usually in therapeutic settings, but occasionally as
the result of an industrial accident. It involves small vessel ischemia, although the resultant diarrhea may or may
not be bloody.
IBD usually leads to recurrent episodes of active colitis with diarrhea with or without bleeding. Malaise,
anorexia, weight loss, and extraintestinal manifestations
including arthritis, cholangitis, skin lesions, and inflammation of ocular structures are seen in IBD patients as
well. Ulcerative colitis presents as bloody diarrhea (unless
an extraintestinal presentation occurs first). Crohn's disease may cause bloody or nonbloody diarrhea or manifest
chiefly as abdominal pain or weight loss. Children may
experience failure to thrive. Careful attention should be
paid to searching for symptoms and signs of IBD that are
extraintestinal. A rare complication of colitis is toxic

megacolon. The colon dilates, becomes paralytic, and if
untended, will often perforate. This surgical emergency is
accompanied by serious constitutional symptoms that can
include shock. Colectomy is usually required.
Gay bowel syndrome is a term, being used less often
more recently, that refers to the sexually transmitted proctitis that occurs with anal intercourse. Gonorrhea, parasites, and other venereal proctidites are seen in greater
numbers among sexually active homosexual males. They
are not confined to this population, however, and all
patients with proctocolitis should have their sexual practices reviewed while being evaluated.
Patients who present to the ED with symptoms and
signs of colitis should have a thorough history and examination performed. Particular attention should be paid to
44 /
EnrncnNcy Mr,orcrNn: Tsn Conn Cunnrculurvr
food and water consumption, travel, sexual practices, the

health of surrounding individuals, past similar histories,
and comorbidities. Volume depletion should be corrected
and hemorrhage managed as needed. Stool should be sent
for white blood cell counts (WBCs), culture and sensitiviry, C. difficile toxin titers, and (if the history is suggestive) ova and parasite examinations. Endoscopy may be
required when ischemia is suspected but should be performed by a fully trained endoscopist. Flexible sigmoidoscopy is often useful for the diagnosis of other types of
colitis. Self-limited colitis (especially with a positive
stool culture) does not require an invasive workup. Treatment is aimed at the underlying cause. Antibiotics are
usually unnecessary in bacterial infectious diarrhea
unless there is systemic illness. Metronidazole is used for
C. dfficile and E. histolytica.Ischemia requires supportive medical care and often, surgical care. Radiation coli-
tis is usually an insidious
disease
with a frustrating
response to therapeutic attempts. Rarely, massive bleeding requires surgery. IBD is managed with S-aminosalicylates and steroids. Patients with IBD are not immune to
bacterial, parasitic, and clostridial infections. Increased
disease activity in patients with IBD may represent such
infections. Stool studies and endoscopy may be considered at such times. Significant volume depletion, serious
hemorrhage, evidence of sepsis, metabolic derangement,
and ischemic colitis warrant admission. Milder cases of
colitis may be handled in the outpatient setting as long as
medical care is readily available.
150,000 cases of colon cancer per year occur in the

United States alone. Surgical removal affords the only
true hope of cure, although adjuvant chemotherapy after
surgery in stage C improves 5-year survival significantly. Most colon cancers form in previously benign
adenomas. The type and size of the underlying adenoma
determine subsequent malignant potential. Certain fam-
ilies are genetically prone to developing adenomas and

adenocarcinomas of the colon. Screening regimens have
been developed to prevent the development of frank
colon cancer. These are not performed in the ED. However, guaiac positive stools, unexplained anemia, or a
rectal adenoma found on proctoscopy should prompt a
referral as well as education of the patient as to the
importance of follow-up. Most carcinomas occur in
people over the age of 50, but familial syndromes occur
at earlier ages. Juvenile polyps have little or no malignant potential but may bleed and result in their disclosure. Hyperplastic polyps have no malignant potential
and are usually found incidentally during endoscopy or
contrast radiologic studies. Histologic review is required
to determine the significance of colorectal neoplastic
lesions. Elective endoscopy is the means by which the
tissue is sampled" although surgery may be required for
lesions unreachable or unresectable by endoscopic technlques.
SELECTED READING
Although diarrhea is a prominent symptom of colitis,

it has many causes. Diarrhea is technically defined
as the
passage of greater than250 g of stool per day. Practically,
it refers to more frequent stooling, greater stool volume,

greater liquidity of the stools, or a combination of these
symptoms. Colonic diarrhea is usually lower volume and
higher frequency than that caused by small intestinal

it may be bloody or "bland." In the
ED it should be determined if volume or blood loss (or
both) will require hospitalization. Stool studies should be
sent as appropriate. Attention should be paid to any medpathology. As above
ications the patient might be taking that could cause diarrhea. Self-limited viral infections are a common cause of
diarrheal illnesses in children and adults. Adequate fluids
and antidiarrheal drugs maintain adequate volume status
and minimize symptoms. Diarrhea may also occur paradoxically as a result of a partial obstruction with "over-
flow" incontinence. Relief of the obstruction restores

normal bowel function. Obstipation may require manual
disimpaction, enemas, and, in extreme cases, surgical
decompression.
Tumors (1.7.5)
Colonic neoplasia is very common in the United

of the world. Over
States and other developed nations
Blacklow NR, Greenberg HB. Viral gasrroenteritis N Engt J Med I99l:

32s(4):2s2-264.
Bleday R, Falchuk ZM. Diagnosis and treatment of constipatron. Comp
Ther 1994;20(l):4449.
Ellis DJ, Reinus JF. Lower intestinal hemorrhage. Crit Care Clin 1995;
I 1(2):369-389.
Freeman SR, McNally PR. Diverticulitis. Med Clin North Am 1993;77(5):
1149-1167.
Guenant RL, Bobak DA. Bacterial and protozoal gastroenteritis. N Engl J
Med t 99 1 ;325(5 ):327 -j40.

Lynn RB, Friedman LS. Irritable bowel sy.ndrome. N Engl J Med 1993;
329(26):1940-t945.
Moriarty KJ. The irritable bowel syndrome. Br Med
1992;304:
1166-1169.
Podolsky DK. Inflammatory bowel disease (two parts). .lr'Ez gl J Med 1991;
325(13):928-937; 1991,;325(14): 1 008-1 0 I 6
RECTUMANDANUS (1.8)
The anorectum is the most distal portion of the lower
GI tract, originating at the terminus of the sigmoid colon
and ending at the outside world. It lies retroperitoneal, is
supplied from the portal and systemic vascular distributions through the superior, middle, and inferior hemorrhoidal arteries, and is innervated by the pudendal nerve
and the autonomic nervous system. It serves as the conduit for colonic solid, liquid, and gaseous waste, and vol-
untary control of this expulsion is by the internal and

external anal sphincters (motor level S2-S4).
Asoo\,rNAr AND
Anal Fissure (1.8.1.1)
An anal fissure, also referred to as fissure in ano, is the
most common source of painful rectal bleeding. It also is
the most common anorectal pathologic process in infants
and children.
An anal fissure is a linear tear of the
anal
canal occurring in the midline. Cases in which the appearance of an anal fissure occurs other than in the midline
should be referred for evaluation of other etiologies such
as inflammatory bowel disease (see below), syphilis,
leukemia, tuberculosis, and neoplasms. The majority of
cases in the midline are found posteriorly. Anterior fissures, when present, are most commonly seen in women.
Anal fissures are generally the result of excess distention of the anus and are most typically the result of either
the passage of large hard stool or the insertion of large
objects, such as foreign bodies. Anal fissures have been
reported following episodes of diarrhea. Acute anal fissures are generally very shallow and not associated with
a sentinel pile. The sentinel pile is a region of swollen
excess skin superficial to the crater of the chronic anal
fissure and given the similariry of appearance, often confused with an external hemorrhoid. Prior perianal surgical intervention may induce greater risk for fissure. The
most frequent complications of anal fissures are infection
and stricture.
The patient usually presents following sharp pain associated with the passage of stool. Small amounts of blood
may be seen in superficial fissures with greater amounts
in deeper craters. With more chronic disease, the pain
may persist for some time postdefecation, a discharge
may be seen, itching can occur, and the history of pain
with intercourse may be elicited. Particular care must be
taken to ensure a gentle examination, as the anal sphincters may go into spasm secondary to pain, resulting in
Iimitation of further examination.
Treatment includes the use of bulk laxatives and stool
softeners to normalize stool consistency. Good hygiene,
such as with sitz baths, and surveillance for infection are
also very important in the care of the patient with an anal
fissure. Care must be taken in the prescription of analgesics to avoid the side effect of constipation, which will
impair recovery. The use of topical ointments is to be discouraged as they may retard healing. Deep chronic fissures, those with complications, and those away from the
midline require surgical referral.
Anorectal Fistula
(1.8.
1.
2)
The anorectal fistula, or fistula in ano, is an abnormal

tract connecting the anal canal and the skin. The majority
of cases, 800/o, have a history of resolved anorectal
abscess. Other predisposing etiologies include inflamma-
GASTRoTNTESTTNAT
DrsonnrRs
45
tory bowel disease (see below) and tuberculosis. Infection

of anorectal fistulae is common and they are particularly
prone to abscess formation. Fistula in ano should not be
confused with other fistulae including vagino-cutaneous
and diverticular-cutaneous fistulae.
Patients typically complain of a discharge staining
undergarments. Occasionally they may provide a cyclic
history of discharge, quiescent period after cutaneous
healing, progressive pain and tenderness during abscess
formation, purulent drainage with abscess rupture, and
back to the foul-smelling discharge associated with fistula in ano, thereby completing the cycle. Examination
during the fistulous phase will reveal the draining anocutaneous tract, which may be palpable during digital
rectal examination. A probe can be passed through the
origin to assist in definition.

Management is elective outpatient surgical repair. In
the ED any persistent local abscess should be incised and
drained. Antibiotics are not felt to be of benefit in uncomplicated cases. Significant cellulitis, particularly in the
immunocompromised warrants admission. Deaths from

sepsis arising from ano-cutaneous fistulae are reported.
In cases where the tub soaks and sitz baths used for
hygiene are not sufficient to reduce inflammation, systemic pain control may be required.
Hemorrhoids (1.8.1.3)
Hemorrhoids are defined as varicosities of the venous
plexus, which lies in the wall of the anal canal. They are
classified with regard to their position of origin with
respect to the pectinate line. Those that arise from the
submucosal space proximal to the pectinate line are covered with columnar epithelium and are defined as internal hemorrhoids. The hemorrhoids that emerge from the
skin distal to the pectinate line are covered with squamous epithelium and are known as external hemorrhoids.
Hemorrhoids are the most common cause of minor rectal
bleeding. Etiologic factors may include pregnancy, straining with stool, paroxysmal forceful coughing, prolonged
sitting, increased intraabdominal pressure, diet, and

hepatic dysfunction (internal only).
Internal
Internal hemorrhoids generally do not thrombose. The
anal mucosa is not innervated with pain afferent fibers
and thus internal hemorrhoids are painless unless they
prolapse and cause anal distention. Profound internal
hemorrhoids are noted with portal hypertension, as they
are one of the three sites of portal-systemic anastomosis.
The patient with internal hemorrhoids is often asymptomatic, and does not present until bleeding or prolapse
occurs. The patient with prolapse may notice bleeding,
itching, and discharge. Bleeding from internal hemor-
46 /
EnrnncsNcy MrorcrNr: Tnn Conr CunnrculuM
rhoids is noted on the surface of stool, on toilet tissue,

and in the bowl, but is not mixed with formed stool.
Asymptomatic internal hemorrhoids may be discovered
during anoscopy. Nonprolapsed internal hemorrhoids
may or may not be palpable on digital rectal examination.
Patients with extremes of age; history of heavy bleeding;
or signs of anemia such as pale skin or loss of color in
palmar creases, gums, or conjunctiva should have determination of their hemoglobin and hematocrit in the ED.
The ED therapy of internal hemorrhoids consists of

conservative measures such as bulk laxatives. The definitive treatment is surgical. In the event of prolapse of an
internal hemorrhoid, reduction in the ED is mandated to
prevent strangulation and mucosal ulceration. Internal

hemorrhoids should not have excisional or incisional
therapy even
if thrombosed.
External
itories are painful to place and are placed proximal to the

site ofpathology, thereby reducing their potential benefit.
Nonconservative measures in the treatment of thrombosed external hemorrhoids include incisional therapy,
excisional therapy, rubber band ligation, sclerosing injection, and cryotherapy. Of these modalities only incisional
therapy is appropriate for the ED. The procedure for exci-
sion
of
thrombosed external hemorrhoids
described in Table
l-10.
is
fully
Some recent authors have suggested that, although excision of thrombus provides
enhanced comfort and symptomatic relief, it does little to
improve the time course of the disease and does not warrant the enhanced risk of infection. Many external hemorrhoids do rethrombose after excision. Excision is felt to
be of greatest benefit in the patient who presents in the
first 48 hours following the onset of symptoms.
Rectsl Prolapse (1.8. 1.4)
Although external hemorrhoids are most common in

young adults, they do afflict all age groups. The patient
with an external hemorrhoid will complain of
severe
pain, a fullness in the region, and itching. External hemorrhoids are purple in color, covered with skin, and easily visible.
External hemorrhoids should be treated with conservative measures, unless they are thrombosed. Conservative
measures in the treatment of nonthrombosed external

hemorrhoids are as follows: normalization of stool consistency, such as with bulk laxatives; sitz baths; and
behavior modification, to reduce risk factors for development. Care should be taken to avoid liquidization of stool
as this is associated with cryptitis, which may progress to
abscess, fistulae, or sepsis. Topical agents are of limited
value and should be avoided in most cases. Anal suppos-
TABLE 1-1O. Procedure for incisional therapy of

thrombosed external hemorrhoids
Proper positioning and exposure
Local infiltrative anesthesia; with larger hemorrhoids a ring
block may be required
Skin preparation and cleansing
lncise over the hemorrhoid directed radially to the anus only
through the cutaneous layer; elliptical incisions are
preferred, but triangular or linear incisions are
acceptable; linear incisions are likely to seal early and
rethrombose more quickly; stab incisions are associated
with a higher rate of infection
Removal of clot(s) with pickups and/or tangential pressure
Packing of the region with gauze or Gelfoam followed by
generous padding and tape
Adequate analgesia; select narcotics that are less
constipating
Frequent warm sitz baths
Good hygiene
Bulk laxatives
Appropriate referral
Rectal prolapse, orprocidentia, is the extrusion ofrectal tissue distal to its normal position. Rectal prolapse can
be divided into two classes----external and internalbased on the position of the prolapsed tissue. External
rectal prolapse may take one of three forms: (1) limited
mucosal prolapse, or false procidentia, in which only rectal mucosal tissue is passed through the rectum; (2) true
rectal prolapse type I, where all layers of the rectum are
extruded; and (3) true rectal prolapse type II, manifested
by intussusception ofthe upper rectum into and through
the lower rectum. Patients with rectal prolapse most commonly present with a complaint of a painless mass that
they may suspect is a hemorrhoid. Less commonly, they
may experience a discharge, small amounts of bleeding,
or loss of rectal sphincter control with resultant involun-
tary flatulence or defecation. The presentation of the

mass may follow Valsalva, straining, lifting, or difficult
passage of hard stool, and may be more prominent in the
upright position.
False procidentia, or limited mucosal prolapse, occurs
most commonly in children under 2 years of age or in
association with the more severe internal hemorrhoids.
This is thought to occur as a result of combined weakness
of the anal sphincter and the cross-linkage between the
submucosal and mucosal layers of the distal rectum.
True rectal prolapse occurs as a result of laxity of the
pelvic fascia and muscles of the pelvic floor, coupled
with weakness of the anal sphincters. This is most commonly seen in nulligravid women. It may occur as a complication of hysterectomy or lower GI tumor and may also
be seen in children with paraplegia and myelomeningo-
cele.
It is also an infrequent complication of multiple
sclerosis, tabes dorsalis, cauda equina syndrome, and

mental illness. True rectal prolapse may also present in
young adulthood as a consequence of the congenital

absence of mesentery. Recurrent true rectal prolapse is
AsooN,{lNAr AND GASTRoTNTESTTNAT Drsonnr,ns

associated with ulceration of the anterior anal mucosa,
and the emergency physician should be careful to evaluate this area and document the quality of the observed
mucosa.
External rectal prolapse can most generally receive ini-
tial treatment in the ED with simple, gentle reduction.

The use of analgesics and sedation may be required in
some cases, particularly in children and in the elderly.
The definitive treatment in the majority of cases is surgical. Patients should be counseled prior to discharge to
avoid straining, Valsalva, or lifting, and to return for

A stool softener such as docusate (Colace)
should be employed to prevent constipation. Should the
prolapse have been prolonged with the rare complication
recurrences.
ischemia secondary to strangulation of tissue, then

prompt surgical evaluation in the ED is required. The
presence of rectal prolapse should alert the clinician to
the possibility of associated carcinoma, and the emergency physician should ensure appropriate follow-up and
of
endoscopic evaluation.
Internal rectal prolapse has a female predominance and
may present with complaints of pelvic or rectal fullness,
pressure, or pain; rectal spasms; back pain; inability to
withhold flatulence or defecation; or retained stool. Sur-
47
by digital rectal examination. Bimanual examination,

direct visualization, and radiographic evaluation may be
helpful in some cases. Both sedation and local infiltrative
anesthesia of the anal sphincter may facilitate the removal
of foreign bodies, particularly when the patient is anxious
or the object is large. In some cases it will be necessary
to release a relative vacuum to facilitate removal of
objects, forming an occlusive seal with the adjacent

mucosa. Should this be necessary, it can be accomplished
by passing a hollow instrument past the item. Agents that
increase GI motility are not appropriate as they may
result in obstruction or retrograde propulsion of the foreign object proximally in the GI tract.
Following removal of an anorectal foreign body,
period of observation for sequelae is required, and careful proctosigmoidoscopy to exclude the complications of
perforation and laceration is mandated. Postrecovery
radiographs to exclude intraperitoneal gas, as evidence of
perforation, are indicated. Patients with fever, abdominal
pain, or objective findings on abdominal examination
may need admission for observation. Any patient with
clinical or radiographic evidence of perforation is an
operative candidate and requires urgent surgical consultatron.
gical consultation and referral is necessary.
Perianal Warts
Foreign Body (1.8.1.5)
Rectal foreign body, although occasionally accidental
or assaultive in nature, is usually self- or sexual partner-initiated. This is typically performed for the purpose
of enhanced sexual arousal. A small subset of ingested
foreign bodies will lodge as they attempt to make the turn
to leave the rectum and pass through the anus. Even less
frequent are iatrogenic foreign bodies such as lost endoscopic or enema equipment, and retained or broken rectal
thermometers.
The range of presenting symptomatology includes anxiety, pain, abscess formation, bleeding, anorectal discharge, rectal spasms, and peritonitis secondary to perforation. Prolonged presence of anorectal foreign bodies is
associated with abscess formation. There is generally a
known history of instrumentation or insertion, but the

patient may try to conceal this history from all health care
workers until they are seen by the emergency physician.
As the nature of inserted foreign bodies is quite variable, so too is the technique and tools used for the
removal of lower GI foreign bodies. Potential devices as
suggested in the literature would include standard or
neonatal obstetric forceps; obstetric suction extractors;
vaginal specula; Parks retractors; tenaculum forceps; ring
forceps; spoons, balloon catheters and tubes; flexible and
rigid sigmoidoscopes; anoscopes; probes affixed with
glue; and other suction devices. Prior to removal, the
nature and position of the foreign body must be defined
Perianal warts are of two main categories-condyloma

acuminatum, from the human papillomavirus, and condy-
loma latum, which is a manifestation of syphilis.

Condyloma acuminata are often sexually transmitted,
and studies of sexual partners reveal a 25o/o coinfection
rate; many patients with genital condylomata may also
harbor other sexually transmitted disease and thus

patients should be appropriately examined, screened, and
treated if indicated. The infection in rare cases may also
invade the rectal mucosa. Patients may complain of mass
and some may experience anal pain or itching.
Treatment for minor cases of perianal papillomata

involves topical treatment with 25% podophyllin.
Patients should be counseled to spend 2 to 3 hours in a
sitz bath or tub in order to wash off excess podophyllin
and minimize the risk of local burns. Repeat treatments
every 7 to 14 days may be required for eradication.
Refractory and more advanced cases require surgical
care.
Condyloma lata are also sexually transmitted, but as a

manifestation of secondary syphilis. On physical examination they may be differentiated from condyloma acuminata by their low horizontal plane, their more rigid texture, and that they are wetter lesions. Secondary syphilis
is treated with 2.4 million units benzathine penicillin G
by
intramuscular injection. Penicillin-allergic patients
may be given a lO-day course of either erythromycin or

tetracycline.
48 /
EunnceNcy MnucrNB: THs Conn CunnrculuM
Perianal Abscess
(1. 8. 1.
exam, formal incision and drainage is required to mini_

mize the risk of recurrence.
6, 1. S. 1, 7)
Anorectal abscesses are classified according to their

location and extension. The different types, in order of
frequency, are perianal abscess, ischiorectal abscess, submucosal abscess, and supralevator abscess. Figure l-2
depicts these various abscesses and their locations.
Abscesses of the anorectum are encapsulated accumu-
lations of infectious and inflammatory material. Colonic

flora are the predominant organisms of these polymicrobial collections. -E coli, Proteus, Bacteroides, Streptococcus, and anaerobes are commonly encountered.
The perianal abscess typically presents with perianal
pain exacerbated by passage of stool or Valsalva. The
majority of patients are afebrile and do well with incision
and drainage in the ED. A significant percentage of
patients with Crohn's disease present with this manifestation as their first indication of disease. The anorectal
abscess cannot be palpated a significant distance proximally during digital rectal examination. A proximal, tender rectal fullness should cause one to doubt the diagnosis of perianal abscess.
Patients with ischiorectal abscess are often febrile.

They often complain of an nondescript pain the buttocks.
Gluteal asymmetry or cellulitis may be noted on inspection. This process may be best appreciated by bimanual
rectal examination, which may reveal asymmetric fullness or firmness. The infection can spread locally to the
contralateral ischiorectal space, forming a ,,horseshoe
abscess."
The patient with a submucosal abscess
will complain
of an uncomfortable sense of incomplete evacuation.

External examination is usually normal. On digital examination a proximal tender mass will be appreciated. Even
in cases where spontaneous draining occurs during the
count. The supralevator abscess may not be palpable to all

examiners due to its cephalad position.
The treatment for any abscess in incision and drainage.
Only the perianal abscess is well suited for ED incision
and drainage (I & D).The greatest difficulty with ED I &
obtain adequate local
o limit the procedure
ary to incomplete initi s
or with immuno de f i c i ency
.n*,T
rtlt,lJ;?HTj;:
antibiotics such as cefoxitin (Mefoxin) prior to
I & D.
Patients with constitutional complaints, fever, immuno_
compromise, or cellulitis should be considered for admis_
sion and parenteral antibiotics. patients who have inci-
sion and drainage require packing changes
and
surveillance for healing and the complication of fistula

development. The diagnoses of fistula in ano and Crohn's
disease should be considered in any patient with recurrent perianal abscesses.

Proctitis (1.8.2.1)
Proctitis is a general term relating to both inflamma_
tion and infection of the anal canal. Specific etiologies
include infectious proctitis, traumatic proctitis, grarrulomatous proctitis, and radiation proctitis. Infectious proctitis can be divided further into the specific syndrome
cryptitis, general anal infections, venereal proctitis, and
those anal infections that only occur in immune deficient
hosts.
Infectious Proctitis
Cryptitis. Cryptitis is the infection of the superficial

Intermuscular
Ischiorectal
FlG. 1-2. Classification of anorectal abscesses by location.

(From Cabot EB, Sugarbaker DJ. Acute anorectal disorders.
ln: May HL, Aghababian RV, Fleisher GR, eds. Emergency

medicine,2nd ed. Boston: Litile, Brown, 1992;1532, with peimission.)
mucosal pockets that are formed as a result of the normal

contraction ofanal sphincters. It has been postulated that
cryptitis may be a precipitant for several etiologies dis-
cussed above, namely anal fissures, anal fistulae, and

perianal and anal abscesses.
Early infection is asymptomatic, but with progression,
pain and possibly bleeding may ensue. As inflammation
increases, papillae hypertrophy and infection spreads to
adjacent crypts. In advanced cases erosions ofthe crypts
form and papillae may hypertrophy to the point of prolapse. Findings are confirmed by anoscopy.
AsnoNarNAL eNo GesrRorNTESTrNAr
Treatment consists of normalization of stool consistency with fiber and stool softeners. Warm soaks, sitz
baths, and rectal irrigation are thought to enhance healing. More advanced cases require surgical referral.
Pinworms are caused by infection with Enterobius ver-
micularis and the patient usually complains of perianal

itching with a nocturnal and morning predominance. The
organism lives in the anal canal and lays eggs that
develop into larvae on the perianal skin. Such can be
found using the "Scotch tape" test. Trichophyton species
albicans,
although a frequent cause of perineal infection in infants,

women, and diabetics, is a relatively rare cause of anal
canal infection. Lice, discussed elsewhere, may also
plague the anal region.
49
Chlamydia anorectal infection is often the cause of

"nonspecific proctitis" and is characterized by discomfort, inflammation, and staining of undergarments. A
purulent discharge and irritation of the mucosa may be
seen on examination. Lymphogranuloma venereum
infection with dramatic inguinal adenopathy. Anorectal
discharge or a perianal papule may be seen. Complications include rectal stricture, perianal abscesses, and fistulae. Treatment consists of a 2l-day course of doxycycline. Erythromycin and sulfa are options in the allergic
patient. Erythromycin is preferred in the pregnant patient.
Acquired immune deficiency syndrome (AIDS) proctitis from atypical pathogens in patients with AIDS is
becoming common. This phenomenon has been contributory to the usage of the term gay bowel syndrome.
Potential opportunistic organisms include herpes simplex
type I, Mycobacterium avium-intracellulare, and cytomegalovirus.
Venereal Proctitis
Venereal disease found in the anorectal region is in

general the result ofanorectal intercourse, although cases
of contamination from vaginal or urethral discharge are
documented. In most instances the treatment is similar
not identical to that recommended for the same organism
if
when found
(LGV) is a specific syndrome of Chlamydia trachomatis
General Anal Infections
may also infest the anorectum. Cqndida
Drsorunns
in other locations, and only variations in
treatment are discussed in this section. The most common

organism in venereal proctitis is Neisseria gonorrhea.
Gonococcal proctitis has been found in l0% of homosexuals and presents with mild discomfort, a sensation of
incomplete evacuation, and discharge. Examination may
be normal or may reveal mucosal irritation and a purulent
discharge.
Anorectal syphilis presents with the chancre of primary syphilis; however, unlike chancres in other locations, the perianal chancre may be exquisitely tender. Primary infection may also demonstrate with anorectal
inflammation, lymphadenosis, and staining of undergarments. Secondary syphilis is seen as condyloma lata.
Herpes infection of the anorectum presents with the
same painful vesicles seen elsewhere on the body. These
will likely have been preceded by innocuous pruritus and
discomfort. Adenopathy of the inguinal chain is often

present as well as pain in the lumbosacral dermatomes.
Incontinence and impotence may also be present. Symptomatic treatment with analgesics, good hygiene with tub
soaks and sitz baths, and systemic antiviral therapy (e.g.,
acyclovir) are appropriate. The patient should be counseled as to the risks of transmission and superinfection, as
well as in the natural course of the disease where vesicles
progress to ulcers and craters over a l0- to l4-day period.
Patients with HIV and active herpes should be admitted
for IV antiviral therapy.
Individuals infected with HI! but who have not yet

met the diagnostic criteria for AIDS, may present to the
ED with one or more of these etiologies. Such patients
may not yet be aware of their HIV infection, and the clinician must consider that a patient presenting with an atypical anorectal infection may have more significant pathology'
Traumatic Proctitis
Traumatic proctitis occurs as the result of repeated

minor insults such as rectal prolapse, manual disimpaction, or anal intercourse. It is typically asymptomatic,
and is discovered on anoscopy where an ulcer is noted on
the anterior anal wall. It generally heals with reversal of
the precipitant. As with anal ulcers from other etiologies,
a biopsy to exclude malignancy must be obtained. Traumatic proctitis can also result secondary to chemical irritation, as might be caused by lubricants, sprays, and sup-
positories.
Granulomatous Proctitis
Granulomatous proctitis may occur with either ulcera-
tive colitis or Crohn's disease. Most patients present to

the ED with known disease. Anal fistulae are commonly
found in patients with Crohn's disease involving the
anorectum.
Radiation Proctitis
Radiation proctitis usually presents 4 to 5 months fola course of local anorectal ionizing radiation. The
initial presentation is usually with bleeding, but watery
lowing
50 /
Err,rnncnNcy
MnlrclNc: THe CoRE Cunnrculurvr
stool or constipation, painful rectal spasms, a feeling of a

need to evacuate, or obstruction may follow. Examination
will typically reveal a swollen, tender, brittle anal canal.
Rectal strictures may be found.
Tumors (1.8.3)
There are several tumors of the anorectum, many of
which are indistinguishable from more benign lesions.
The majority of anorectal malignancies are squamous
cell tumors (70%) and adenocarcinomas. Leukemia may
present with an anal leukemic infiltrate. Less frequent
etiologies include basal cell carcinoma, melanoma, epidermoid carcinoma, Kaposi's sarcoma, Bowen's disease,
extramammary Paget disease, and others. Risk factors for
anorectal tumor are family history of lower GI cancer,
personal history of breast or uterine carcinoma, pelvic
irradiation, ureterosigmoidostomy, familial polyposis,
and inflammatory bowel disease.
The most common presentation for anorectal tumors is
that of bleeding and pain. Any nonhealing or suspicious
lesions should be referred for excision and biopsy. It

should be noted that tumors of the anal canal are more
aggressive, earlier to metastasize, and associated with
greater morbidity and mortality than their perianal counterparts. Tumors of the anorectum, when identified and
referred early, are preventable causes of death, and it is
imperative that the emergency physician do an appropriate visualization and evaluation for every anal and perianal complaint.
SELECTED READING
Bassford R. Treatrnent ofcommon anorectal disorders. Am Fam Physician
1993 ;4s (4): 17 87 -17 9 4.
Fry RD. Anorectal trauma and foreign bodies. Surg Clin North
Am
505.
Glauser JM. Thrombosed external hemorrhoids. In: Roberts JR, Hedges
JR, eds. Clinical procedures in emergency medicine,2nd ed. Phitadelphia: Saunders, 199 1 ;7 04-'l 07 .
Modesto VL, Gottesman L. Sexually hansmitted diseases and anal manifestations of AIDS. Surg C I i n N orth Am 199 4 ;7 4(6) : | 43 3-l 464.
Rosen L. Anorectal abscess-fistulae. Surg Clin North Am 1994;74(6):
1994;7 4(6):1 49
-l
I 293-1 308.
Seow-Choen FS, Nicholls RJ. Anal fistol^ Br J Surg 1992;79(3):197-205.
CHAPTER 2
Cardiovascular Disorders
Marc C. Restuccia
Cardiovascular Disorders (2.0) ; Pathophysiology (2.1)
John T. Meredith and Charles K. Brown
Cardiac Failure (2.2.1) ; Cardiomyopathy (2.2.2) ; Myocarditis (2.2.6)
David P. Hightower and Charles K. Brown

Ischemic Heart Disease (2.2.3)
Michael K. Kerr and Charles K. Brown

Endocarditis (2.2.4) ; Vahular Heart Disease (2.2.5)
John T. Meredith
Diseases of the Pericardium (2.3)
John E. Gough and E.Jackson Allison,Jr.

Diseases of the Conduction System (2.4)
P"ggy E. Goodman and Amy A. DeStefano

Diseases of the Circulation, Acquired: Arterial (2.5.1)
P.ggy E. Goodman
Diseases
of the Circulation, Acquired: Venous (2.5.2), Lymphatics (2.5.3)
Richard A. Craven
Congenital Abnormalities of the Cardiovascular System (2.6)
Cardiac Transplan tation (2.7 )
Gury S. Setnik and Arshad Khan
Hypertension (2.8)
G. Richard Braen
Primary Tumors of the Heart (2.9)
Robert L. Brown and William J. Meggs
Myocardial Manifestations of Systemic Disease (2.10)
Robert L. Brown, Richard C. Hunt, and Francis L. Counselman

Treatment Modalities (2.1 1)
51
52 /
EunncrNcy MnucrNr: THr Conn CunnrcuI-uM
CARDTOVASCULAR DTSORDERS (2.0)

Cardiovascular disease is one of the most common and
challenging problems to confront the practicing emergency physician. In the United States 1.5 million people
experience acute myocardial infarctions (AMIs) each
year and of these 40% wrll die. In addition, approximately 400,000 people suffer sudden cardiac death from
dysrhythmias. In patients presenting to the emergency
department (ED), life-threatening disorders can be confused with and may overlap with less serious conditions.
Timely, definitive diagnostic testing often does not exist,
making difficult the task of sorting the serious from the
benign. In addition, the current economic climate
demands that the emergency physician limit interventions
and admissions to only those patients who absolutely
require it.
More optimistically, the practicing emergency physician has at his or her disposal many diagnostic and therapeutic interventions that simply did not exist a few years
ago. In his or her armamentarium are multiple medications and procedures that can be employed to open
blocked coronary arteries, stabilize the irritable myocardium, assist the failing ventricle and help prevent recurrent episodes.
The following sections discuss the most common complaints and disorders with which the emergency physicians are presented in their practice. The outline follows
the core content curriculum outline set forth by the American College of Emergency Physicians.
PATHOPHYSTOLOGY (2.1)
Congenital Disorders (2.1.1)
Congenital heart disease occurs in approximately 8 to
10 per 100 live births. Since most emergency physicians
will only occasionally see a patient with a congenital cardiac disorder, it may be helpful to approach the subject
via the presenting complaint.
Cyanosis
This is the most common presenting complaint in
culation through the placental vessels. Oxygenated blood

is sent from the right ventricle into the pulmonary vein.
From there a small percentage goes to the pulmonary circulation while the majority crosses the ductus arteriosus
and enters the descending aorta for systemic distribution.
After birth, the cardiovascular system must rapidly transition to support aerobic metabolism of the infant. The
placental-umbilical circulation is disrupted and the pulmonary circulation must almost immediately begin
replacing it as a source for oxygenation. As the lungs
expand after birth, the pulmonary vascular resistance
falls dramatically. The ductus arteriosus, which during
intrauterine life has been kept open by prostaglandin,
rapidly closes. In the normal infant it is functionally
closed in 12 to 24 hours and is permanently sealed with
thrombosis and intimal proliferation by 3 weeks.
Cyanosis itself is a physical sign that is characterized
by the slate-blue color of the mucous membranes, nail
beds, and skin. The color is due to an excessive amount
of deoxygenated hemoglobin in the blood. Most congenital defects leading to cyanosis will present in the neonatal period. Cyanosis will usually be present when the oxygen saturation is less than 85% or when the amount of
deoxygenated hemoglobin in the blood is greater than 5
gidl.
Examples of Cyanosis Producing Lesions
Transposition of the Great Vessels. This is the single

most common cause of cyanotic cardiac disease in the
newborn. Nearly all patients will present with cyanosis
within 24 hours of birth. The most common configuration of the great vessels is the following:
.
.
the aorta arises from the right ventricle and receives

deoxygenated blood from the systemic venous circulatron;
the pulmonary artery arises from the left ventricle and
receives oxygenated blood from the pulmonary venous
return;
there is a connection between the circulations, either a
patent ductus arteriosus or an atrial or ventricular septal defect.
in a newborn is usually secondary to cardiac or pulmonary disease, and it may be difficult to determine
which is predominant. A general rule of thumb is that in
the first 24 to 48 hours infants with pulmonary causes of
their cyanosis will have greater respiratory distress and
be more tachycardiac and tachypneic than those with pri-
Tetralogy of Fallot. This relatively common cause of

cyanosis accounts for about 10% of all congenital heart
disease. Children with tetralogy have a large ventricular
septal defect, right ventricular outflow obstruction, aortic
override, and right ventricular hypertrophy. The amount
of pulmonary outflow obstruction determines the presentation of the patient, with the greater the stenosis, the earlier the presentation.
mary cardiac disorders. Cardiac causes of cyanosis relate

primarily to partial or complete failure of the circulatory
system to transition from fetal to neonatal function.
Before birth, the fetus is oxygenated via the maternal cir-
Tricuspid Atresia. In this disorder there is no direct

connection of the right ventricle to the right atrium.
Blood enters the left-sided circulation from the systemic
circulation via an atrial septal defect.
neonates with severe congenital heart disease. Cyanosis
CeRolovescut-AR DTsoRDERS
Truncus Arteriosus. In this disorder a single artery
arises from the heart, supplying all of the circulation.
Cyanosis is in relationship to the amount of admixed oxygenated and deoxygenated blood.
Tbtal Anomqlous Pulmonary Venous Connection. This
occurs when the pulmonary veins drain into the systemic
venous system. Right to left shunting supports the systemic circulation.
Ebsteinb Anomaly. This is a malformation of the tricuspid valve. The valve is usually stenotic or incompetent. A variable percentage of patients with this anomaly
will be cyanotic due to a right-to-left shunt through an
atrial septal defect. The degree of symptoms
each
patient experiences is due to the amount of pulmonary

outflow obstruction that is present. Patients with minimal or no obstruction to pulmonary flow do uniformly
well. Those with severe obstruction tend to be difficult
to manage.
C ardiovascular C ol lap s e
Cardiovascular collapse or shock is a true medical

emergency that requires prompt therapy to resuscitate
and stabilize the patient. Congenital disorders leading to
collapse usually are manifest in the first week or two of
life. Most frequently the underlying cause of collapse is
due to systemic outflow obstruction. In general these
children will not have progressed well after birth with
poor feeding and weight gain as well as tachypnea. Usually they present when the ductus closes.
Hypoplastic Left-Heart Syndrome
This syndrome, which is always fatal, consists of a tiny
left ventricle, critical aortic stenosis, mitral stenosis, and
hypoplasia of the proximal aorta. Although there are usually signs of abnormality such as cyanosis, tachypnea,
and tachycardia soon after birth, once the ductus closes
cardiovascular collapse is rapid.
Aortic Stenosis
This entity also becomes critical when the ductus

occludes. The prognosis is directly tied to the adequacy of
the left ventricle. If the left ventricle is sufficient to support the systemic circulation, surgical repair is usually
successful.
Coarctation of the Aorta
In this disorder, where the descending aorta is significantly narrowe4 and in the related interrupted aortic arch
syndrome, the infant can initially appear well. When the
ductus closes, the patient usually presents in shock. Quite
53
often associated intracardiac abnormalities such as ven-
tricular septal defects are present.

Congestive Heart Failure (CHF)
Most often, patients presenting with congenital causes

of CHF do so after the first 2 weeks of life and usually in
a less dramatic fashion than patients with cyanosis or collapse. Patients will be symptomatic due to left-to-right
shunting of blood. In the normal neonate pulmonary vascular resistance gradually decreases. This allows increasing left to right shunting in those patients with intracardiac defects.
Examples of Congestive Heart Fqilure
Ventricular Septal Defect. This defectranges in severity
from mild to severe, requiring surgical repair depending on
the size ofthe defect and the general health ofthe child.
Atrioventricular Septal Defect. This defect consists of
a common atrioventricular valve, primum atrial septal

defect, and ventricular septal defect. Atrioventricular
(AV) valve regurgitation may be present. Trisomy 2l is
associated with a huge percentage of congenital cardiac
disorders, and AV canal defect is the most common single entity.
Patient Ductus Arteriosus. Occasionally, for reasons

that are usually unclear, the ductus arteriosus fails to
close. This anomaly is associated with a continuous,
machinery-like murmur.
Murmurs
A murmur heard in the first 24 hours of life has a l\Yo
to l5oh chance ofbeing due to a congenital cardiac disorder. Since murmurs are heard with turbulent or abnormal blood flow, it is not surprising that most murmurs are
due to defects in the septal walls or abnormalities of the
valvular apparatus.
Ventricular Septal Defect (VSD)
The smaller the defect, the more likely it will be found
early. As the pulmonary outflow resistance drops, the
abnormal flow associated with a VSD is more likely to be
heard. With alarge VSD, the fall in pressure in the pulmonary circulation will be slowed and the murmur's
appreciation delayed. Many small defects will close

spontaneously and no intervention is needed ifthe child
is thriving.
Patent Ductus Arteriosus
This is the classic machinery murmur, heard over the

second left intercostal space. These lesions usually close
54 t
EurnceNcv MrorcrNn: Tsn Conn Cunnrcuruna
spontaneously; however,
are unlikely to do so.
ifthey have not by I year, they
Atrial Septal Defect (ASD)

Most children with ASDs are asymptomatic. These are
usually systolic ejection munnurs, heard best at the upper
left sternal boarder. These also will often close spontaneously and only rarely require surgical repair.
Aortic Stenosis
This condition is very common, but unless it is severe
it usually does not present in infancy.

The above represent some of the more usual presentations of the most common congenital cardiac defects.
Although the average emergency medicine physician will

only rarely encounter patients with these defects, a reasoned, logical approach to the patient with potential car-
diac malformation will allow him or her the greatest

opportunity to correctly diagnose and treat these challenging patients.
cardial cells. Ischemic heart disease kills over 650,000

Americans each year.
Ischemia can be brought on by spasm of coronary
arteries
or by arterial occlusion from
atheromatous
plaques. The latter is more comnon, but both may be

operative in any patient.
The process of atheromatous accumulation usually

develops over years. The endothelium of the coronary
vessels is damaged in some way such as disruption in
normal flow as occurs at branch points. In turn this damage causes accumulation of lipids and macrophages,
leading to aggregation of platelets. Smooth muscle proliferation and further accumulation of lipids and macrophages is encouraged by growth factors elaborated by the
macrophages and platelets. At some point, either the
occluded vessel lumen is insufficient to supply adequate
blood to the myocardium, or the plaque may rupture,
totally occluding the vessel with the resultant thrombus.
Ischemic damage to the myocardium occurs when the
delivery of oxygenated blood is insufficient to the
demands of the working cardiac cells. This can occur
when the flow is disrupted by spasm, thrombus, or plaque
rupture, or with increased oxygen demand as is seen with
exercise or hypoxia.
Acquired Disorders (2.1.2)

Cudiomyopathy
Myocardium
The myocardium is the muscular pump of the heart and
its dysfunction leads to significant changes in normal

cardiovascular function. Coordinated functioning of the
myocardial contractile elements is basic to normal cardiac output. Lessened or absent contractile force or a loss
of coordination of contraction can lead to dysfunction.
Failure
Cardiac failure implies inadequate myocardial function. Generally seen in patients who have experienced
ischemic events, it can also be seen in patients who have
experienced viral, toxic, or some other insult to the
myocardium. The etiology of cardiac failure can be on
the basis of three major categories: first, structural
abnormalities of cardiac valves, pericardium, endocardium, and great vessels that impede normal cardiac
filling and emptying; second" primary dysfunction of
the myocardial contractile unit leading to inadequate
pump action; and third, alterations in organization or
conduction of cardiac contraction, e.g., dysrhythmias,
can lead to failure.
Ischemia
Ischemic cardiac dysfunction is brought on when the
oxygen supply is inadequate to the demands of the myo-
Cardiomyopathies are usually classified as dilate4

restrictive, or hypertrophic.
Dilated Cardiomy op athy

This is the most common of the cardiomyopathies. The
etiology can be idiopathic or secondary to a wide variety
of toxic, metabolic, or infectious insults. Alcohol and

antineoplastic medications are typical of cardiac toxins
leading to dilated cardiomyopathy. Infectious etiologies
include human immunodeficiency virus (HIV), coxsackie virus, and Trypanosoma cruzi, and Toxoplasma
gondii.
Classical findings
for dilated cardiomyopathy are
perivascular and interstitial fibrosis. Systolic dysfunction

leads to heart failure.
Restri ctiv e Cardiomy op athy
This is characterized by replacement of normal myocardium with a fibrotic material. Interestingly, although
the diminished ventricular compliance leads to diastolic
dysfunction, systolic function is usually preserved. Multiple syndromes and diseases lead to restrictive cardiomyopathy; some of the more common are amyloidosis, Pompeis disease (an accumulation of glycogen due to
deficiency of cr-glucosidase), Fabryis disease (glycolipid
Ceuovascut-A,R DTsoRDERS
accumulation due to an error of glycosphingolipid
metabolism), Loffler's endocarditis (accumulation of
eosinophils), hemochromatosis (abnormal iron deposition), and hypertrophic cardiomyopathy.
55
and increased myocardial oxygen demand. This in turn

can lead to ischemia, syncope, congestive heart failure,
and sudden death.
Aortic Regurgitation
Endocarditis
Although cardiac infections are relatively uncommon,
some groups are at increased risk such as intravenous
drug users and patients with prosthetic heart valves. The
common pathophysiologic thread is an abnormality of
cardiac endothelium. If the abnormality exposes colla-
gen-containing connective tissue, platelet aggregation

occurs. It is in this aggregation that bacteria can lodge
and multiply, relatively secure from the patient's immune
system. The results of this infection carry significant
morbidity and mortality for the patient: abscess formation, embolization, valvular incompetence, rupture of
chordae tendinae, valvular stenosis, congestive heart failure, and" in patients with prosthetic valves, valvular
destruction.
Myocarditis
As the name implies, myocarditis is present when the
myocardium itself is infected. The most common etiology
of such an infection is viral in nature. The most common
virus causing heart muscle infection is coxsackie. Other
agents implicated in causing myocarditis include rickettsial diseases such as Lyme disease and Rocky Mountain spotted fever, Salmonella, fungi, trypanosomiasis,
and toxoplasmosis. Symptoms range from none in the
majority of cases to congestive heart failure, arrhythmias,
and, rarely, death.
Valvular
The two valves most commonly involved in causing

clinical symptoms are the aortic and mitral. The pulmonic
is implicated less frequently.
Aortic Valve
The aortic valve usually has three cusps, with the left
coronary artery originating behind the left posterior cusp.
The acute form ofaortic regurgitation is quite different

from the chronic version. Acutely, compensation of the
left ventricle has no time to happen and left ventricular
failure occurs. Common etiologies of acute aortic regur-
gitation include trauma, infective endocarditis, prosthetic
valvular dysfunction, and aortic dissection involving the

aortic root.
Hemodynamically, acute aortic regurgitation
causes
markedly increased left ventricular and diastolic volume

with a concomitant rise in diastolic pressure. This pressure can be transmitted back to the atria, resulting in pulmonary congestion. The relatively stiff left ventricle suffers decreased forward output, resulting in compensatory
increased peripheral vascular resistance. This exacerbates
the regurgitation and a vicious cycle ensues, leading to
cardiac failure.
In the chronically regurgitant aortic valve, the left ventricle dilates and hypertrophies, maintaining more normal
hemodynamics for a time.
Mitrul
Valve
The normal mitral valve consists
of two
cusps.
Attached to these are the chordae tendinae and the papillary muscles. Abnormality in any of these components
can lead to valvular dysfunction.
Mitral
Valve Prolapse
This is probably the most common cardiac
valve
abnormality, with an incidence of 5oh to l3%. Although

the majority of persons with this condition will be asymptomatic, patients with mitral valve prolapse are at an
increased risk of endocarditis, chest pain of unknown etiology, and, rarely, sudden death. The abnormality present
is billowing of the mitral cusps into the atria with systolic
ventricular contraction. This can be due to anomalies in
the mitral annulus, redundant cusp tissue, or relative laxity of either the chordae tendinae or papillary muscles.
Rarely it is associated with connective tissue diseases
such as Ehlers-Danlos slmdrome.
Aortic Stenosis
Mitral Regurgitation
Causes of aortic stenosis include congenital, rheumatic
heart disease, and age-related degeneration. With the

obstruction to left ventricular outfloq ventricular hypertrophy is seen. This allows for preservation of cardiac
output but at the cost of decreased coronary artery flow
Like aortic regurgitation, this differs greatly whether it

develops acutely or chronically. Acute regurgitation can
be seen with ischemic damage to the papillary muscles or
chordae; similarly, infective endocarditis can lead to
56 /
ElmncnNcn MtoIcINn: THe CoRE Cunnlculutr,t
In the acute form, mitral regurgitato left atrial overload and failure,
rapidly
lead
tion can
acute regurgitation.
with life-threatening pulmonary edema. The ability of the
left ventricle to compensate is important in determining

the outcome, which is worsened if the left ventricle is
compromised due to ischemia or previous dysfunction.
In the chronic form, mitral regurgitation develops
slowly and the left atrium dilates, minimizing pulmonic
congestion. This dilatation can predispose the patient to
atrial fibrillation and left atrial thrombus formation with
the attendant risk of systemic embolization.
Mitral Stenosis
TABLE
2-1.
ldiopathic
Radiation
Drugs
Viral
Acute Ml
Trauma
Tuberculosis
Neoplasm
Myxedema
Bacterial infections
Uremia
Autoimmune disease
ventricles are unable to
This is most commonly seen in patients with a history
of rheumatic heart disease. As the area of the
valve
decreases, the pressure gradient rises across the valve.
This in turn causes left atrial enlargement and pulmonary
As the stenosis becomes critical, pulmonary congestion is seen.
hypertension.
fluid accumulation.
The right-sided system is a low-pressure system.

Because of this, right-sided valvular lesions are in gen-
Conduction System
eral less critical than left. Abnormalities of the pulmonic

and tricuspid valve can be seen with congenital connective tissue diseases and with infective endocarditis.
Pericardium
The pericardium consists of two layers of tissue that
reflect back on themselves. The pericardium can be the
site of an infectious process, either bacterial, viral, or fungal. In addition, the pericardial sac, which typically contains only a small amount of flui4 can become a collection site for flui{ bloo4 gas, or interstitial fluid.
Pericarditis
Acute inflammation of the pericardium has
multitude
2-l.
Pericardial Effusion or Tamponade. The two surfaces of the pericardium usually contain only a small
amount of fluid. In some conditions this can change as
the pericardial space becomes a reservoir of fluid. Such
conditions include trauma, uremia, and neoplasms. As
the fluid accumulates, if there is insufficient time for
the pericardial space to, accommodate, intrapericardial
pressures begin to rise. If they rise to levels approach-
ing
intraventricular pressures, cardiac output is
impaired. Initially the heart compensates with increasing stroke volume, but with continued pressure rise the
fill
adequately and cardiac out-
put falls.
If the fluid accumulates more slowly and compensation does occur, this fall in cardiac output is not seen,
even with massive distention of the pericardial sac. Some
of the more common etiologies for such noncompressing
cardiac effrrsions are uremia, malignancy, autoimmune
disorders, and medications. Anything that potentially can
irritate the pericardium can lead to chronic pericardial
Pulmonic Vulve
of causes. Some of them are listed in Table
Etiologies of pericarditis
The normal cardiac electrical impulse originates in the
sinoatrial (SA) node, located high in the right atrium.

From there, impulses traverse the atria, are slowed at the
atrioventricular node, are retransmitted through the HisPurkinje system and thence to the ventricles. In some
individuals, there may be extra or accessory pathways
short-circuiting the above conduction scheme. Multiple
factors can impact the smooth operation of the heart's
electrical system. Trauma, ischemia, medications, infec-
tion, and varying neural impulses affect the rate and

rhythm of the heart.
When discussing arrhythmias, two mechanisms predominate: abnormal automaticity and reentry. Abnormal
automaticity is the result when the SA node is superseded
as the pacing site by other cardiac tissue. Reentry occurs
when conduction occurs down a normal pathway and
then is routed back to an area that has just been depolarized due to accessory or bypass paths. These can be congenital or arise due to myocardial damage, such as occurs
with ischemia. The returning impulse can then be transmiued down the normal pathway again, setting up a circular pattern of depolarization.
Atrial Arrhythmias
Atrial fibrillation and flutter as well as most supraventricular rhythms are reentrant tachycardias involving the
AV node. Wandering atrial pacemaker and multifocal
Cenorovescut-AR DrsoRDERs
atrial tachycardia are examples of rhythms that are atrial
in origin and are due to abnormal automaticity.
57
SELECTED READING
R\ ed. Emergency management of cardiovascular disease.

Boston: Butterworth-Heinemarur, 1994.
Burton DA, Cabalka AK. Cardiac evaluation of infants, the first year of life.
Pediatr Clin North Am 1994;41(5):991.
Chakko S, Kessler KM. Changes with aging as reflected in noninvasive cardiac studies. In: Lowenthall DT, ed. Geriatric cardiology. Cardiovascular clinics. Philadelphia: Davis, 1993;35-47.
Driscol DJ. Evaluation of the cyanotic newborn. pediatr Ctin North Am
1990:37(1):1.
Lewis JE, Maron BJ. Cardiovascular consequences ofthe aging process. In:
Lowenthall DT, ed. Geriatric cardiology. Cardiovascular clinics. phlladelphia: Davis, 1993;25-34.
Rudolph A. Rudolphb pediatrics, lgth ed. New York: Appleton & Lange,
Aghababian
Ventri cul ar Arr hy t hmias
Ventricular fibrillation and tachycardia are poorly tolerated and are usually due to reentry. The conduction is
quite abnormal and results in the wide, slurred QRS complexes associated with ventricular rhythms.
The Effects ofAging on the Heart (2.1.3)
As the life span of the population increases, interest

has grown in what effects aging has on myocardial struc-
ture and function. Geriatric cardiology has to concern

itself with the changes brought about by aging itself and
the changes caused by living with underlying diseases
longer. Coronary artery disease is more common in the
elderly. This is in itself not due to aging but to living with
a condition for a longer period of time. What are the
changes caused by aging alone?
Morphologically, as the person ages, the heart weight
increases. The ventricular cavities diminish while the
atrial enlarge. Left ventricular wall and septum increase
in thickness with aging. Amyloid is deposited in the heart
in increasing numbers as people age. Up to 50oh of persons older than 56 have such cardiac deposits. The elas-
ticity and compliance of the heart diminish with time.

The valves are often the site of extensive calcium
deposits. These may lead to valvular stenosis or regurgitation. The aortic valve seems to be affected earlier and to
a greater degree than the mitral valve.
The conduction system is not spared. Fibrous infiltration of the sinus node and the normal conduction pathways can lead to various degrees of heart block. Functionally, these changes have some effect on the heart.
Systolic function appears to be fairly well preserved even
into very advanced years. By contrast, diastolic function
is not nearly as well preserved. A seemingly less compliant and distensible ventricle appears to have a prolonged
1991
DISEASES OF THE MYOCARDIUM, ACQUIRED

(2.2)
Cardiac Failure (2.2.1)

Cardiac failure is one of the most common presentais encountered in emergency medicine.
Approximately 400,000 new cases are diagnosed annually with an estimated 2 to 3 million Americans suffering
some degree of cardiac failure. Patients hospitalized
with cardiac failure have tripled over the past two
decades and it is now the most common diagnosis in
patients over age 65 years. In addition, survival rates are
exceedingly low with the 5-year survival for men being
40Yo and 55Yo for women. For patients with cardiac failure who display symptoms at rest [NewYork HeartAssociation (NYHA) class IVl, the mortality is 50% within I
tions that
year (Table 2-2). Therefore, it is imperative that the

emergency physician be familiar with all aspects of cardiac failure.
The most common causes of cardiac failure are coro-
nary artery disease, idiopathic cardiomyopathy, and

hypertension. All of these etiologies result in decreased
cardiac output along with decreased peripheral perfusion
and associated maladaptive compensatory mechanisms.
2-2.
filling time and increased need for the atrial kick for
complete filling. As people age, their exercise capacity
TABLE
decreases, and cardiac output, maximal heart rate, and

vital capacity all diminish. Despite all of this, functional
deterioration, often seen in the elderly, is not just a byproduct of diminished cardiac function; inactivity and
Class
Class
ll
Class
lll
Class
lV
other diseases play major roles.
There is a wide range of function and capabilities in

the elderly. Some persons are vigorous and without significant disease until very late in life. Others have relatively rapid decline in function and health. Level of activity, other illness, good health care habits, and genetic
fortune (or misfortune) serve to make for quite diverse
cardiovascular health in the aged population.
New York Heart Association (NYHA) functional

classification of heart failure
No limitation of physical activities. Ordinary

physical activity does not cause undue fatigue,
palpitation, dyspnea, or anginal pain.
Slight limitation of physical activities. Comfortable
at rest, but ordinary activity results in fatigue,
palpitation, dyspnea, or anginal pain.
Marked limitation of physical activity. Comfortable
at rest, but less than ordinary physical activity
results in fatigue, palpitation, dyspnea, or anginal
pain.
Unable to carry on any physical activity without
discomfort. Symptoms of cardiac insutficiency or
angina at rest. Discomfort increases with any
physical activity.
58 /
EnnncrNcv MnorcrNr: Tnn Conn CunnrculuM
However, this specific syndrome is complex and involves

not just cardiac failure but also a series of pathophysiologic circulatory derangements that exacerbate the heart's
failure. Of importance to note is that cardiac failure is a

syndrome and not a specific disease. Cardiac failure
refers to the physiologic focal point of the heart's inability to maintain adequate cardiac output in relation to the
physiologic needs of adequate tissue perfusion.
Cardiac
Output
Mild Heart Failure
Moderate Heart Failure
Low cardiac
output
symptoms
Pathophysiology
Left Ventricular End-diastolic Pressure (mm Hg)
Cardiac
In cardiac failure, cardiac output is decreased as a

result of impaired myocardium. By definition cardiac
output is a function of heart rate and stroke volume.
Therefore, myocardial performance is also a function of
heartrate, stroke volume, contractiliry and electrical conduction. Heart rate is determined by sympathetic outflow.
Autonomic tone is frequently increased in cardiac failure
resulting in tachycardia. Stroke volume is determined by
preload, myocardial contractility, and afterload. Preload
refers to the ventricular volume at the end of diastole and
is often expressed as left ventricular end-diastolic pres-
sure (LVEDP). This volume physiologically loads the

ventricle for contraction. Both a decrease and increase in
preload will change stroke volume evoking compensatory mechanisms. Factors that can affect preload
include intrathoracic pressure, pericardial pressure, and
amount of venous return to the atrium. The relation
between preload and cardiac performance can best be
expressed by a ventricular function curve relating the car-
diac output to left ventricular end diastolic pressure as

illustrated in Fig. 2-1.
In mild cardiac failure, cardiac output is decreased
secondarily to impaired myocardial contractility. Physiologic adaptations to mild failure include an increase in
venous tone and an expansion of intravascular volume.
These two adaptations increase the LVEDP and increase
In mild failure this increased filling

in a compensatory increase in cardiac
output to the point where the ventricleis function is
maximal on the Starling curve. As cardiac failure profilling
pressure.
pressure results
FlG. 2-1. Ventricular function curve depicting cardiac output

in relationship to left ventricular end diastolic pressure based
on Frank-Starling principles.
increased left ventricular afterload. The net product is a

progressive decrease in cardiac output to the point of
being unable to meet the metabolic demands of other
organ systems.
Extracardiac
Although this chapter's primary focus is the cardiac

system, there exists a complex continuum of pathophysiologic changes occurring throughout the body to compensate for the progressive cardiac failure. The most sig-
nificant compensatory adaptations are neurohormonally

activated and are the renin-angiotensin-aldosterone axis,
the sympathetic nervous system, and the atrial natriuretic
system.
The function of the renin-angiotensin-aldosterone axis
in cardiac failure is to increase intravascular volume and

augment preload. This action is accomplished by an
increase in aldosterone, which leads to renal conservation
of sodium and increased water retention, as outlined in

Fig.2-2. Angiotensin II plays a major deleterious role. It
increases vasoconstriction of peripheral arterioles, which
increases afterload, ultimately resulting in increased left
ventricle workload.
Sympathetic outflow
is increased in cardiac failure.

The effect is frequently a tachycardia with increased
gresses there is an increasing degree ofdysfunction, and
pressures are associated with a

cardiac output. In chronic failure, an
increase in left ventricular size, hypertrophy, or dilatation, maintains stroke volume and cardiac output as the
ventricular ejection fraction decreases. This increase in
ventricular size is the principal cardiac adaptation to
failure, the myocardial cells undergoing remodeling in
which the length and volume of individual myocytes are
increased. Left ventricular enlargement and increased
filling pressure result in increased ventricular wall
stress with an elevated myocardial oxygen demand and
increasing
decrease
filling
Physiologic
Angiotensinogen Cardiac
effects
of
(Renin)
Angiotensin
(Converting Enzy-")
az
- I
ltngiotensbll\
secretion
I
Aldosterone
.,, :
. Aldostemne
\
t't
\
Increased
Increased water md
sodium retension
FlG.
2-2.
effects
Increased Afterload
(Decreased cardiac output)
Increased Preload
(Increased fi lling pressures)
Ren in-an giotensin-aldosterone
axis.
CenDrovescut-{R DTsoRDERS
myocardial oxygen consumption and increased peripheral vascular resistance. The increased peripheral vascular resistance results in an increase in both afterload and
preload. Afterload is increased primarily by arteriolar
vasoconstriction; however, this increase results in elevated systemic vascular resistance, decreasing renal
blood flow, leading to further sodium and water conservation. Increased afterload also results in increased left
ventricular wall tension and elevated myocardial oxygen
consumption. The increase in sodium and water retention
compounds the increasing ventricular filling pressure and
ventricular wall tension. The wall tension stimulates

myocyte remodeling and altered ventricular wall morphology. The net outcome is a maladaptive response that
steadily exacerbates the failing myocardium.
Atrial natriuretic peptide released from the atria has
been shown to temporally counteract the effects of fluid
retention and vasoconstriction, increasing the renal
excretion of sodium and water. However, its effects are

very minor and short lived in comparison to other adaptations present in cardiac failure. Atrial natriuretic hormone is felt to have no significant impact on the progressive course ofcardiac failure.
Left-Sided Failure
High Output (2.2.1.1)

By definition, high-output cardiac failure is a hyperdynamic circulatory state in which there is a failure to maintain cardiac output and a concomitant decrease in peripheral vascular resistance. In this specific condition, cardiac
output and contractility are initially increased. However,
as the bodyis metabolic and oxygen demands increase, so
do those of the myocardium. As tachycardia progresses,
the time interval for diastolic filling decreases and stroke
volume decreases with a subsequent decrease in myocardial perfusion. The result is eventually myocardial failure
secondary to ischemia. Underlying etiologies include thyrotoxicosis, early sepsis, hyperthermia, arteriovenous fistulas, Paget's disease, beriberi, anemia, and pregnancy.
Typically, these patients present with an associated tachycardia, widened pulse pressure, and vasodilation. Initial
treatment is aimed at restoring myocardial perfusion by
controlling the rate of tachycardia and maximizing oxygen delivery to the myocardium. Intravascular volume
may need reduction with diuresis if chronic, and vasoconstricting agents may be utilized if vasodilation is acute
or severe. Definitive treatment is accomplished by treating the underlying pathology.
Low Output (2.2.1.2)

Low-output failure characterizes most forms of heart
failure, and is frequently seen in ischemic heart disease,
59
end-stage valvular heart disease, hypertensive heart disease, congenital heart disease, and the cardiomyopathies.
The focus of low-output failure is left ventricular dys-
function, with the hallmark being systemic hypoperfusion with widespread end-organ dysfunction. Principal
clinical characteristics are impaired peripheral circulation
with subsequent mental confusion, weakness, and sodium
and water retention. Renal underperfusion and resultant
sodium and water retention lead to pulmonary congestion
as the cardinal manifestation
of low-output failure. Nev-
ertheless, the retention of sodium and water is an important compensatory mechanism for increasing preload. As
the derangement advances, stroke volume decreases and
pulse pressure narrows, exacerbating the degree offailure
causing the clinical presentation.
These patients frequently experience dyspnea with

activity or rest, orthopnea, generalized fatigue, and
paroxysmal nocturnal dyspnea. Nocturia is common,
resulting from a redistribution of venous blood in the
supine position. Physical findings are consistent with
those of pulmonary congestion and left ventricle failure:
rales on lung auscultation, a protodiastolic gallop or 53 on
cardiac auscultation, peripheral edema, venous hypertension fiugular venous distention and hepatojugular reflux),
and pulsus alternans. The electrocardiogram can be

extremely variable, displaying acute ischemic changes,
interventricular conduction delays and blocks, or various

arrhythmias. The chest radiograph invariably shows cardiomegaly and evidence of pulmonary congestion.
Treatment is primarily directed at the underlying type
of heart failure. In the acute setting, of paramount importance is the utilization of supplemental oxygen as one
goal to maximize the level of oxygen available to the
failing myocardium. One way this intervention is most
effectively achieved is with continuous positive airway
pressure through a tight-fitting mask or positive endexpiratory pressure if the patient is intubated. Both
adjuncts are effective in overcoming the decrease in pulmonary compliance associated with pulmonary congestion. Treatment is directed at optimizing myocardial oxygen utilization by reducing the myocardial work load and
enhancing oxygen availability, decreasing the volume of
retained fluid, and improving contractility.
A reduction in cardiac work load is achieved by reducing the level ofphysical activity and reducing afterload.
Afterload reduction is best accomplished by vasodilation
of both the arteriolar and venous systems. Pharmacologically this action is best achieved by use of nitrates for
venodilation and calcium channel blockers for arteriolar
dilation. Agents with combined venous and arterial dilator effects include nitroprusside, angiotensin-converting
enzyme inhibitors, prazosin, and phentolamine. Of the
later agents, angiotensin-converting enzyme inhibitors
are the most important.
Reduction in fluid retention is best achieved through
the use of diuretics and dietary sodium restriction.
60 /
EnrncrNcy MBnrcrur: Tne Cons CunrucuLUM
Diuretics reduce preload by reducing intravascular vol-
50%. COPD is the leading cause of chronic cor pul-
ume through increased excretion of sodium and water at
monale. Cor pulmonale is only exceeded in prevalence by

coronary artery disease and hypertensive heart disease.
These facts make it a common presentation that is often
not considered in the acute presentation of the COPD
patient. In addition, chronic cor pulmonale is part of the
natural course of pulmonary interstitial fibrosis and the
sleep apnea disorders.
the level of the renal distal tubule. The most commonly

used diuretics to achieve this result are loop diuretics,
furosemide, ethacrynic acid, and bemetanide, acting

directly on the loop of Henle. Furosemide has been
shown to provide a degree of venodilation, further augmenting preload reduction. Other diuretic classes that are
useful include the thiazides and the potassium-sparing
agents. The potassium-sparing diuretics, spironolactone
and triamterene, can potentiate the diuresis achieved by
the loop diuretics. Overdiuresis can occur when these
classes are used together, resulting in a precipitous
decrease in preload" further aggravating the degree of
failure as the body compensates to counteract this iatrogenic insult.
Inotropic agents are the mainstay for augmenting myocardial contractility. Historically the use of digitalis glycosides have been and continue to be the first choice as
an inotropic agent. In short, inotropic agents such as dig-
italis move the ventricular function curve upward

increasing stroke volume and contractility for a given
preload. In addition, digitalis reduces the cardiac
response to sympathetic tone and prolongs the refractory
interval of the atrioventricular node, the net effect being

the control of the ventricular response in arrhythmias
such as atrial fibrillation. Diltiazem, a calcium channel
blocker, also prolongs atrioventricular node conduction
and controls the ventricular response to atrial fibrillation.
A negative inotropic agent, dlltiazem is employed in the
rapid conversion of supraventricular tachycardia to sinus
rhythm, but must be used with caution in those patients
with heart failure. Other inotropic agents include epinephrine, dopamine, norepinephrine, dobutamine, and
amrinone. These agents are typically of benefit when the
degree of failure is severe, cardiogenic shock is present,
or the need for rapid stabilization is paramount.
Right-Sided Failure
Cor Pulmonale
(2. 2.
1.3)
In the strictest definition, cor pulmonale refers to right

heart failure and hypertensive pulmonary disease. The
term cor pulmonale was introduced more than 50 years
ago to describe the cardiopulmonary state whereby the
right ventricle is enlarged and impaired secondary to pulmonary hypertension. Traditionally, this process has been
thought of as either chronic or acute with differing eti-
ologies and pathophysiologic processes occurring in

each.
The chronic aspect of this disease accounts for up to

of all patients with cardiac disease; furthermore, the
incidence of cor pulmonale in patients with chronic
obstructive pulmonary disease (COPD) is approximately
l0o/o
Regardless of the inciting etiology, the pulmonary

hypertension associated with chronic cor pulmonale is a
result ofan increased resistance to blood flow throughout
the pulmonary vasculature. The pathologic lesion is one
of medial hypertrophy and intimal hyperplasia of the pulmonary arterioles and small arteries. The subsequent
alveolar hypoxia contributes to further pulmonary hypertension by causing pulmonary vasoconstriction. Alveolar
hypoxemia is the strongest known stimulus for pulmonary vasoconstriction. The result of these physiologic
changes is an increased demand for right ventricular output, an increase in pulmonary blood volume, and reactive
polycythemia. As these physiologic derangements continue, right ventricular dilatation and hypertrophy occur
as an adaptation to the increased pulmonary aftery pressure. Without treatment, the reserve of the right ventricle
is exceeded and right ventricular failure occurs with its
characteristic findings. The occurrence of venous con-
gestion predominates and
is
manifested
by jugular
venous distention, tricuspid insufficiency, hepatomegaly,

hepatojugular reflux, ascites, and peripheral edema.
The diagnosis of chronic cor pulmonale is difficult

because signs and symptoms are often nonspecific in the
early course ofthe disease process. Thus, there is often a
delay between the onset of the disease and the specific
diagnosis as the physical findings occur late
in
the
process.
Typically, hypoxemia is present with associated dyspnea, orthopnea, fatigue, dizziness, and syncope. Cardiac
auscultation reveals evidence of right ventricular hypertrophy with an increased P2 component of Sz and a rightsided S+. If significant venous congestion is present, an
S: summation gallop may be heard. Chest radiographs
when viewed in succession from a number of studies may
reveal right ventricular enlargement and the characteristics of pulmonary hypertension. The classic electrocar-
diographic findings
of chronic cor pulmonale
demonstrate right-axis deviation, P pulmonale, and R:S ratio in

V I of more than I , and an R: S ratio in V5 and V6 of less
than l. However, the most definitive tests are those of

two-dimensional echocardiography, first-pass radionuclide angiography, and cardiac catheteization.
Treatment of chronic cor pulmonale is directed at
relieving pulmonary hypertension. However, the interstitial fibrosis that accompanies pulmonary hypertension is
irreversible. Management is thus directed at relieving the
hypoxic pulmonary vasoconstriction through the use of
supplemental oxygen. Not only does supplemental oxy-
Cer,nrovescur-AR DTsoRDERS
/ 6l
gen therapy decrease the degree ofpulmonary hypertension by decreasing the hypoxic-vasoconstrictive effect on
pulmonary vasculature, it also results in improved myocardial oxygenation, decreased right ventricular afterload,
In the setting of acute pulmonary embolism, the presentation is typically that of significant hypoxemia, sudden dyspnea, sudden onset ofsyncope, chest pain, tachycardia, tachypnea,
hypotension. However, the
improved right ventricular performance, and improved

oxygenation to other organ systems. The goal of supplemental oxygen therapy is to achieve an arterial pOz of
more than 60 mm Hg or an arterial Oz saturation of
greater than 90%o. Although, other modalities of treat-
presentation
ment including digitalis, diuretics, and sodium restriction
may be helpful, their use is controversial. In these

severely hypoxic patients, digitalis can induce arrhyhmias, and diuretics can alter electrolytes or increase pulmonary vascular resistance by hemoconcentration from
excessive diuresis. In short, supplemental oxygen therapy
is the mainstay of treatment for chronic cor pulmonale.
Definitive treatment may consist of lung transplant or
combined heart-lung transplant depending on the etiology of the pulmonary hypertension.
Acute Right Ventriculqr Failure
Other causes of right ventricular failure include isolated right ventricular infarction, acute pulmonary
thromboembolic disease, tricuspid and pulmonic valvular insufficiency, dilated cardiomyopathy, pulmonary
hypertension, and infiltrative diseases involving the right
ventricle. Typically isolated right ventricle infarction and
pulmonary embolism present acutely, though thromboembolic disease may have an insidious presentation.
During acute right ventricular infarction, the presentation is typically that of chest pain with the standard electrocardiographic changes consistent with inferior ischemia. Right ventricular infarction is present in 20o/o to
40%o of inferior myocardial infarctions. Right-sided precordial leads will demonstrate ischemic changes in leads
V3R through V6R. Typically, AV node ischemia is present secondarily to occlusion ofthe right coronary artery
and various degrees ofAV nodal block are present with
associated bradyarrhythmias. The major determinate of
low cardiac output in patients with right ventricular
infarction is a decrease in the left ventricular preload.

The clinical presentation is that of hypotension, jugular
venous distention, and clear lung fields on auscultation.
Treatment after considering the appropriateness of
thrombolytic therapy versus emergent coronary angioplasty is directed at augmenting the preload of the left
ventricle and maximizing oxygenation of the right ventricular myocardium. This preload augmentation may
require substantial amounts of IV fluids. The use of
nitrates and diuretics may be detrimental as right ventricular preload needs to be maximized. Swan-Ganz
catheter monitoring should be strongly considered in
these patients, as nitrates and diuretics may result in a
precipitous drop in blood pressure.
or
of pulmonary embolism can be insidious
and difficult to diagnose. Frequently, the diagnosis can be

made by considering the pertinent historical and physical
findings in addition to arterial blood gas analysis demonstrating a significant Aa gradient. However, a ventilation
perfusion lung scan is required in most cases as may be
pulmonary angiography. The principal event is a sudden
elevation ofright ventricular filling pressures as the outflow of the right ventricle is obstructed. This decreases
pulmonary intravascular volume, which determines left
ventricular filling, LVEDP, stroke volume, and cardiac
output. Electrocardiogram findings, though in no way
specific, may consist of a right ventricular strain pattern
encompassing an 31 Q3 T3 pattern. The most common
electrocardiographic finding is that of tachycardia with
nonspecific ST:-segment changes.
Treatment is directed at stabilization with aggressive
correction of hypoxemia, augmentation of cardiac output
with fluids, and inotropic support. Definitive therapy may
consist of anticoagulation, thrombolytic therapy, or
embolectomy.
Other Considerations of Right-Sided Failure

The most common cause of right ventricular failure is
left ventricular failure. This fact is not surprising given
the hemodynamic considerations. In part this occurrence
is due to both ventricles sharing a common wall, the
interventricular septum, which contributes a substantial
part of right ventricular performance. In addition, as pul-
monary congestion develops, lung compliance decreases.

The result is increased right ventricular filling pressures,
increased work load, and increased right ventricular myocardial oxygen demand. In turn, the initiating pulmonary
congestion substantially decreases oxygen saturation levels, thus aggravating existing myocardial ischemia and
failure. In biventricular failure, treatment is directed at
primarily restoring left ventricular function. Invariably,

this involves inotropic support, preload and afterload
reduction, and diuresis as in isolated left ventricular failure. Aortic balloon pump augmentation maybe needed"
and if the patient is an appropriate candidate with endstage dysfunction, heart transplantation.
SELECTED READING
Alpert JS, Becker RC. Cardiogenic shock: elements ofetiology, diagnosis,
and therapy. Clin Cardiol 1993;16:182-190.
Chatterjee K. Pathogenesis of low output in right ventricular myocardial
infarction. Chest 1992;102'.590s-595s.
Dargie HJ, McMurray JJV Diagnosis and management of heart failwe. Br
Me d
99 4;308 :32
l-328.
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EunncoNcv MruclNn: TsB Conn Cunnrculul,r
Fishman AP, Palevsky HL Pulmonary hypertension and chronic cor pulmonale. Heart Dis Stroke 1993.'2:315-341.
Harley A. The management of heart failure: a matter of definition? Cardiovasc Drugs Ther 1993;7:661469.
Okura H, Takatsu Y. High output heart failure as a cause of pulmonary
hypertension. Int Med 1994;33:363165.
Patterson JH, Adams KF. Pathophysiology of heart failwe. Pharmacotherapy 1993;13:73s-81s.
Sherman S. Cor pulmonale, treatment implications of right versus left ventricular impairment. Postgrad Med 1992;9I(6): 227 -236.
Vandiviere HM. Pulmonary hypertension and cor pulmonale. South Med J
1 993;86:2s7-2s I 0.
Cardiomyopathy defines a group of myocardial disin which there is notable derangement in myocardial function, and is one of the most common conditions
affecting the heart following ischemic heart disease and
eases
hypertensive heart disease. The principal characteristic is

that of myocardial dysfunction in the absence of myocardial ischemia, hypertension, valvular disease, or pericardial disease. This group has often been classified according to primary cardiomyopathies in which the underlying
the
myocardium is
unknown, and secondary cardiomyopathies in which the

underlying pathologic process is known.
Traditionally these diseases have been classified into
three distinct types-dilated, hypertrophic, and restrictive-based on the type of pathophysiologic functional
abnormality present. This classification is most useful to
the emergency physician. Of these three distinct types of
cardiomyopathy the dilated variant is most comnon,
characteizedby dilation of all four chambers of the heart
and associated left ventricular failure. Most importantly,
there is a high association of dilated cardiomyopathy with
viral myocarditis. Hypertrophic cardiomyopathy is often
identified by left ventricular hypertrophy with preservation of contractile function. The least common variant,
restrictive cardiomyopathy, is characterized by rigidity of
the ventricular wall and associated diastolic dysfunction.
This functional classification is most useful when

describing the cardiomyopathies, but there frequently
exists a degree of overlap among the three principal
types.
ilated C ardio
the left ventricle, there is impaired contractility and

decreased myocardial oxygen utilization. The result is a
markedly reduced ejection fraction and increased endsystolic volume. Because of the left ventricular systolic
dysfunction, congestive heart failure is frequently
noted.
Clinical Features
ardio myop athy (2.2.2\
pathologic process involving
are usually patent and the cardiac valves are normal.

The focal point of the dilated cardiomyopathy (DCM) is
that of left ventricle failure. As a result of the dilation of
myop at hy
Pathophysiology
The dilated variant is most common and characteristically there is dilation of all four chambers of the heart
with the ventricles dilating to a greater degree than the
atria. Often there is a component of left ventricle hypertrophy, but the degree of wall thickening is often inconsequential to the extent ofdilation. Frequently, intraventricular thrombi are present, because of the turbulent
blood flow through the ventricles. The coronary arteries
Clinically, patients with DCM present with congestive

heart failure. The most common symptom is dyspnea
with exertion followed by orthopnea, paroxysmal nocturnal dyspnea, fatigue, pulmonary edema, and peripheral
edema. Physical examination often reveals rales on pulmonary auscultation and an S: and S+ on cardiac auscultation. In addition, a degree of mitral regurgitation is
often heard and less frequently tricuspid regurgitation.
When tricuspid regurgitation is present, a pulsatile
enlarged liver is noted on abdominal examination. Pulsus
alternans is often present with severe left ventncular failure.
The ECG frequently demonstrates sinus tachycardia

when failure is present. Most often though, there is electrocardiographic evidence of left ventricular enlargement.
Characteristically the ECG in dilated cardiomyopathy has
high voltage ratios of the R wave in V6 to that of leads I
to 3. Arrhythmias are the second most common manifestation of dilated cardiomyopathy and a wide variety of
both atrial and ventricular arrhythmias may be present,
with atrial fibrillation most common. Chest radiograph
displays left ventricular enlargement, a corresponding
increase in cardiac silhouette, and evidence of pulmonary
venous congestion. Two-dimensional echocardiography
ofall four chambers, decreased left ventricular ejection fraction, and elevated systolic and diastolic volumes.
reveals dilation
Therapy
Specific treatment of idiopathic dilated cardiomyopathy is unknown as the specific cause is not known. Therapy is the same as for congestive heart failure and is
symptom oriented. Physical activity is restricted to avoid
exacerbation of severe symptoms. Pharmacologic interventions consist of digitalis glycosides to increase myocardial contractility, diuretics to relieve preload, vasodilators and angiotensin-converting enzyme inhibitors to
decrease afterload and preload, and B-adrenoceptor
blockade. Beta-blockade, in addition to improving diastolic relaxation and thus improving myocardial oxygen
utilization, is beneficial for its negative chronotropic
effects. Anticoagulation is employed when intracavitary
Cerurovescut-AR DrsoRDERs
63
thrombi or thromboembolic disease is present. It must be

stressed that oxygen therapy is the principal cornerstone
of management.
Historically, left ventricular ejection fraction has been
the best predictor of mortality in dilated cardiomyopathy.
The mortality is substantially higher in patients with an
ejection fraction of less than20o/o than in patients with an
ejection fraction of 30Yo. Ventricular tachycardia is the
single most common event resulting in sudden death in
these patients, accounting for up to 80% of sudden cardiac death. Pharmacologic treatment of ventricular tachy-
because of a lack of symptomatology and the fact that the
cardia has neither decreased the incidence of sudden

death nor improved survival. Patients who have received
an automatic implantable cardioverter-defibrillator do
with
physical exertion and often heralded by syncopal
episodes. Interestingly, the presence of ventricular outflow obstruction has no association with the occurrence
of sudden death. Disqualification from athletics is
strongly recommended for patients with HCM because of
the strong association with sudden death and vigorous
physical activity. Other factors associated with an
increased risk of sudden death include a family history of
sudden death, marked ventricular wall thickening, and
have improved survival rates.
Hy p ertro p hi c
C ardio myop at hy
Hypertrophic cardiomyopathy (HCM) is a genetically

transmitted cardiac disease of autosomal dominant
expression. The clinical spectrum of HCM is extremely
broad, making definition very difficult. However, the
central feature is asymmetrical left ventricular hypertrophy without ventricular dilation in the absence of other
cardiac or systemic disease. Prevalence in the general
population has been estimated at O.loh, whereas in
patients with known heart disease it is estimated at 0.5o/o.
Ventricular outflow obstruction occurs in approximately
25Yo of patients with HCM, further defining two distinct
types of HCM: those with outflow obstruction and those
without ventricular outflow obstruction.
Pathophysiology
At the cellular level, the principal feature of HCM
is
one of circular arrays of myocytes and abnormal arrangement of large cardiac muscle bundles in the area of hypertrophy. Commonly there is disproportionate involvement
ofthe interventricular septum as opposed to the left ventricular free wall. The physiologic result is a marked
decrease in ventricular compliance. In turn, there is dias-
tolic dysfunction of the left ventricle with associated

impairment of ventricular filling and elevated filling
pressures. The symptomatology is directly related to the
diastolic impairment of the ventricle. Systolic function of
the left ventricle is preserved as evidenced by the frequent finding ofnormal cardiac output and ejection fraction. Often, end-diastolic and end-systolic volumes are
normal.
Clinical Findings
The clinical presentation of HCM is extremely variable
and a number of patients with HCM go undetected,
nonobstructive form predominates in approximately 75Yo

of the patients with HCM. In symptomatic patients, dyspnea, especially exertional dyspnea, is the most common
symptom followed by ischemic chest pain, palpitations,
syncope, and sudden death. The ischemic chest pain
results from an imbalance between the oxygen demands
of the markedly enlarged myocardium and the resultant
diastolic dysfunction. Paroxysmal nocturnal dyspnea,

orthopnea, and dizziness occur much less frequently.
dramatic presentation in young patients, 12 to 35
years ofage, is one ofsudden death often associated
nonsustained ventricular tachycardia on Holter monitor-
ing. Most patients remain asymptomatic until midlife,

however, and symptoms appear later at 40 to 50 years of
age, with severe functional limitation, ischemic cardiac
pain, and heart failure. In these adult patients with HCM,
the incidence of left ventricular outflow obstruction is

high and the most important predictor for sudden death is
nonsustained ventricular tachycardia.
Physical examination may reveal evidence of mild cardiomegaly with an apical systolic thrill and heave, and a
brisk carotid upstroke. Commonly there is an 54 present
corresponding to the systolic apical impulse. The first
and second heart sounds are normal. Often a harsh and
diamond-shaped systolic murmur is present. The radiation pattern is toward the lower sternal border, axilla, and
base of the heart but not the neck. Valsalva maneuver
increases in intensity by decreasing left ventricular filling. Correspondingly, the murmur is decreased by squatting and passive leg raising, which increase left ventricu-
lar filling.
The ECG is almost always abnormal. ST:segment and
Tlwave abnormalities are the most common finding followed by evidence of left ventricular hyperhophy. Marked
T-wave inversion is not infrequent in the precordial leads.
Abnormal Q-waves are found in the inferior and lateral
leads, and occur in 20oh to 50o/o of patients with HCM.
These Q-waves are a result of depolarization of the abnormal septal myocardium, hence the term septal Q-waves.
Anther cofirmon abnormality is ventricular amhythmia

occurring in approximately 75%o of the adult patients. Of
this subset, 25%o to 50% display supraventricular tachycardia, while atrial fibrillation occurs in only 5o/o to l0o/o
of patients. Nonsustained ventricular tachycardia is the
most significant rhythm disturbance as it is a harbinger of
sudden death and should be treated aggressively. Other
64 /
ErurncrNcv MrucrNn:
Tsr Conr
CunnrcuI-uM
less common abnormalities are those of an abnormal axis

and nonspecific P-wave abnormalities.
The chest radiograph is variable. The cardiac silhouette
can appear normal in size and configuration or may show
marked left ventricular enlargement. Typically there is
only a mild to moderate increase in cardiac silhouette.

Echocardiographic findings often show the cardinal manifestation of left ventricular hypertrophy. Specifically,
this is most often seen as asymmetrical septal hlpertrophy. Ifthere is outflow obstruction, a narrow ventricular
outflow tract is seen. Other echocardiographic features
are a small left ventricular cavity, reduced septal motion
during systole, decreased rate of mitral valve closure,
mitral valve prolapse, normal or increased posterior wall
motion, and partial closure of the aortic valve during systole. Cardiac catheteization often reveals decreased left
ventricular compliance, mitral regurgitation, and
dynamic left ventricular outflow gradient.
Therapy
Therapy is stratified among asymptomatic and symptomatic patients. In asymptomatic patients who have a
family history of sudden death, prophylactic administration of B-adrenergic blockers or verapamil is recom-
The prognosis for HCM is variable. The annual mortality has often been reported tobe2%oto 4%o, but recent
prospective studies have indicated a lower mortality of
lYo, indicating a more benign course than previously
thought. Clinical factors thought to result in an increased
risk of sudden death are young age of onset, family history of sudden death, history of syncope, and, most
importantly, nonsustained ventricular tachycardia.
Re strictiv e C ardio my op athy
Restrictive cardiomyopathy (RCM) is the least common of the cardiomyopathies. Primary RCM may be a
genetic disorder with dominant inheritance and incomplete penetrance. Causes of secondary RCM are the infiltrative diseases of amyloidosis, hemochromatosis, and
sarcoidosis, in addition to endomyocardial fibrosis and

Loffler's endocarditis. However, the rarity RCM frequently makes identification of the exact etiology difficult. Although RCM is the only cardiomyopathy without
accepted diagnostic criteria, several studies have defined
this specific disease as heart failure in the presence of a
nonhypertrophic, nondilated left ventricle with normal
contractility, and abnormal diastolic function.
mended. However,
Pathophysiology
Beta-blockade decreases myocardial oxygen demand by

increasing ventricular compliance and blunting the innate
chronotropic action of the myocardium. Calcium channel
blockers are the next line of therapy as they appear to
increase left ventricular diastolic filling. Verapamil has
been shown to improve the exercise tolerance in patients
who have failed to improve with beta-blockade. Nevertheless, the use of calcium channel blockers is not without some risk. They can induce hypotension, alter ati-
At the cellular level, restrictive cardiomyopathy typically demonstrates myocyte hyperhophy or interstitial
fibrosis or both. At the organ level, there is an absence of
in symptomatic patients, beta-blockade has been used extensively as the first agent.
oventricular conduction, and inhibit sinus node

automaticity.
Treatment of arrhythmias is of paramount importance as
nonsustained ventricular tachycardia is a prelude to sudden
in low doses has often been cited to

the risk of sudden death. Other antianhythmic
death. Amiodarone
decrease
agents have not been shown to reduce the incidence ofven-
tricular tachyarrhythmias. Digitalis glycosides are men-
significant coronary artery atherosclerosis, valvular heart

disease, and left ventricular hlpertrophy. The problem is
one of marked diastolic dysfunction with elevated ventric-
ular filling pressures and normal end-diastolic volumes.

In the past, restrictive cardiomyopathy was strictly characterized by the left ventricular pressure tracing during diastole, often described as a dip and plateau or square root
pattern because of the rapid rise and sudden leveling offof
the intraventricular pressure during diastole. This diastolic
intraventricular pattern
is no longer considered
absolute requirement for the diagnosis
an
of RCM. Impor-
is often a diastolic pressure difference

between the right and left ventricles of at least 5 mm Hg
in RCM. This pressure differential is exacerbated by phystantly there
tioned only to be avoided. They increase myocardial contractility and thus can exacerbate myocardial oxygen
demands. Their role is limited to the small select group of
patients with HCM who have atrial fibrillation with a rapid
ventricular response and no outflow obstruction. Nonpharmacologic treatment consists of surgical excision of a portion of the hypertrophic septum or mitral valve replace-
ical activity and is extremely useful in differentiating
ment, yet no definitive evidence exists that surgery

significantly prolongs survival. Some authorities recommend an implantable cardioverter-defibrillator if the
Clinical Findings
response to medical management is ineffective.
intolerance. The most cornmon symptoms are dyspnea and
between RCM and constrictive pericardial disease where

both the right and left ventricular diastolic pressures are
equal. The net result of this left ventricular diastolic dysfunction is the inability to increase cardiac output according to demand and in spite of a compensatory tachycardia.
Clinically, patients with RCM present with exercise
Cenuovescut-AR DTsoRDERS
fatigue. Chest pain of ischemic etiology is an infrequent
presentation. Often these patients display elevated pulmonary and systemic pressures. Jugular venous distention
is present and accompanied by an inspiratory increase in
venous pressure (Kussmaulis sign). Pulmonary auscultation may display rales consistent with pulmonary edema.
Cardiac auscultation reveals an 53, or 54, or both, and
mitral regurgitation is commonly noted. In advance diseased right ventricular failure is present with an enlarged
pulsatile liver, ascites, and peripheral edema or anasarca.
The ECG can display a vaiety of nondiagnostic findings. Atrioventricular conduction defects, intraventricular
conduction defects, and low precordial voltages are common. These defects are a result of the interstitial fibrosis
frequently pathognomonic of restrictive cardiomyopathies.
Chest radiograph may show a mild to moderate increase in
cardiac silhouette depending on the degree ofprogression
of the disease, although a normal cardiac silhouette is not
infrequent. Computerized tomography and magnetic resonance imaging may be employed to help differentiate
between constrictive pericardial disease and restrictive
myocardial disease. Echocardiography frequently demonstrates normal systolic function and a normal-sized left
ventricular cavity. Often there is an increase in left ventricular mass and infrequently an increase in the left ventricular wall thickness. If amyloidosis is present, a granular or
sparkling pattern is seen in the left ventricular wall.
Therapy
65
Larsen L, Markham J, Haffajee CI. Sudden death in idiopathic dilated cardiomyopathy: role of ventricular arrhythmias. PAC E 1993 ;l 6: 105 l-1 05 9.
Lessmeier
TJ, Lehmann MH, Steinman RT, et al. Outcome with
implantable cardiovert-defibrillator therapy for survivors of ventricular

fibrillation secondary to idiopathic dilated cardiomyopathy or coronary
artery disease without myocardial infarction. Am J Cardiol 1993;72:
91
l-9r5.
Marian AJ, Roberts R. Molecular basis of hypertrophic and dilated cardiomyopathy. Texas Heart Inst J 1994;21:6-15.
Momiyama I Mitamura H, Kimura M. ECG characteristics of dilated cardiomyopat\ -r E/e c trocordiol 1994;27 :323-328.
Siu S, Sole MJ. Dilated cardiomyopathy. Curr Opin Cardiol
1994;9:
337-343.
Tamburro R Wilber D. Sudden death in idiopathic dilated cardiomyopathy.
Am Heart J 1992;124:1035-1045.
Hypertrophic and Restrictive Cardiomyopathy

Borggrefe M, Chen X, Block M, et al. The role of the ICD in patients with
dilated and hypertrophic cardiomyopathy. PACE 1992:15:616426.
Davies MJ, McKenna WJ. Hypertrophic cardiomyopathy: an introduction to
pathology and pathogenesis. Br Heart J 1994;72:s2-s3.
Katritsis D, Wilmshurst
Pl
Wendon JA, et al. Primary restrictive car-
diomyopathy: clinical and pathologic characteristics. J Am Coll Cardiol

19911'18:1230-1235.
Kofilard MJ, Waldstein DJ, Vos J, ten Cate FJ. Prognosis in hypertrophic
cardiomyopathy observed in large clinic population. Am J Cardiol 1993;
72:939-943.
Maron BJ, Goldenberg IE, Pedersen WR. Management of hypertrophic cardiomyopathy. Heart Dis Stroke 1993 ;2:203-208.
McKenna WJ, Sadoul N, Slade AKB, Saumarez RC. The prognostic significance of nonsustained ventricular tachycardia in hypertrophic cardiomyopathy. Circulation 1994;90:31153117.
Spirito B Bellone P. Natural history of hypertrophic cardiomyopathy. Br
He art J 199 4;7 2 :sl0-s12.
Stewart Jl McKenna WJ. Management of arrhythmias in hyperhophic cardiomyopathy. Cardiovasc Drugs Ther 1994;8:95-99.
Spyrou N, Foale R. Restrictive cardiomyopathies. Curr Opin Cardiol 1994;
9:344-348.
As with the other cardiomyopathies, therapy in primary RCM is symptom directed. Treatment is oriented
Ischemic Heart Disease (2.2.3)
toward enhancing the diastolic function of the left ventricle. Diuretics can decrease preload and vasodilators may
affect afterload but only at the risk ofinducing hypotension. Digitalis glycosides can enhance contractility; however, digitalis is only of limited benefit since contractility
is minimally impaired. Two of the most important aspects
of therapy are arrhythmia control and minimizing conduction disturbances, as cardiac output can substantially
Annually 1.3 million individuals suffer from nonfatal

acute myocardial infarction (AMI). Of all deaths in the
United States approximately 45Yo are due to cardiovascular disease. This accounts for approximately 650,000
deaths per year from ischemic heart disease (IHD). Half of
these deaths are in patients under 65 and over 507o ofthese
deaths occur before the individual arrives at the hospital.
for secondary RCM, special attention is

given to infiltrative etiologies. Digitalis glycosides and
calcium channel blockers are contraindicated in amyloidosis. Significant toxicity can result when using these
agents as they bind to the amyloid fibrils. Alkylating
agents are often employed in the treatment of amyloidosis. Chelation therapy with deferoxamine is an important
adjunct in the treatment of hemochromatosis, as is steroid
improve. As
therapy in sarcoidosis.
Pathophysiologlt
Clinically the spectrum of IHD can be divided into

subsets ofdisease: chronic stable angina, unstable angina,
variant angina, AMI, and sudden cardiac death. These
TABLE
24.
Determinants of oxygen supply and demand
Oxygen demand
SELECTED READING
Dilated Cardiomyopathy
Di Lenarda A, Secoli G, Perkan A, et al. Changing mortality in dilated cardiomyopathy. Br Heart J 1 994;72(suppt):s46-s5 1.
Contractility
Wall stress (preload and afterload)
Heart rate
Oxygen supply
Diastolic pressure and duration
Coronary vascular resistance
66 /
EuBncrNcy MnorcrNn:
TABLE
24.
Cardiovascular
Tnr
Conn Cunruculurvr
Differential diagnosis for chest pain
Angina, acute myocardial infarction

(AMl), aortic dissection, pericarditis,
myocarditis, mitral valve prolapse
Pulmonary
Pulmonary embolism, spontaneous

pneumothorax, pneumonia, asthma,
chronic pulmonary obstructive disease
Gastrointestinal
Esophagitis (including esophageal

ref lux), esophageal spasm,
Boerhaave's syndrome, gastric peptic
ulcer disease, or reflux
Herpes zoster, chest wall pain
(coPD)
Other
Variant
Variant (Prinzmetalb) angina occurs primarily at rest.

Chest pain increases with severity and/or duration from
normal anginal pain. These attacks tend to recur at similar times and are most prevalent at night. ST:segment elevation is usually noted with chest pain. AV block, tachyarrhythmias, and bundle branch blocks are associated
with these attacks. Presently, it is felt that variant angina
is caused in part by spasm of the epicardial coronary
arteries alone or in combination with coronary artery disease.
constitute acute ischemic coronary syndromes (AICS).
Unstable
IHD occurs when myocardial blood flow fails to satisfz

myocardial oxygen demand resulting in ischemia. Causes
of myocardial ischemia and determinants of myocardial
oxygen supply and demand are listed inTable 2-3.
The majority of IHD is due to atherosclerotic coronary
artery disease (ASCAD). ASCAD is the focal narrowing
of the large and medium-sized coronary arteries due to
intimal proliferation of smooth muscle cells and deposition of lipids, both of which form plaques. In normal
Unstable angina represents a clinical state between stable angina andAMI. Unstable angina is thought to be due
to the progression in severity of atherosclerosis, disruption of atheromatous plaque, coronary artery spasm, or
hemorrhage into nonoccluding plaques with subsequent
occlusion developing over hours to days. It is important
individuals, as increased demand is needed, autoregulation increases coronary blood flow. Fixed obstruction or
stenosis of coronary blood flow prevents normal coronary blood flow and any increase in coronary blood flow
needed to compensate for increased demand. These
changes may reduce the supply and produce ischemia
with or without an increase in demand. Decreased cardiac
including discontinuation of prescribed medications or a

new underlying medical problem, emotion, or stress.
Classification proposed by Conti et al., divides unstable
output and hypotension cause decreased flow across the

fixed obstruction. Similarly, the fixed lesion prevents the
increased flow and oxygen delivery necessary with
severe anemia and the increased inotropic states of myocardial hypertrophy and tachycardia.
There are multiple causes of chest pain in addition to
IHD. The differential diagnosis is included inTable 24.
to consider factors in those patients with a history of

angina that have potentially exacerbated their stable state,
angina into three subsets distinguished by pain syndrome:

(1) recent onset exertional angina, occurring within 4 to
8 weeks previously; (2) chest pain with changing pattern,
which is described as increasingly more severe, longer in
duration, or increased requirement for nitroglycerin; (3)
chest pain occurring at rest. ST and T wave change s (66%
with ST depression and 33o/o with ST elevation) may
occur up to several hours after the episode, but no transmural infarction (new Q waves) is noted. Serum enzyme
levels show minor elevation without definite serial
changes. Troponin
indicating the presence of microinfarcts, is found to be elevated in about one-third of the
Angina (2.2.3.1)
episodes.
Stable
Myocardial Infarction
Stable angina is characterizedby episodic chest pain,

lasting usually 5 to l5 minutes. It is commonly described
as a pressure, heaviness, squeezing, or sharp discomfort,
which is classically localized to the retrosternal area and
radiates to the neck, jaw, and shoulders, or down the
inside of one or both arms. This is usually caused by exertion or stress (heavy meals, emotion, or exposure to cold),
and relieved by rest or sublingual nitroglycerin. ECG
shows changes including ST:segment depression;T wave
inversion occurs less than half the time, and ST:segment
elevation is seen less commonly. Elevation of cardiac
enzymes does not occur since the myocardium is not
injured.
Cocaine Induced
(2. 2. 3.
2)
Chest pain is the most common cocaine-related medical problem. The typical patient with cocaine-associated
myocardial infarction is a young tobacco-smoking male
with a history of repetitive cocaine usage. Cocaine causes
myocardial ischemia by increasing myocardial oxygen
demand while decreasing coronary blood flow through
vasoconstriction, enhancement of platelet aggregation, in
situ thrombus formation, premature atherosclerosis, left
ventricular hypertrophy, hypertension, and tachycardia.
Complications include dysrhythmias, rupture of the ventricular free wall, ventricular septum or papillary muscles,
Cenuovescur.qR Drsononns
and bradycardia secondary to inferior
MI. Cocaine
causes
complications in nearly all organ systems. Other associated symptoms may include dyspnea, anxiety, palpitations, dizziness, nausea, increased motor activity, hyperthermia, skeletal-muscle injury, and rhabdomyolysis.
Clinically, symptoms of chest pain location, duration,
or qualiry plus associated symptoms, are predictive of
myocardial infarction. Cocaine withdrawal may also

cause myocardial ischemia. Retrospective studies of
cocaine users with chest pain have found that the incidence of myocardial infarction ranges from 0 to 3lo/o.The
frequency ofAMI was approximately 60/o in two prospective studies.
Diagnosis
Electrocardiogram (ECG).
With
cocaine-associated
chest pain, the ECG is abnormal 1n 56o/o to 84o/o of

patients. J-point and ST:segment elevation often make the
identification of ischemia difficult due to early repolar-
ization or left ventricular hypertrophy. Patients with

cocaine-induced myocardial infarction in one study are as
likely as not to present to the hospital ED with a normal
or nonspecific ECG that has led to the discharge of up to
l5o/o of patients with cocaine-induced AMI.
Serum Enzymes with Cocaine-Induced AMI. Whether
or not they are undergoing infarction, approximately 50%
of cocaine intoxicated patients have elevations in the
semm creatine kinase concentration myocardial band
(CK-MB). This increase can occur in the absence ofAMI.
Continuously rising enzyme concentrations are more
likely to occur in patients with cocaine-induced AML
Treatment
Treatment of cocaine-induced AMI is patterned after

the treatment of IHD; however, there have been no welldesigned, randomized, prospective clinical trials. Benzodiazepines are the first-line treatment for cocaine-intoxicated patients with chest pain. Though mainly used for
their anxiolytic effects, benzodiazepines also diminish

cardiac toxicity by reducing blood pressure and heart
rate, thus decreasing myocardial oxygen demand. Treatment with aspirin is controversial; some authors feel it
should be administered to prevent the formation of

thrombi, while others feel it must be avoided in those
patients who are at risk for subarachnoid hemorrhage.
Nitroglycerin reverses cocaine-induced coronary

artery vasoconstriction, limits the size of AMI, relieves
symptomatic chest pain, and reduces infarct-related complications in patients with myocardial ischemia unrelated
to cocaine.
Those patients who continue to have severe chest pain
after the administration
of oxygen,
benzodiazepines,
aspirin, and nitroglycerin may be treated with either low-
67
dose phentolamine or verapamil, or thrombolyics. Calcium antagonists or phentolamine should be considered as

second-line therapy. Phentolamine, an ct-adrenergic antagonist, reverses cocaine-induced coronary -artery vasoconstriction. Use of a low dose (e.g.,
avoids the
hypotensive effects of the drug while maintaining its antiischemic effects. Calcium antagonists have no proven benefit in AMI unrelated to cocaine; however, verapamil does
reverse cocaine-induced coronary -artery vasoconstriction.
Thrombolytic agents do carry the risk of intracranial hemorrhage or dissection in severely hypertensive patients.
Administration of p-adrenergic blockers is not recommended. Their use has been found to exacerbate cocaineinduced coronary vasoconstriction, increase blood pressure, fail to control heart rate, increase the likelihood of
seizures, and decrease survival. Theoretically, an unopposed cr-adrenergic-mediated vasoconstriction can
occur, causing severe hypertension and coronary vasoconstriction that could lead to intracranial hemorrhage
and increased cardiac ischemia respectively. Additionally,
I -g)
p-adrenergic blockers can also produce profound

hypotension due to their negative inotropic and type Ia
antiarrhythmi c effects.
For treatment of a wide complex tachydysrhythmia

secondary to cocaine toxicity, lidocaine should be used
with caution due to its association with an increased incidence ofseizures. Cocaine additionally has a local anesthetic (quinidine-like) effect similar to other type Ia antidysrhythmics and tricyclic antidepressants. Though not
widely accepted, Parker and his colleagues suggest intra-
venous administration of bicarbonate in patients with

cocaine-induced MI. Sodium bicarbonate is felt to
reverse cocaine-induced QRS prolongation, and may rep-
resent a safe alternative for patients whose ventricular

arrhythmias immediately follow cocaine use.
The use of thrombolytic agents is controversial. Some
authors recommend that with new ST:segment elevations
and no improvement with the above therapy, it is reason-
able
therapy in the
of traditional contraindications. Others feel that
to give the patient thrombolytic
absence
although thrombolytic agents may be safe, they should not

generally be recommended. These authors cite the overall
mortality from cocaine-associated AMI, which
is
extremely low in patients who reach the hospital alive, and

the clinical benefit of thrombolytic therapy in this context
has not been studied. Additionally, young patients may
demonstrate a pseudoinfarct pattern (early repolarization)
on ECG, which may prompt unnecessary administration
of thrombolytic agents to patients without AMI who also
have a greater potential for intracerebral hemorrhage.
P ro gno
s is
/Di sp o s iti o n
Because of the difficulty in identiffing patients with

cocaine-associated chest pain who are at low risk for myo-
68 /
ErrmncrNcy MnorcrNr,: Tun Conn CunrucuLUM
cardial infarction, most patients are hospitalized. Patients
with cocaine-associated chest pain have a l-year survival

of 98%. Overall approximately 6%o wlllhave AMI, 4%o to
12% wlll have lifethreatening ventricular arrhyhmias,
and 5Yo to 7o/o wlll have chronic heart failure. Late complications can be predicted in the first 12 hours after
arrival in those with diagnostic ECG ischemic changes,
elevated CK-MB, and initial cardiovascular complications. Overall, a low index for admission should be held
for patients with cocaine-induced AMI, considering that
approximately 6% of these patients have myocardial
infarction. Overall, it is recommended that patients with
the above findings should be admitted to monitored beds.
Acute
Pathogenesis
Like unstable angina, multiple factors have been felt to

AMI, including coronary artery embolism, coronary artery spasm, progression of the atherosclerotic
process to the point of total occlusion, platelet aggregation and thrombosis at the site of preexisting narrowing,
plaque fissuring, and subintimal hemorrhage within an
intimal plaque. Ischemia leads to infarction affecting
lead to
both electrical depolarization and contractility of the

myocardial cell. Duration of ischemia determines the
extent of infarction. Prognosis, morbidiry and mortality
are determined by the amount of infarcted tissue; therefore, the time period from onset of symptoms to initiation
of therapy is the key to improved outcome.
Clinicql Features
There is no single presenting symptom uniformly diagnostic or specific to IHD. The classical presentation is an
individual who presents with severe substernal chest

pressure that lasts longer than 15 minutes; radiates to the
neck, jaq or medial aspect of one or both arms; and is
associated with nausea, vomiting, diaphoresis, dyspnea,
or syncope. Auscultation of the heart may reveal a tran-
sient S+ or apical systolic murmur indicative of mitral

regurgitation. Unfortunately, the physical examination in
all types of IHD may be deceptively normal.
Atypical presentations can include abdominal pain or
vague chest discomfort. The elderly are more likely to
present with nonspecific symptoms, dizziness, weakness,
dyspnea, syncope, nonretrosternal chest pain, epigastric
pain, or with no pain in comparison with younger
patients. Silent coronary artery disease is significant IHD
without clinical symptoms and is found in 2.5%o to l0o/o
of middle-aged individuals. Those diagnosed with silent
TABLE
2$.
Major risk factors for ischemic heart disease
Age
Family history (Ml in first-degree relative age <55)
Smoking
Hypercholesterolemia
Hypertension
Diabetes mellitus
Male gender
Morbid obesity
Cocaine
artery bypass graft (CABG) patients. During the initial

interview, questioning should be directed toward cardiac
risk factors to more quickly distinguish cariogenic chest
pain from other causes (Table 2-5).
Pathophysiology
AMI can be divided into Q wave and non-Q wave MI.

This distinction is used to discern whether acute ischemia
is confined to the subendocardial area (manifested by ST
segment depression) or involves the transmural wall
(manifested by presence of Q waves and usually ST-segment elevation). Non-Q wave AMI accounts for 30% to
40% of AMIs. Of particular concern with these individuals is extension of the infarct into a transmural infarction.
Q wave infarctions tend to be larger, damaging more
myocardial tissue, resulting in higher peak serum CK-
MB levels and lower ejection fractions. Non-Q
wave
infarctions possess a lower in-hospital mortality rate, yet

are more likely to be complicated by recurrent infarction
or subsequent angina. Long-term mortality tends to be
equal after about 3 years.
Diagnosis and Ancillary Tbsts
Precisely differentiating these IHD syndromes (a.k.a.
AICS) may require the use of sophisticated diagnostic
tools and prolonged observation. Presently, the use of
ancillary studies for the evaluation of ED patients with
chest pain has focused on three key issues: (1) early iden-
tification of those with AMI to reduce time to definitive

treatment (percutaneous transluminal coronary angioplasty or thrombolytic therapy) and minimize the extent
of myocardial infarction; (2) differentiating acute
ischemic coronary disease from nonischemic causes of
chest pain so as to avoid unnecessary costly hospitaliza-
tions; apd (3) identiffing those patients with AMI who

would be discharged inadvertently from the ED.
Electrocardiogram and Interpretation
AMI usually
have vague complaints of discomfort. A

high index of suspicion must be taken particularly with
diabetics, the elderly, alcoholics, and post-coronary
Although ECGs are used extensively in the ED, they

are diagnostic in only 25o/o to 50o/o of patients subse-
Cerurovescur-AR DISoRDERS
TABLE 2-6. Localization of infarction using Q waves and
ST elevation
Posterior
Lateral
lnferior
Anterior
Right
ventricular
Large R with ST depression in Vr and

Vz, mirror test
Q's or ST elevation in I and AVL
(circumflex coronary artery)
Q's or ST elevation in ll, lll, and AVF
(R or L coronary artery)
Q's or ST elevation in Vr, Vz, Vs, and Vq
(left anterior descending coronary
artery)
ST segment elevation greater than
1 mm in lead Vsn and/or V+n
quently diagnosed with AMI. A normal or nonspecific

ECG does not exclude ischemia and should not negate
the need for hospital admission. Due to these factors an
estimated 2o/o to 4oh of ED patients with AMI are undiagnosed while others are inappropriately included or
excluded from aggressive reperfusion therapy. The most
frequent ECG findings in chronic IHD are nonspecific
ST andT wave changes. These are usually defined as ST:
69
of repolarization, causing it to occur in the endocardialto-epicardial rather than the normal direction. Usually T
wave inversion is noted in the same leads as acute infarction (ST elevation and Q waves). Isolated (noninfarction)
ischemia may also be located by those leads where T
wave lnverslon occurs.
The l5lead ECG may better identifu the extent of

injury in patients suffering from an inferior AMI and
thereby have therapeutic implications for the emergency
physician (Table 2-7).In90%o ofpalients, the right coronary artery supplies the inferior wall of the left ventricle
as well as the right ventricle. RV infarction occurs in

about 60% of patients who suffer an inferior MI and
becomes hemodynamically significant in approximately
10% of left ventricular inferior wall and right ventricular
AMI. Additional leads in conjunction with the standard
l2-lead ECG attempt to improve sensitivity in the identification of right ventricular and posterior AMIs associated with inferior AMIs.
Serum Markers
segment depression or elevation of less than 1 mm, and T
wave flattening or inversion.

Q waves indicate infarction. A significant Q wave is I
mm wide (.04 sec) or greater than or equal to one-third
the amplitude of the QRS complex. Q waves remain for a
lifetime and are no indication of the acuity of an infarct.
ST:segment elevation indicates an acute process an4 with
associated Q waves, indicates an acute transmural infarct.
ST segments return to baseline with time. Non-Q wave
infarction is suspected with significant ST:segment elevation alone. ST:segment depression can represent subendocardial infarction, a variety of non-Q wave infarction.
Localization of ischemia can be identified by the leads in
which it occurs (Table 2-6).
T wave inversion indicates ischemia. The inverted T

wave of ischemia is symmetrical (left half and right half
are mirror images). T waves are normally in the same

direction as the QRS and often become inverted because
of ischemia or infarction. Ischemia reverses the sequence
TABLE
2-7. lndications for 1S-lead
ECG
ST-segment depression or suspicious isoelectric STsegments in Vr through V3

Borderline ST-segment elevation in leads Vs and Vo or in
leads ll, lll, and aVF
All inferior Mls (ST-segment elevation in leads ll, lll, and
aVF)
lsolated ST-segment elevation in lead Vr or ST-segment
elevation in lead V1 or ST-segment elevation in lead Vr >
Yz
Symptoms suggestive of RV ischemia (e.9., epigastric pain,

significant hypotension after nitroglycerin, administration,
or hypotension with distended neck veins and clear lung
fields)
Identifuing AMI candidates for reperfusion as soon as

possible in the ED has become a priority. Unfortunately,
the accurate diagnosis of AMI in the early hours remains
difficult. Clinical signs and symptoms and ECG manifestations are not sufficiently specific to distinguish noncardiac chest pain from necrosis, let alone from transient
myocardial ischemia. As few as llo/o of patients hospitalized with suspected acute myocardial necrosis are eventually confirmed to have AMI; however, up to 4% who
have AMI are released unintentionally from the ED,
which is even more disturbing. Serum markers may have
the potential to rapidly identify candidates for aggressive
therapy and address the unintentional discharge of AMI
patients from the ED.
Serum CK-MB. Traditionally, patients have
been
admitted for at least 24 hours in order to be ruled out for

AMI. Routine assays indirectly measure CK and CK-MB
concentration by determining enzymatic activity, not the
quantity or mass of enzyme. Due to the variable normal
serum levels of CK-MB, its presence in noncardiac muscular tissues, and its delayed entry into the serum (3-6
hours), a single CK-MB determination is not reliable in
eliminating the diagnosis of AMI. However, patients with
no ST:segment elevation on their initial ECG and two
consecutive negative CK determinations, performed at
least 6 hours apart, have only a 2Yo chance of having
AMI.
Rapid CPrK. Newer assays of CK-MB directly measure enzymatic mass, not activity, and give results within
minutes using immunologic techniques. In a multicenter
study, Gibler and associates found that of the patients
with nondiagnostic ECGs, 55 out of 64 had AMI; 80%
had a positive ED serial CK-MB enzyme study within 3
70 /
EvrncrNcy MntlcrNr: Tun Conn CunnrculuM
hours of presentation. They reported a 99.6% negative

predictive value for no AMI (94% specificity). This
study, however, did not assess the application of cardiac
enzyme measurement for patients under consideration for
discharge from the ED. Addressing this issue directly,
Green et al. found negative predictive values of 100%. In
summary, although further research is needed, these studies suggest that the new rapid CK-MB assays may assist
the emergency physician in identifying some patients
with unsuspected AMI and inadvertent discharge from
the ED, or prevent admission to unmonitored beds.
MB Subforms. CK-MB exists as single form in tissue
(MB2).
is modified by the plasma enzyme car-
It
boxypeptidase-N, yielding
its
subform
MBl.
Until
recently, obtainable assays lacked adequate sensitivity to

detect the individual MB subforms in normal plasma,
preventing the use of MB subforms for early diagnosis of
infarction. According to Puleo and associates, an

MB2:MBl ratio of 1.5 or more in samples collected 6
hours after AMI were 96% sensitive compared to 48o/o for

the conventional plasma creatine kinase assay. Rapid
determination of MB subforms may become important
for the early determination of AMI in the ED.
Myoglobin. Myoglobin is a cellular oxygen-carrying
protein normally located in all muscle tissue, but when
noted in serum is a sensitive indicator of muscle damage.
Myoglobin has been evaluated as an indicator for myocardial injury. Serum levels can be elevated within I to 2
hours after symptom onset, peaking at 4 to 5 hours after
AMI. Gibler et al., in a small study of 59 patients, found
that serum myoglobin elevation at 3 hours identified all
21 patients with AMI. CK-MB determination was eventually positive in 19 of these 21 patients; initially, CKMB was positive in only three individuals. Kilpatrick et
al. proved that myoglobin concentration was as specific
as CK-MB concentration (93% in both cases), but myoglobin had a sensitivity of l00o/o at 1.5 hours after admission in comparison to CK-MB at 4 hours after admission.
Brogan et al. found in a l-hour observation period that an
increase
in serum myoglobin of 40 ng/ml increased the
sensitivity of the myoglobin assay forAMI in ED patients

from 73o/o to 9lo/o. Myoglobin from skeletal muscle is
indistinguishable from myocardial tissue and lacks specificity for myocardial infarction. It is also elevated in
skeletal muscle trauma, shock states, and renal failure.
Immunoturbidimetric myoglobin assay has been introduced requiring only 2 to l0 minutes to perform.
Troponin. Troponin complex consists of three protein
subunits
(!
I, and C) each with different structures and
functions. The troponin complex, a structural component

of cardiac muscle cells, has distinct differences in the
subunit amino acid composition and can differentiate cardiac from skeletal muscle immunologically. Cardiac-specific troponin in serum is measured using monoclonal
immunoassay, providing a specific tool for diagnosis of
AMI. Bakker and colleagues, in a prospective study,
found that troponin T had the highest sensitivity for prediction of AMI; however, it also had the highest falsepositive rate. According to Adams et al., increases in troponin I do not occur even when plasma levels of CK-MB
are increased with severe acute or chronic skeletal mus-
cle injury unless associated cardiac injury is present.

They suggest that measurements of troponin I provide
information comparable with that from echocardiography
in clarifuing the presence or absence of cardiac injury
when elevations of CK-MB occur.
Serum markers currently are an adjunct to clinical
evaluation. Presently, normal serum levels of CK-MB,
myoglobin, and troponin shed light on the absence of
myocardial infarction, but do not rule out ischemic heart
disease. The decision to discharge a patient is based on
the history and an assessment of the patient's potential for
suffering a cardiac event if sent home, not solely on a laboratory result. Additionally, these ancillary tests should
not prevent timely treatment of individuals with a history
and symptoms consistent with AMI.
Nuclear Imaging. Thallium-2Ol is a nuclide that following intravenous injection distributes into myocardial
tissue and is therefore dependent on coronary blood flow
and the ability of myocardial cells to extract the thallium.
It is used to identifyAMl and reversible ischemia. Areas of
transient ischemia from stenosis or spasm do not actively
take up thallium and
will therefore
appear as an area lack-
ing isotope on the scamed image. Studies have suggested

that in those patients presenting within 6 hours thallium201 can identifu AMI with 100% sensitivity. This may be
useful for patients presenting to the ED with chest pain
suggestive of myocardial ischemia, but with nondiagnostic
ECG. The advantage is that it is a relatively noninvasive

procedure that can be obtained quickly. Cost, decreasing
accuracy with time, inability to distinguish old from new
infarct, and overall poor availability limit its use.
kchnetium-99m. Technetium-99m is a radioisotope
that is more readily available than thallium-2O1 because
it is generator produced. It is taken up by myocardium in
proportion to blood flow. Varetto et al. found that perfusion defects persist in patients with angina for several
hours after resolution of symptoms. In studies by Hilton
and Varetto, sensitivity was 100% and specificity was
83oh to 92o/o in patients with active chest pain and a nondiagnostic ECGs. For recognizing AMI and myocardial
ischemia, the nuclear imaging studies described suggest
high sensitivity and specificity. However, factors such as
the availability of radionuclides, availability of trained
personnel to interpret the image, and the study's cost may
limit their use.
Treatment of Ischemic Heart Disease
Treatment of IHD can be divided into both inpatient

and outpatient therapy. Outpatient treatment of stable
Cenorovescur-A,R DrsoRDERs
TABLE
2-8. Becommended doses for AMI
treatment
adjuncts
Agent
Nitroglycerin,
sublingual
Nitroglycerin,
intravenous
Aspirin
Heparin
0.4 pg every 5 min x 3 doses

Begin at 10 pg/min and titrate as needed
160-325 mg/day
80 units/kg bolus, then 10 units/kg/hr
infusion or 5000 units bolus then 1000
units/hr
Beta-blockers
Esmolol
Metoprolol
500 pg/kg lV over 1 min, then

50 pg/kg/min titrated max dose of 200
pg/kg/min;or 5 mg lV every 5 min x 3,
then 50 mg every 6 hr orally
5 mg lV every 5 min x 2, then 100 mg/d
Atenolol
1-4 g IVP over 5-20 min
Magnesium
Morphine
2-5 mg every 5-1 5 min

1-4 g IVP over 5-20 min
2-5 mg every 5-15 min
orally
angina includes correction of modifiable risk factors and

drug therapy. The latter usually consists of nitroglycerin,
p-adrenergic antagonists, and calcium channel antagonists. For patients with unstable angina or AMI, the individual should simultaneously be examined while oxygen
is given, intravenous access is established, and cardiac
monitor and pulse oximeter placed. Detection of other
causes
of
chest pain, particularly thoracic aortic
aneurysm, pneumothorax, and pneumonia, must be ruled
out. Medications used in the ED inpatient setting

listed in Table 2-8.
are
Oxygen. Supplemental oxygen should be provided to

all patients with AMI. The size of infarct may be reduced
with supplemental oxygen. A non-rebreather mask, continuous positive airway pressure, or endotracheal intubation should be strongly considered if supplemental oxygen fails to correct significant hypoxemia.
Nitroglycerin Nitroglycerin (NTG) helps relieve the
pain from myocardial ischemia by dilating epicardial
arteries, increasing collateral blood flow, and decreasing
left ventricular preload. Additionally, it has been found to
have antiplatelet effects, and will improve ventricular
remodeling and reduce infarct size and mortality. Treatment should be directed toward pain relief with initial
administration of NTG 0.4 mg tablets sublingually given
every 3 to 5 minutes. If chest discomfort continues after
three doses, an intravenous NTG drip should be initiated
(50 mg NTG in 250 ml dextrose 5% in water) and titrated
until pain subsides while keeping systolic blood pressure
greater than 90 mm Hg. The initial infusion rate is 10 to
20 tg/min. It can be increased in increments of 5 to l0
ug/min at 5- to l0-minute intervals until chest discomfort

resolves, or the mean arterial pressure decreases by l0%.
The majority of patients respond to infusion rates of 50 to
77
200 ug/min. The major complications of and contraindications to nitrate therapy (SL or IV) are hypotension and
marked bradycardia. It should not be used with a systolic
blood pressure of less than 90 mm Hg. Hlpotension usually will respond to small boluses of normal saline.
Aspirin. A rapid antithrombotic effect due to inhibi-
tion of
thromboxane and cyclooxygenase-dependent
platelet aggregation is produced by aspirin. Additionally,

aspirin has been found to prevent reocclusion and reinfarction. In the Second International Study oflnfarct Survival (ISIS-2), aspirin alone exhibited a23o/o reduction in
mortality rates, a 49o/o reduction in nonfatal reinfarction
rates, and a 46oh reduction in nonfatal stroke rates. Adequate therapy is 160 to 325 mg qday.
Heparin. The role of heparin in the treatment of AMI
has been controversial. It works by catalyzing antithrombin III, which inactivates thrombin and factor Xu. More
importantly, heparin has been found to reduce reocclusion and reinfarction (20 to 30%) and mortality rate (16
to 2lo/o). Its antithrombotic activity complements the
antiplatelet activity of aspirin to prevent progression of

ischemia to AMI. Current recommendations by Sirois for
the use of heparin in the treatment ofAMI are as follows:
heparin is indicated if no thrombolytic agents are given,
but the dose and route of administration remain controversial; no added benefit has been shown if streptokinase
is used, and heparin is contraindicated due to subsequent
increased risk of hemorrhage. However, if tissue-type
plasminogen activator (t-PA) is to be given or primary
percutaneous transluminal coronary angioplasty (PTCA)
is to be conducted, then IV heparin is necessary to pre-
vent reocclusion. Initial dosing should be based on

weight to ensure consistent and adequate anticoagulation.
The recommended weight-based dose of heparin is an
initial bolus of 80 units/kg followed by a constant infusion rate of l8 units/kg/h. Many institutions use an initial
5,000-unit IV bolus, then a continuous infusion of 1,000
units an hour.
Beta-Blockers. p-Adrenergic blockers should be considered when symptoms of chest pain are not resolved or
are poorly responsive to NTG therapy. Nonselective padrenergic antagonists have been shown to be useful in the
treatment of angina, but only moderately useful in patients
with unstable angina or AMI. Trials have shown conclusively that beta-blockers decrease infarct size, reduce reinfarction, and decrease mortality due to increased coronary
artery blood flow, decreased myocardial contraction, and

decreased heart rate and thus decreased myocardial oxygen demand. They have also been found to decrease cardiac rupture, reduce the incidence of ventricular fibrillation, and reduce overall mortality. The greatest beneficial
effect is achieved when beta-blockers are started early in
ofAMI. Selective agents such as esmolol

and metoprolol preferentially inhibit Br receptors of the
heart at low and intermediate doses. In those patients with
unstable angina or AMI who do not respond to aspirin,
the management
72 /
EnrncnNcv MnorcrNn: THn Conn CunnrcuI-uM
intravenous NTG, and intravenous heparin, intravenous

beta-blockers such as esmolol, metoprolol, or atenolol
should be considered. This is especially true in patients
with
tachycardia and/or hypertension. The dose of

esmolol is 500 ug/kg intravenously over I minute followed by an intravenous infusion at 50 rgikglmin titrated
to a maximum dose of 200 ug/kg/min. Metoprolol and
atenolol can also be used. Beta-blockers are, however,
generally contraindicated in patients with asthma,
obstructive lung disease, congestive heart failure, bradycardia, atrioventricular block, hypotension and variant
angina, and insulin-dependent diabetes mellitus.
Morphine. Morphine blocks central sympathetic efferent discharge, leading to a reduction in cardiac work by
reducing both preload and afterload, therefore decreasing
myocardial oxygen demand. Pain and anxiety reduction
to
leads
decreased circulating catecholamines and
decreased myocardial irritability. When pain from myocardial ischemia is not otherwise rapidly controlled by
other methods, 2 to 5 mg IV boluses of morphine every 5
adverse effects
include respiratory depression, hypotension, and bradycardia; the latter two respond well to fluids and atropine,
respectively. Morphine is contraindicated in patients with
significant bradycardia or hypotension.
Calcium Channel Blockers. The use of calcium channel blockers is controversial in unstable angina and AMI.
In patients with unstable angina who do not respond to
aspirin, intravenous nitroglycerin, intravenous heparin,
and intravenous esmolol , dlltiazem may be considered.
Although diltiazem has been shown to decrease early
reinfarction and recurrent angina in patients with non-Q-
to l5 minutes should be used. Potential
wave
AMI, neither diltiazem nor verapamil has been
shown to decrease mortality. Dllliazemmay be given as a

bolus of 25 mg IV over 5 minutes followed by a l5-mg/hr
infusion. In small studies of unstable angina and AMI
patients, nifedipine was found to increase mortality and
therefore is not recommended.
Magnesium. In the second Leicester Intravenous Magnesium Intervention Trial (LIMIT-2), the use of magne-
sium resultedina25%o reduction in left ventricular fail-
in 28-day mortality rates;

of other interventions were uncontrolled. The ISIS-4 trial showed no benefit from magneure and a
24o/o reduction
however, the effects
sium; however, magnesium therapy was delayed more

than 12 hours. Though the mechanism is unknown for
this reduction in mortaliry some feel it may be due to a
direct cardioprotective effect. It is unclear how much of
the observed reduction in mortality can be ascribed to
magnesium alone. Patients with AMI who stand to gain
the most are those who are not candidates for thrombolytic, aspirin, or beta-blocker therapy. Magnesium,
when considered, is a one-time dosage of I to 4 g given
IV over
5 to 20 minutes.
Lidocaine. The routine use of prophylactic lidocaine

has been shown to have no added benefit and may
increase mortality rates; therefore, its prophylactic use

should be discontinued. Indications for its use in patients
with AMI include more than six premature ventricular

contractions (PVC) per minute, closely coupled PVCs
(R-on-T), multiform PVCs, bursts of three or more
PVCs, ventricular tachycardia, and ventricular fibrillation.
A g g res s iv e Rep erfu s i o n Th
e rapy
Aggressive reperfusion therapy (ART) includes thrombolytic agents or PTCA. Currently three thrombolytic
agents are available: r-TPA, streptokinase, and urokinase.
The usage of the first two is by far more common. ART

strategies focus on the combination of thrombolytic therapy and PTCA, either concurrent, immediately within 90
minutes, within 18 to 48 hours, or delayed for 5 to 14
days. None of these combined strategies has been shown
to be more eflective than thrombolysis alone. Addition-
ally, performing PTCA after failed thrombolysis
has
shown mixed results.
Thrombolytic Therapy
Cessation of blood flow to the canine myocardium initiates necrosis in approximately 20 minutes, with complete transmural necrosis occurring between 3 and 6
hours. Minimizing infarct size is associated with

improved residual left ventricular function. This is the
major factor in decreasing both early and late nonarrhythmic mortalify after AMI. A decrease in mortality of
two to three patients per 100 treated and a reduction in
relative mortality of 22Yo in comparison with nonthrombolytic management versus placebo were found in pooled
results of five major randomized trials. In most cases, the
decision to administer thrombolytic therapy should be
made based on the clinical presentation and the initial
ECG alone. Though some authors feel that these alone
should be the only decision criteria, it is my opinion that
expedient comparison of old ECGs and a portable chest
radiograph
to rule out dissecting aneurysm,
if
readily
available, are important. The time from symptom onset to

initiation of treatment has been consistently shown to be
the most important factor in reduction of mortality. The
Gruppo Italiano per lo Studio della Sopralvivenza nel

'Infarto Miocardico (GISSI-I) showed patients treated
after 6 hours had no significant reduction in mortaliry
while those treated within I hour of symptom onset had a
of 50%o.Indications and
contraindications are shown in Tables 2-9 and2-10.
Streptokinase. In two placebo-controlled megatrials of
mortality (GISSI-I and ISIS-2), mortality decreased by
23%o and 300/o, respectively, in patients with AMI randomly assigned to IV streptokinase (SK) or compared
with placebo within 6 hours of symptom onset. Initially,
relative reduction in mortality
Cenorovescur.r\R DTsoRDERS
TABLE 2-9. lndications for thrombolytic therapy
Probable harm
lndications
Chest pain of >30 min and <12 hr

duration
ECG ST-elevation (>1 min >2leads)
Anterior, inferior or RV AMI
New bundle branch block LV
dysfunction
Chest pain and ECG changes
persist after administration of
sublingual nitroglycerin
Age <75 yr; for patients 76 Yr and
older, the decision to administer
thrombolytic agents should be
made on an individual basis
Symptoms 12-24 hr
Non-Q-wave AMI
ECG ST-depression
Cariogenic shock
Unstable angina
relevant superiority
73
of t-PA considering the similar
patency rates (70o/o vs 7 5oh) at 3 hours versus the statistically significant increase in hemorrhagic stroke rates of
those who receive t-PA. Unfortunately, though both

agents result in recanalization of infarct-related coronary
arteries, both have a high-grade stenosis rate, which persists after thrombolysis. Cost differences are substantial.
SK costs approximately $100 to $300, while t-PA costs
$2200 to $2500.
Thrombolytic therapy limitations include limited

applicability (only 15 to 25oh eligibility); failure to
achieve artery patency (15 to 40o/o); continued presence
subgroup analysis could only prove a benefit in patients

treated within the first 6 hours after pain onset, those hav-
ing a first AMI, those younger than 65 years old, those

with anterior AMI, and those with no or few signs of
heart failure. Howeveq further trials have shown benefit
in older patients, patients with inferior AMI, patients with
a longer duration of pain, and those individuals with pulmonary edema and AML Due to the potential for allergic
reactions, SK is not recommended to be readministered
for a period of 5 days up to 6 to 12 months after previous
use, or to patients with a history of a recent streptococcal
throat infection.
Recombinant t-PA. Recombinant t-PA (t-PA) activates
the fibrinolytic system, catalyzingthe conversion of plasminogen to plasmin. t-PA has the shortest half-life, has no
allergic side effects, and is clot-specific. The 1988 Anglo-
Scandinavian Study of Early Thrombolysis (ASSET)

placebo-controlled trial demonstrated a significantly
lower mortality of 28oh for patients treated with t-PA
within 6 hours of symptom onset. Initially, studies found
a superior ability of t-PA to open arteries at 90 minutes
compared with SK. Controversy surrounds the clinically
of high-grade residual stenosis after successful thrombolysis (75 to 80%); immediate reocclusion (15 to 20%);
serious bleeding complications (3%), and poor results in
certain subgroups. The most common reason for exclusion is symptoms without ECG ST:elevation.
Early administration is the most critical determinant of
response to and outcome with thrombolytic therapy. The
prehospital Myocardial Triage and Intervention Trial

(MITD group from Seattle showed a mortality rate from
AMI of 1.2%with initiation of treatment within 70 minutes; however, patients treated after 70 minutes had a
more "normal" AMI mortality rate of 9o/o.
Thromb o lyti c C omp li cations
Complications of IV thrombolytic therapy include the

inability to open 20Yo of occluded coronary arteries,
reocclusion in approximately l5Yo to 20oh of patients,
and a 0.5o/o to l.0o/o incidence of intracranial bleeding.
Megatrials addressing mortality directly compared t-PA
and SK randomized to more than 100,000 patients. Only
small absolute differences were found between thrombolytic agents, in terms of lives saved and major complications, including hemorrhagic cerebral vascular accident. Arrhythmias are commonly observed and are
neither dangerous clinically nor consistent markers of
TABLE 2-1O. Contraindications to thrombolytic therapy

Absolute
Relative
Hemorrhagic cerebrovascular accident within last 6 months

Uncontrollable acute hypertension (>180 systolic, 120 )
diastolic
Major surgery or trauma less than 2 weeks before or spinal
or intracranial surgery within 2 months
Cerebral aneurysm, arteriovenous malformation or
neoplasm
Active internal bleeding, such as gastrointestinal
Bleeding disorder or anticoagulation
Aortic dissection or pericarditis
Pregnancy
Recent head trauma
Previous use or allergy to streptokinase (SK therapy should
not be repeated within 6-12 months; this is not a
contraindication for use of other agents)
Major surgery or trauma more than 2 weeks before

Puncture of noncompressible vessel
Prolonged or traumatic cardiopulmonary resuscitation
Hemorrhagic retinopathy
Recent gastrointestinal or genitourinary bleeding (within 10
days)
Bacterial endocarditis
Recent transient ischemic attack
Known left heart thrombus
Active peptic ulcer disease
History of chronic, uncontrolled hypertension (diastolic
>100 mm Hg), treated or untreated
Any other reason that would result in a signilicant bleeding
hazard
74 /
EuencrNcy MnorcrNr: THr Conn CunrucuLUM
reperfusion. Routine use of antiarrhythmics is not recommended universally. Rapid return to baseline or normalization of the ST segment suggests opening of the
occluded vessel. A small or negligible change may indicate lack of reperfusion. Patients with AMI who do not
experience a decrease in ST:elevation may therefore be
candidates for PTCA.
PTCA
PTCA is probably superior to thrombolysis in patients

it can treat the underlying fixed
obstructed coronary artery lesion in addition to relieving
the acute thrombosis. In three randomized trials, lower
rates of death and recurrent ischemia, no intracranial
hemorrhage, less in-hospital complications, and no
increase in hospital costs were noted. For cardiogenic
shock it is clearly the treatment of choice. Studies of primary PTCA have shown it to be effective in obtaining
infarct-related artery patency, preserving myocardium,
and reducing mortality. Additionally, PTCA is more
with AMI because
effective in preventing reocclusion of the infarct-related

artery, and it has a lower incidence of recurrent ischemia,
reinfarction, and death. These effects are particularly evident in patient subgroups with increased risk of bleeding
complications, the elderly, those in cariogenic shock, and
those who have undergone previous bypass surgery.
Patients with multivessel disease, however, do worse with
primary PTCA. The time constraint is a major limitation
of primary PTCA. In all published studies to date, the
time to initial balloon inflation has been less then 60 minutes from presentation. Additionally, 8Yo to 30% of
patients have unfavorable anatomy upon angiography;
only 18% of hospitals perform PTCA; and trained staff
must be on call 24 hours a day with cardiothoracic
surgery backup.
Conclusion
The standard of care is now reperfusion therapy for all
patients who meet eligibility criteria. Primary PTCA
appears to be the best overall strategy for ART for those
who are ineligible or poor candidates for thrombolysis
and when it is available in less than 60 minutes. If the
projected time to PTCA is longer than 60 minutes and the
patient meets eligibility criteria, then thrombolysis

should be considered and is indicated. More important
Disposition
An undiagnosed AMI is the fourth most common clinical entiry but ranks first with respect to monetary
awards for malpractice suits against emergency physicians. A normal or nonspecific ECG does not exclude
ischemia nor should it negate the need for treatment and
hospital admission. Such decisions continue to be based
on risk factors, clinical assessment, judgment, and most
importantly, the history.
For patients with AICS, it is most important for the
emergency physician to recognize and admit patients
with AMI and unstable angina. Many hospitals use ED
observation units to deliver low-cost, short-stay treatment
and diagnostics for patients with chest pain. This provides
a rapid" cost-effective way to rule out AMI for large numbers of people. As managed care progresses, a more rapid
method of ruling out or ruling in AMI in the outpatient
setting is more likely to be used" not only to decrease hospitalization, but also to target patients expected to benefit
most from aggressive therapy.
At two institutions that use ED chest pain observation
units, 7 4o/o of patients were ruled out in the ED observation unit and were released home. Another 24oh were
admitted with changes in their ECG, changes in the CKMB levels, recurrent unexplained symptoms, or positive
stress thallium testing. The average observation stay was
l2 to 18 hours, depending on time of arrival and whether
stress testing was done after the period of observation.
The ED observation unit charges range from $1,000 to
$2,000, whereas the hospital bill for inpatient evaluation
was in excess of $5,000. Most importantly, the "miss"
rate for sending home a patient with AMI was less than
0.1%.
Cardiogenic Shock
(2.
2.3.3)
Cardiogenic shock is a dramatically presenting entity

that, simply defined, is the loss of myocardial pump function resulting in poor perfusion to the rest of the body; it
affects virtually every organ system and is a leading
cause of inpatient mortality after AMI. Mortality rates
range from as low as 30%o with aggressive reperfusion
strategies to as high as 80% to90Yo if untreated. Although
treatment is urgent and optimally guided by hemodynamic monitoring, in the ED it is usually guided by physical examination and clinical parameters.
are treatment adjuncts that are universally available, such
as oxygen, aspirin, nitroglycerin, morphine, and betablockers, which are frequently overlooked in routine ED
management despite all of their advantages and benefits.
These should be considered in all patients without specific contraindications. In many patients, heparin has a
clear role. The benefit of magnesium has not yet clearly
been determined.
Etiology
Although several etiologies are possible, including cardiomyopathy, myocarditis, acute valvular failure, and left
atrial myxoma, the most prevalent is loss of critical left

ventricular muscle mass secondary to AMI. Nearly 70%
of these patients will have severe multivessel coronary
Cenorovescut-AR DTsoRDERS
disease. Mechanical complications of AMI such as right
ventricular infarction, acute mitral insufficiency, septal
rupture, ventricular aneurysm, or free wall rupture will
also lead to cardiogenic shock. Loss of approximately
40o/o of left ventricular muscle will result in cardiogenic
shock. Poor underlying left ventricular function with a
superimposed smaller infarction-induced loss of muscle

produce the same result. Medications that depress
ventricular function or affect compensatory mechanisms
will
may cause a tenuous situation to deteriorate into the

shock state.
Presentation
Patients
with cardiogenic shock are typically
older,
75
striction as well, impedes ventricular ejection, and often

negates any gains made in ventricular hemodynamics.
Tachycardia causes increased myocardial oxygen con-
sumption that worsens preexisting ischemia and

decreases the time during which left ventricular filling
occurs, resulting in decreased stroke volume, a major
determinant of cardiac output.
The net result is a vicious cycle: poor systolic function
leads to poor diastolic function and worsening systolic
function, which decreases coronary filling and oxygen
delivery in the face of accelerated myocardial oxygen
demand, leading to worsening ischemia. Further attempts
by the body to compensate perpetuate the cycle and ultimately result in multiple organ failure and death if left
untreated.
have sustained an acute anteriorAMI, have previous ECG
evidence of
AMI, or have underlying conditions such
as
congestive heart failure or diabetes. Clinically these

patients have the classic signs of shock: cyanosis, cool
and clammy skin, decreased mental status, weak pulses,
decreased urine output, anda systolic blood pressure less
than 90 mm Hg. Additionally, they manifest pulmonary

congestion resulting in tachypnea, dyspnea, accessory
muscle usage, wheezing, pink frothy sputum, and rales or
wheezes on lung auscultation. Cardiac examination may
reveal an abnormal apical impulse or thrill, an S: or S+
gallop or both, jugular venous distention (JVD), or
peripheral edema if underlying ventricular dysfunction is
chronic. The holosystolic murmur of acute mitral insufficiency or the harsh murnur of ventricular septal rupture
may be present. All cardiac sounds, however, may be
obscured by abnormalities of breathing or external noise
within the ED, making examination difficult.
The presentation is dramatic and diagnosis may be
obvious ifAMI is noted on the ECG in patients with these
symptoms. Otherwise, past history, medication usage,
and circumstances precipitating the collapse may be
essential in determining the etiology.
Pathophysiology
Although simply defined cardiogenic shock is a complex mixture of myocardial dysfunction and neurohormonally mediated attempts to correct the situation. Ischemia and/or loss of contractility leads to poor cardiac
output and activation of the B-adrenergic and reninangiotension systems to improve vital organ perfusion.
Tachycardia, increased contractility, vasoconstriction,
and salt and water retention result. During ischemia the
left ventricle has difficulty compensating and malfunctions further.
Vasoconstriction augments preload" which initially

improves ventricular function, but may shift ventricular
function detrimentally on the Starling curve and worsen
pulmonary congestion. Afterload increases with vasocon-
Diagnosis
Diagnosis can be easy with shock, pulmonary edema,
and the classic ECG findings of AMI. Hemodynamic

monitoring is the most precise method of diagnosing cardiogenic shock and guiding therapy, but the emergency
physician usually does not have this luxury in the ED and
must depend on history, physical examination, and a few
ancillary studies to initiate therapy.
If
Swan-Ganz moni-
toring capability is available, filling pressures and cardiac

output can be measured. In cardiogenic shock, pulmonary capillary wedge pressure or left ventricular filling pressure is elevated, above 18 mm Hg, and often
above 25 mm Hg, and cardiac index is depressed below
2.2 Llminlm2. Major entities to be considered in the
patient with chest pain and hypotension include pericardial tamponade, massive pulmonary embolism, aortic
catastrophes, papillary muscle rupture, ventricular rupture, and right ventricular infarction. History may reveal
previous ischemic heart disease, known aortic aneurysm,
or risk factors for pulmonary embolism. Physical examination will reveal all or some of the previously described
findings with cardiogenic shock. Pericardial tamponade
may have the classic findings of Beck's triad (JVD,
decreased blood pressure, and muffled heart sounds) if
fluid accumulation is rapid. More commonly the fluid
accumulates less rapidly and patients appear agitated and
tachypneic with hypotension. The lungs will remain clear
or much less congested than expected. Chest radiograph
classically reveals a "water bottle" heart shadow in

chronic tamponade and absence of pulmonary vascular
congestion, but may show cardiomegaly or a normal or
small heart shadow if acute. Definitive diagnosis is made
echocardiographically with evidence of a large pericardial effusion, right atrial compression, and right ventricular diastolic collapse.
Aortic catastrophes must be immediately
suspected
when a history ofhypertension or connective tissue disease accompanies tearing chest pain radiating to the back.
76 /
EurncrNcv MnlrcrNr: Tur Conn CunrucuLUM
A pulsatile abdominal mass, loss of pulses, unequal blood

pressures, or acute neurologic deficits may be noted.
Physical examination will reveal clear lungs unless heart
failure is preexisting. Chest radiograph may reveal mediastinal widening, loss of the aortic knob, and other findings of dissection (displacement of the trachea or esoph-
or aortic calcification).
Definitive diagnosis is made by computed tomography
(CT). transesophageal echocardiography (TEE), or aortography. TEE is portable, while CT will delineate other
mediastinal pathology and aortography identifies the
arterial anatomy. All are very sensitive and specific and
the decision may rest with availability and the consulagus, apical pleural cap
tants' preference.
Massive pulmonary embolism causes acute hypoxemia
and affects the right ventricle directly. The left ventricle
receives poor filling pressure with which to produce cardiac output. Physical examination reveals hypotension
and dypsnea with minimal pulmonary findings unless
congestive heart failure is preexisting. ECG classically
shows the Sr Q: T: pattern, but tachycardia with nonspecific ST:segment or T:wave changes is most common.
Chest radiograph is without pulmonary congestion and
may demonstrate the uncommon findings of pulmonary
infarction (Hampton's hump) or vessel cutoff with

enlargement of the pulmonary outflow tract (Westermark's sign).
Right ventricular infarction usually presents with chest
pain and ECG evidence of inferior AMI. Hypotension
may occur as the right ventricle cannot supply the needed
left ventricular filling pressure. Nitroglycerin used by the
patient may exacerbate the hypotension and is a clue to
the presence of right ventricular infarction. Physical
examination may reveal JVD, hepatomegaly, hepatojugular reflux, and clear lungs on auscultation. Performance
of the VrR and V+R leads in conjunction with the l2-lead
ECG is essential for making the diagnosis. Complete
right-sided ECG may be beneficial.
Intervention
Evaluation and treatment must occur simultaneously
and with a sense of urgency. The patient will continue to
deteriorate unless specific interventions occur. Oxygenation with 100% oxygen is crucial and delivered by either
endotracheal intubation and positive end-expiratory pressure or continuous positive airway pressure (PAP) techniques. Bi-PAP is being studied as well. Endotracheal
intubation and 100% oxygen delivery alone may be adequate. Should positive end-expiratory pressure be used,
begin with 2.5 to 5.0 cm HzO and reassess the patient
carefully. The increased intrathoracic pressure may
impede venous return and decrease preload resulting in
decreased cardiac output and negate any benefit. Restoration of coronary perfusion with myocardial systolic and
diastolic function is the ultimate goal. Contractility must

be improved, and preload and afterload should be judiciously reduced without worsening the imbalance in myo-
cardial oxygen delivery and utilization. If the lungs are

clear, a fluid bolus should be administered and the patient
examined frequently for response and to detect pulmonary edema, while noncardiogenic or right ventricular
causes are sought. Findings of rales or pulmonary edema
indicate the need for inotropic support and discontinuation of the fluid bolus.
Dobutamine infusion beginning at 2.0 to 2.5

mcglkg/min and rapidly advancing by 2- to 3mcglkg/min increments every 5 to 10 minutes guided by
blood pressure and peripheral perfusion is indicated for
patients with systolic blood pressure greater than 80 to 90
mm Hg. Favorable response is usually noted in the 7.5- to
1S-mcg/kg/min range. Although dobutamine
is
not
known to produce tachycardia, care must be taken not to

induce or worsen an existing tachycardia.
Dopamine may be used alone or in conjunction with
dobutamine for patients with systolic blood pressure less
than 80 to 90 mm Hg. Dopamine produces positive
inotropic effects in the 2.5- to 5.0-mcg/kg/min range that
are dose dependent. However, in doses approaching 10
mcg/kg/min the Br effects intensify, causing tachycardia
and cr-mediated vasoconstriction that will increase both
preload and afterload, negating improvement. In doses
approaching 20 mcglkglmin, dopamine is similar to norepinephrine and has only deleterious vasoconstrictive
effects in the setting of cariogenic shock. The combination of dopamine and dobutamine is recommended at
lower doses (both in the 5.0- to 7.5-mcg,&g/min range) to
provide the beneficial effects of both, while minimizing
their disadvantages.
If pulmonary congestion is improved and if systolic
blood pressure has improved to approximately 100 mm
Hg, then vasodilator therapy can be very carefully begun
to improve pulmonary congestion by reducing preload
and afterload. Nitroprusside and/or nitroglycerin, or usually the combination, are most commonly utilized. Hemodynamic central monitoring should be strongly considered
before instituting these agents as hypotension, decreased
coronary blood flow, and worsening ischemia can be precipitated. Ideally, this therapy will reduce both preload
and afterload" causing improved diastolic filling,

increased ejection fraction, and a shift to a more favorable
hemodynamic state.. Nitroglycerin is begun at 5 to l0

mcg/min and titrated upward in 5- to 10-mcg/min increments at 5- to l0-minute intervals. Response is usually
noted in the 10- to 50-mcg/min range. Nitroprusside is
begun in the 0.5-mcg/kg/min range and carefully titrated
upward in 1.0- to 2.0-mcg/kg/min increments at 5- to 10minute intervals. This author has noted that response can
be expected in the 2.5- to 7.5-mcg/kg/min range. Nitroprusside does have the potential for decreasing coronary
flow by inducing widespread arterial dilatation at low
Cenorove.scur.AR DTsoRDERS
77
doses. It is emphasized that hemodynamic monitoring

should be instituted prior to use of these agents.
Severe shock states unresponsive to these pharmacologic measures will require consultation for placement of
a ventricular assist device to temporize until a revascular-
necrotic tissue, but eventually its walls become more
izalion procedure can occur. Survival has not been
ward bulging
improved with these devices unless revascularization is
aneurysm may cause malalignment of papillary muscles

with consequential mitral regurgitation and congestive
heart failure (CHF). Most patients are minimally symptomatic. Patients may develop angina pectoris, progressive
accomplished.
Aggressive reperfusion therapy in eligible candidates
is indicated in the setting of AMI with shock
because
ischemia is the presumed cause of the acute left ventricular dysfunction. Both the GISSI 2 and the Global Use
of Strategies to Open Occluded Coronary Arteries
(GUSTO-1) trials noted a lower mortality rate (55-650/o)
with streptokinase than with rt-PA (63-78%) in those
patients presenting in cardiogenic shock. PTCA in small
and uncontrolled studies has decreased mortality to
30oh in this setting due to the overall improvement in
reperfusion, but carries the drawback of requiring cardiothoracic surgical capability on standby. If the emergency physician has angiography and surgical support
readily available, then emergent PTCA is recommended; if not, then thrombolytic agents should be utilized and arrangements made for expeditious transport
to a facility where more aggressive reperfusion can
occur or continue.
densely fibrotic and may even calcify.

A ventricular aneurysm causes the heart to have a
mechanical disadvantage. Normal contractile energy is
expended and virtually wasted by passive systolic out-
of the aneurysm.
Additionally,
the
congestive heart failure, cardiomegaly, and predisposi-
tion to recurrent ventricular arrhythmias and sudden

death. A high frequency of associated mural thrombosis
(15% to 77o/o at necropsy or surgery) is also noted, but
surprisingly the frequency of clinically recognized systemic embolism is low (2%o to 5oh). Unlike pseudoaneurysm, rupture of a true aneurysm is rare.
Diagnosis
Diagnosis should be considered when early or late

severe hearl failure develops, a noted bulge is noted on
the left heart border on a chest x-ray (CXR), and ST:-seg-
ment elevation persists beyond 2 weeks following AMI.

Additionally, embolic events or a diffuse or dyskinetic
left ventricular apical impulse may help in
diagnosis.
Clinical suspicion can be confirmed by two-dimensional

echocardiography (2-DE) or a radionuclide ventriculogram to differentiate a true aneurysm from a pseudoa-
Disposition
Patients should be transferred expeditiously to critical
invasive hemodynamic monitoring can
care units
"l'here
optimally guide pharmacologic therapy, appropriate consultation for placement of a ventricular assist device can
be made, and a revascularization procedure can be done.
neurysm.
Ventricu lar Aneurysm (2.
or an aneurysm producing refractory or recurrent lifethreatening arrhythmias. This includes ventricular

aneurysmectomy, revascularization, and mitral valve
repair or replacement if the papillary muscles are
involved. No controlled studies, however, have documented the efficacy of any treatment to prevent embolic
2. 3.
4)
Infarct expansion represents fixed, permanent, regional
thinning and dilatation of the infarct zone and is caused

predominantly by the slipping of necrotic myofibrils.
This process begins within hours of a transmural infarc-
tion and progresses over the first week. Infarct expansion

further predisposes to development of a true ventricular
aneurysm, pseudoaneurysm, or free-wall rupture.
True Ventricular Aneury sm
Treatment
Surgical removal ofthe aneurysm and or anticoagulant
therapy is indicated when there is refractory heart failure
events.
Pseudoaneurysm
pseudoaneurysm is rare. It is a complication of

AMI resulting most often from rupture of the
ventricle. Nearly 75o/o of pseudoaneurysm cases
transmural
Chronic left ventricular aneurysm occurs in l0% to

38% of patients who survive acute transmural AMI. Most
often it is noted with anterior AMI involving the left ventricular apex, the anterolateral wall, and the septum. A
true ventricular aneurysm is a circumscribed, noncontractile outpouching of necrotic tissue that stretches, thins,
and expands. In the early stages, the aneurysm contains
left
of cardiac
surgery, chest trauma, or bacterial endocarditis. The wall
reported have resulted from complications
of the
pseudoaneurysm, unlike the true aneurysm, is
composed of pericardial adhesions and is devoid of myocardial tissue and coronary arteries. The rupture and
resulting hemopericardium is contained by circumferen-
78 /
EnsncrNcy MsucrNn,: THE Conn CunrucuLUM
tial
adhesions between the pericardium and the epicardium. Although the precise incidence is not known, a
retrospective review by Catherwood et al. detected a
pseudoaneurysm in 0.5% of patients referred for cardiac
catheterization.
Diagnosis
Dalen JE, Gore JM, Braunwald E, et al. Six- and twelve-month follow-up
of the phase I Thrombolysis in Myocardial Infarction (TIMI) tnal. Am J
C ard io
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79-l 85.
Dole WP, O'Rourke RA. Pathophysiology and management of cardiogenic

shock. Curr Probl Cardiol 1983;8:1-72.
Dykewicz MS, McGrath KG, Davison R. Identification of patients at risk
for anaphylaxis due to streptokinase. Arch Intern Med 1986;146:305.
Gibler WB, Gibler CD, Weinshenker E, et al. Myoglobin as an early indicator ofacute myocardial infarction. Ann Emerg Med 1987;16:851-856.
GISSI-2 (Gruppo Italiano per lo Studio della Soprawivenza nel ,Infarto
Miocardico) Effectiveness of intravenous thrombolytic treatment in
Clinically,
pseudoaneurysm may be silent or manifest

a true aneurysm. Patients with a pseudoaa
similarly to
neurysm may have worsening congestive heart failure,
recurrent ventricular arrhythmias, cardiomegaly, an
abnormal bulge on the cardiac border, systolic and dias-
tolic murmurs, and persistent elevation of the ST segment

on the ECG. Howeveq a pseudoaneurysm, unlike a true
aneurysm, is susceptible to free rupture in approximately
one-third of these patients with an invariably fatal outcome. Free wall rupture is estimated to occur in 1.5%o to
8% of transmural AML Rupture usually occurs within 5
days afterAMI. Conformation of clinical suspicion can be
made by 2-DE, radionuclide ventriculography, or invasive
cardiac catheterization and left ventricular angiography.
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treatment with verapamil for suspected acute myocardial infarction. lrll
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Bakker AJ, Koelemay MJ, Gorgels JP, et al. Failure of new biochemical
markers to exclude acute myocardial infarction at admission. Lancet
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Beck LS. Two cardiac compression triads. JAMA 19351'104:714-716.
Braunwald E. Myocardial reperfusion, limitation of infarct size, reduction
of left ventricular dysfunction, and improved survival: should the paradigm be expanded? Circulatiott 1989;79:441.
Brett JH. Late assessment of thrombolyic efficacy with alteplase (rt-PA)
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the Thrombolysis in Myocardial Infarction (TIMD 5 Trial. J Am Cotl
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1980:62:294-303.
Cercek B, Shah PK. Complicated acute myocardial infarction: heart failure,
shock, mechanical complications Cardiol Clin l99l;9:4
acute myocardial infarction. Lancet 1986;I :397
GISSI-2. Long-term effects ofintravenous thrombolysis in acute myocardial infarction: final report ofthe GISSI stttdy. Lancet 1987;2:871.
GISSI-2. A factorial randomized trial ofalteplase versus striptokinase and
heparin versus no heparin among 12,490 patients with acute myocardial
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GISSI-2 and International Study Group. Six-month survival in 20,891
patients with acute myocardial infarction randomized between alteplase
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Gitter MJ, Goldsmith SR, Dunbar DN, Sharkey SW. Cocaine and chest
pain: clinical features and outcome ofpatients hospitalized to rule out
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ENDOCARDTTTS (2.2.4)
Etiology
While bacteria are, by far, the most common infectious
in endocarditis it must be remembered that any
organism can be the causative agent. Cases ofendocardiagent
/79
TABLE 2-11. Common pathogens in

endocarditis
Native valves
Staphylococcus aureus
Staphy lococc u s epide
Streptococcus
r m i d is
vi ridans
Enterococci
Group A p-hemolytic streptococcus
Streptococcus bovis
lV drug abusers/immunocompromised
Pseuomonas
Serratia
Haemophilus
Gram-negative bacteria
Fungi
Prosthetic valves
Staphyl ococcu s e p id e rm id i s
Streptococcus vi ridans
Fungi (Candida and Aspergiilis)
Gram-negative bacteria
Group D streptococcus
tis have been reported secondary to viruses, fungi, and

rickettsiae. With HIV and the rise in immunocompromised patients, these atypical organisms are becoming
more prevalent. A list of the more common offending
organisms is shown in Table 2-11.
Presentation
Endocarditis has a bimodal age distribution reflecting
valve replacement and the increase in AIDS and IV drug
usage. The older population, over age 50, is more likely
to have endocarditis after valve replacement or from
undiagnosed valve disease existing in an asymptomatic
phase. A younger population is more affected by congenital defects, IV drug abuse, and HIV
The incidence of endocarditis with a prosthetic valve is
0.5oh to 4Yo a year. The overall recurrence rate of endocarditis is 2.5%o to l7o/o but IV drug abusers (IVDAs)
have a 4lo/orecwrence rate.
The presenting symptoms of endocarditis are frustrat-
ingly vague and nonspecific. Intermittent fever (85%),

malaise (95%), dyspnea, chest pain, cough, headaches,
and anorexia are commonly reported. Rarely, there will
be neurologic complaints and/or focal CNS deficits from
emboli. In more severe cases the patient will present with
rigors, fever, chills, petechiae, hypotension, or a sepsis
syndrome. Symptoms start subclinically and progress in
severity. The mean time of presentation from onset of
symptoms is 20 days.
Endocarditis is a diagnosis easily missed if symptoms
are mild and it must always be kept in the differential.
Patients with any risk factors or a murmur presenting
80 /
ErurncpNcy MnorcrNn: TsE Conr CunrucuLUM
with fever must have this diagnosis considered and the
etations or emboli. Empiric treatment without a known
appropriate consultation or referral arranged.
organism is usually required in such cases.

ECG is usually normal unless there is extensive myocardial damage, such as from an abscess. Chest radiography is also usually noncontributory except in cases of
right heart endocarditis. Septic emboli from right heart
Pathophysiology
The pathophysiology of endocarditis is fairly simple.
People are bacteremic for short periods of time each day.
Simple acts such as brushing teeth or chewing hard
candy, as well as invasive medical and dental procedures,
introduces bacteria into the circulation. In a normal host
these bacteremic episodes are of no concern. In patients
with damaged heart valves from trauma, inflammation,
high-flow lesions, orprevious cases ofendocarditis, normal laminar flow is disrupted, allowing bacterial deposition on the irregular valves. There they flourish, worsening the valvular damage and producing the hallmark of
endocarditis, the vegetation.
Damaged valves act as foreign bodies allowing bacte-
ria
increased adherence and physical shielding from

blood flow that protect them from normal body immune
defenses. Bacteria are shed into the bloodstream, causing
the intermittent fever, other general systemic symptoms,
and characteristic physical findings and ancillary results.
Valvular damage may be severe, sometimes causing valve
rupture and acute insufficiency. Vegetations can interfere
with normal valve function, producing either stenosis or
insufficiency, or may dislodge, becoming systemic or
CNS emboli. Abscess formation at the site of vegetation
or distal emboli is common.
Diagnosis
The diagnosis of endocarditis is usually a coupling
of
clinical and laboratory evidence. Physical signs ofendocarditis are helpful when present but are absent in many
patients, especially early in the disease course. A new
regurgitant murnur is often hear{ but in IVDA patients
this is often absent or very minimal and easily overlooked. More than 50% of the patients will have vascular
lesions of septic emboli including petechiae, splinter
hemorrhages, Osler nodes, or Janeway lesions. Roth
spots, whitish spots on fundoscopic examination, thought
to be from microemboli, are present in less than l0% of
patients.
Laboratory evidence
is
largely nonspecific. Most
patients will have a leukocytosis with a shift and an elevated erythrocyte sedimentation rate. Microscopic hema-
turia is common (40 to 50%), from renal microemboli,

and is an important finding in a patient at risk.
The most productive test is the blood culture. In bacte-
rial endocarditis, properly obtained blood cultures are

almost always (80 to 90%) positive and will help guide
therapy. In cases of atypical bacterial or fungal endocarditis, blood cultures are rarely positive and the diagnosis is sometimes made on histologic examination of veg-
lesions, especially
in IVDA with S. aureus
infections,
will
appear as multiple pulmonary abscesses or areas of

pneumonia.
Echocardiography is useful in diagnosis when vegetations can be visualized but a negative examination does
not rule out endocarditis. Vegetations may be too small,
be missed on exam, or not formed yet on the grossly

infected and inflamed valve.
Intervention/Disposition
Appropriate treatment
is
depends
on the clinical
appearance of the patient. In the early stages, with mild

symptoms or fever alone, the main goal should be making the diagnosis so appropriate antibiotics can be insti-
tuted. Minimally, three blood cultures from different

sites over the course of I hour should be drawn prior to
administration of any antibiotic. In severe cases with
hemodynamic instability, antibiotics should not be withheld prior to blood cultures. For antibiotic coverage see
Table 2-12.
Indications for surgery in an active case ofendocarditis include vegetations greater than 10 mm in size, CHF,
infection uncontrolled by parenteral antibiotics, fungal
infections, abscess formation, or recurrent emboli.
Endocarditis in patients with prosthetic valves should
always have early consultation with a cardiothoracic surgeon.
Obviously, the emergency physician will not have the

results of blood cultures to guide therapy. Patients with
prosthetic valves or history of IV drug use who present
with fever should be admitted and treated for presumed
endocarditis until the blood cultures prove otherwise. All
TABLE 2-12. lnitialtreatment of endocarditis

Native valve
Penicillin G 20 million units lV (continuous or divided
q4h)
or
Ampicillin 3.0 g lV q4h plus nafcillin (oxacillin) 2.0 g lV
plus gentamicin 1.0 mg/kg q8h lM or lV
lf penicillin allergic
Vancomycin 1.0 g lV q12h plus gentamicin 1.0 mg/kg
q8h lM of lV
Prosthetic valves/lV drug abuse
Nafcillin 2.0 g lV q4h plus gentamicin 1.0 mg/kg lV q8h
plus rifampin 600 mg po qd or
Vancomycin 1 .0 g lV q12h plus gentamicin 1 .0 mg/kg
q8h plus rifampicin 600 mg po qd
Ce-mrovnscur-AR DTsoRDERS
TABLE 2-13. Antibiotic prophylaxis for endocarditis
Dental/upper respiratory procedures
po dosing
Amoxicillin 3.0 g t hr prior to procedure and 1.5 g
6 hr post or
EES
800 mg 2 hr prior to procedure and
400 mg 6 hr post or
Erythromycin 1.0 g 2 hr prior to procedure and 500 mg
6 hr post or
Clindamycin 300 mg t hr prior to procedure and
150 mg 6 hr post
lV dosing
Ampicillin 2.0 g lV/lM 30 min prior to procedure and
1.0 g 6 hr post or
Clindamycin 300 mg lV 30 min prior to procedure and
150 mg lV/po 6 hr post or
Vancomycin 1.0 g lV over t hr, starting t hr prior to
procedure; no repeat dose needed
Gl/GU procedures
po dosing
Amoxicillin 3.0 g t hr prior to procedure and
l.5gGhrpost
lV dosing
Ampicillin 2.0 g lV + gentamicin 1.5 mg/kg lV (not
exceeding 80 mg) 30 min prior to procedure, then
amoxicillin 1.5 g po 6 hr post or repeat lV dose 8 hr
after first dose or
Vancomycin 1.0 g lV over t hr, starting t hr prior to
procedure + gentamicin 1.5 mg/kg (not exceeding
80 mg) t hr prior; may repeat gentamicin 8 hr post
Pediatric dosing
As per above regimens with total dose not to exceed
adult dose; repeat doses half initial
Ampicillin/amoxicillin 50 mg/kg
EES/Erythromycin 20 mg/kg
10 mg/kg
Clindamycin
2.0 mg/kg
Gentamicin
20 mg/kg
Vancomycin
other patients need to be evaluated on an individual basis,

weighing the predisposing risk, clinical symptoms, and
ability for follow-up should cultures be positive.
Patients at risk should also be treated with antibiotics
prior to any dental or medical interventions likely to
cause bacteremia. Many of these procedures are com-
monly done in the ED, and antibiotic prophylaxis may

easily be overlooked or forgotten. See Table 2-13 for
commonly used doses.
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/ Bl
VALVULAR HEART DISEASE (2.2.5)

Valvular heart disease may produce virtually all of
the symptoms of congestive heart failure and myocardial ischemia. Furthermore, valvular dysfunction can
result from other cardiac disorders, and it should be
considered more frequently than it is. The emergency
physician (EP) can play a critical role in its prevention,
need of early intervention and treatment, prevention of
complications, and acute management of its end-stage
pathology.
Rheumatic fever is a major cause of valvular pathology. Prevention with appropriate evaluation and treatment of streptococcal pharyngitis is important for the EP
to consider.
An important task of the EP is to recognize valvular
pathology early, as most valvular lesions have an asymptomatic phase lasting several decades. With the current
capability of echocardiography to visualize valves and
monitor flow and pressures, coupled with advanced surgical valve replacement or repair techniques, early recognition is critical. Patients can be identified, followe4 and
referred for surgical intervention before irreversible cardiac damage has occurred. Young patients in the asymptomatic phase often have no primary physician and present to the ED for unrelated reasons. The EP should
ideally listen for muffnurs and other signs of asymptomatic valve pathology on all patients so that appropriate
referral can be made.
The EP should also be aware of antibiotic prophylaxis
in patients with valvular lesions (see Endocarditis,
above). Many procedures commonly done in the ED
will cause a transient bacteremia and place the patient
with altered valve morphology at risk for endocarditis.
Patients with dental injuries or about to undergo abscess
incision and drainage (I&D), anoscopy, or other invasive procedures should be questioned about past history
of heart problems as well as screened for murmurs.
Appropriate prophylactic antibiotics should be given.

Timing of administration is crucial for adequate coverage.
When the valvular pathology remains unrecognized

until it causes systemic symptoms or collapse, the EP's
duty is to resuscitate, stabilize, and recognize valvular
etiology. End-stage valvular pathology mimics or coexists with many other disease processes; unlike many
processes, valvular changes can be treated with surgery.
The EP can significantly reduce mortality with appropriate intervention and referral.
Valvular lesions vary widely in presentation. They

may be stenotic and obstruct the outflow of blood; they
may be incompetent and allow retrograde regurgitant
flow; or they may be a combination of both. Multiple
valves may be affected. Lesions can cause a slow, gradual increase in symptoms over many years; or they may
decompensate, acutely causing a previously "healthy"
82 /
ElrpncrNcv MrrrcrNn: Tnn Conn CunrucuLUM
person to go into cardiogenic shock. While echocardiography and cardiac catheterization are the gold standards in diagnosis and evaluation ofvalvular pathology,
the EP must rely heavily on physical diagnosis. Most

lesions can be diagnosed accurately based solely upon
history and physical examination. Ancillary tests such
as ECG and CXR help in the diagnosis but are not
required.
with age, and rarely causes any symptomatic pathology

requiring intervention.
Pathophysiology
In acute AI, the left ventricular end-diastolic pressure

(LVEDP) increases dramatically as blood rushes backward through the incompetent aortic valve. These elevated pressures are transmitted backward, causing rapid
Aortic Insufficiency
(2.2. 5. 1)
Etiology
pulmonary hypertension and edema. The effective cardiac output (CO) drops precipitously and tachycardia
results. As end-organ perfusion drops, the body compensates
The top three causes of aortic insufficiency (AI) are

endocarditis (active or healed), rheumatic heart disease,
and congenital. Most cases (80%) are chronic, resulting
from the slow destruction of the leaflets or dilatation of
the aortic annular ring. Both rheumatic heart disease and
congenital unicuspid and bicuspid valves cause progressive scarring of the leaflets, and mixed stenotic and regurgitant pathology. Diseases that dilate the aortic wall
include Marfan syndrome, ankylosing spondylitis,
Reiter's syndrome, tertiary syphilis, rheumatoid arthritis,
and cystic medial necrosis of the aorta.
Acute AI occurs with the sudden failure of valve
leaflets, usually from infective endocarditis, rheumatic
fever, prosthetic valve dysfunction, trauma, and, rarely,
idiopathic spontaneous rupture. Sudden dilation of the
aortic root from aortic dissection will also cause acute AI.
Presentqtion
Acute
AI has fairly dramatic presenting signs and
symptoms. Patients will have sudden dyspnea, tachycardia, and chest pain, as well as signs ofdecreased cardiac
output such as hypotension, diaphoresis, pale extremities,
peripheral cyanosis, and confusion. There may be associated information depending on the etiology of the acute
valve failure: fever in endocarditis, recent blunt trauma,
chest pain from aortic dissection, or past medical and
family history.
Chronic
AI
has a more insidious presentation. People
are usually asymptomatic until their third or fourth

decades of life. The first symptoms are exertional fatigue,
followed by exertional dyspnea, orthopnea, and paroxysmal nocturnal dyspnea. Finally they may present with
pulmonary edema, angina, and evidence of right-sided
heart failure, ascites, edema, and hepatosplenomegaly.
'Atypical" symptoms are common, including chest wall
pain from forceful left ventricular contractions, palpitations, postural dizziness, neck or abdominal pain from
excessive stretching of the carotids and aorta, and
increased stroke volume.
Mild AI is commonly seen in all patients over 80 years

old. This is from the widening of the aortic annular ring
with vasoconstriction, which increases
afterload
and worsens regurgitation. Cardiogenic shock from acute

AI is very refractory to treatment, and in-hospital mortal-
ity approaches 50%.

Chronic AI has a much more indolent, compensated
course, but the basic pathophysiology is essentially the
same. Increasing LVEDP and end-diastolic volume cause
increased pulmonary pressure and a drop in CO. The ven-
tricle compensates by first dilating then by hypertrophy.

The increased stroke volume (SV) maintains effective
CO temporarily, but systolic blood pressure
will
increase
to reflect increased blood flow while diastolic pressure

will fall, widening the pulse pressure. As pulmonary
hypertension and edema worsen, patients
the symptoms of
will
experience
dyspnea and fatigue. Ventricular
arrhythmias, angina, and sudden death are common
as
the noncompliant hypertrophied left ventricle compresses
the coronary arteries and small vessels with resultant

ischemia. Chronic AI is progressive. The regurgitation
will gradually increase over many years until the compensation mechanisms fail. After symptoms appear the 5year survival drops to 75o/o. Most patients die within 5
years after onset of angina and within 2 years of congestive heart failure.
Complications of AI include endocarditis and aortic

root rupture. The floppy, scarred valve predisposes to
bacterial deposition and endocarditis. Antibiotic prophylaxis is recommended for all patients with AI.
Those patients with disease affecting the aortic root
[Marfan, systemic lupus erythematosus (SLE),
syphilis] are at risk for sudden root rupture, turning
chronic AI into acute AI, which is often refractory to all
interventions.
Diagnosis
Patients with acute AI may remain compensated for
some time or present in extremis. They will complain of
significant dyspnea and apprehension. Pulmonary examination reveals inspiratory rales. A normal apical impulse
can be palpated. The blood pressure will be normal or
decreased and there will not be peripheral pulse signs
common in chronic AI.
Cenorovescut-A,R DTsoRDERS
83
A murmur may be difficult to hear due to tachypnea,

tachycardia, and pulmonary rates. There will be a diminished Sr as the mitral valve is closed before systole by the
retrograde blood in the ventricle. 52 ma] be absent with
the destroyed aortic valve, but an S: is common. The
murrnur is an early phase, medium pitch diastolic murmur that ends quickly as pressures equ'alize. It is best
treatment are essential for good outcome. Treatment is

geared toward improving forward cardiac output while
heard at the left sternal border in the third/fourth intercostal spaces (ICS).
ECG findings in acuteAl are minimal, with sinus tachycardia and nonspecific S-T changes predominating. Conduction disturbances can be seen in cases of endocarditis.
Chest radiographs are more dramatic, commonly showing
pulmonary edema, normal heart size, and possibly a
dilated aortic root, wide mediastinum, or aortic dissection.
Of special note for the EP: IV drug abusers (IVDAs)
who present with sinus tachycardia, pulmonary edema,
and a normal heart size may have acute AI secondary to
endocarditis. These patients may have a minimal or
absent heart murmur.
Chronic AI has a number of both peripheral and cardiac findings. There is a widened pulse pressure. The left
ventricle is usually hyperkinetic with a left chest heave
and an inferolateral strong apical impulse secondary to
left ventricular hypertrophy (LVH). As the condition
worsens, increasing respiratory distress from pulmonary
edema and right-sided heart failure with ascites, edema,
imize therapy. Vasodilators (nitroglycerin and nitroprusside) reduce afterload and increase effective CO, and, in
cases of ischemia, help LV function. Dobutamine is useful to increase CO but may worsen ischemia. Vasoconstricting agents (dopamine and norepinephrine) should
not be used to maintain blood pressure as they will
WD are seen.
A large number of findings seen with chronic AI have
been described: Corrigan's (water-hammer) pulse-a
pounding, rapid rise and fall of the pulse; Duroziez's
sign-a singsong bruit over the femoral arteries;
Mueller's sign-pulsations of the ulula; DeMusset's
sign-head bobbing with each systole; Quincke's sign-
lar arrhythmias and need close cardiac monitoring. In

acutely decompensated chronic AI, treat as above plus
vasodilators and dobutamine as in acute AI. This condition has a worse prognosis than acute AL
and
pulsations in the capillary nail beds; and prominent pulsations in the retinal arteries.
The murmur of chronic AI is classically a decrescendo,
high-pitched, blowing murmur best heard at the left sternal border in the third/fourth ICS. A diastolic thrill may
be palpable at the left sternal border. 31 is usually normal,
while Sz is normal or slightly decreased. A mid-diastolic
to early systolic munnur caused by regurgitant blood
forcing the anterior mitral leaflet into blood flowing from
the atria to the ventricle (Austin-Flint murmur) may also
be heard.
ECG findings usually reflect the chronic changes in

the left ventricle. Signs of LVH with strain pattern are
conrmon and l0o/o of patients with chronic AI will have a
reducing pulmonary edema. Oxygen, diuretics, and

vasodilators are the mainstays of initial treatment. Invasive monitoring should be used early because overdiuresis is common and will worsen the condition. Filling
pressures and cardiac parameters should be used to max-
worsen regurgitation. Intraaortic balloon pumps will also

worsen insufficiency. Other treatments should be geared
toward the underlying cause, e.g., antibiotics for endocarditis. Immediate cardiothoracic surgical consultation
is required.
In chronic AI, treatment is geared toward the severity

of symptoms. Mild AI can be treated with fluid and salt
restriction, mild diuresis, and follow-up on an outpatient
basis. Patients should also be instructed about prophylactic antibiotic use for invasive procedures. Ifpatients show
symptoms such as palpitations, dyspnea, or angina, they
needed to be admitted for treatment with nitrates and
diuretics, but more importantly
to be evaluated
for
surgery. These patients are at increased risk for ventricu-
Aortic Stenosis (See 2.2.5,1)

Etiology
Aortic stenosis (AS) accounts for 25o/o of all vahulopathies and falls into three main types. Flow may be
impeded by subaortic, valvular, or aortic obstruction.
Subaortic stenosis is usually congenital, consisting of
either tunnel, discrete, or hypertrophic pathology. Tirnnel
defects are least common and consist of a congenitally
narrow hypoplastic aortic outflow tract and annular ring.
Discrete lesions are 160/o of all subaortic lesions and are
a congenital membranous obstruction partially blocking
the outflow tract. Hypertrophic stenosis is an autosomal
dominant trait with incomplete penetrance, causing an
idiopathic thickening of the left ventricle and outflow
left bundle branch block. Chest radiographs usually show

an enlarged heart and the lung fields will range from
normal appearance to frank pulmonary edema.
tract. It may also cause abnormal placement of the mitral
Int erv en t i on /D i sp o s i t i o n
unicuspid aortic valve that predisposes toward later

stenotic pathology. Less common causes of AS include
In acute AI, the diagnosis may be difficult due to the

patient's critical nature; however, rapid recognition and
rheumatic heart disease, end-stage renal disease, Paget's

disease, rheumatoid arthritis, and idiopathic sclerosis.
valve.
Valvular stenosis is also most commonly congenital.

Approximately 2% of the population has a bicuspid or
84 /
EurncnNcy MtorcrNn: Tnr Conn CunrucuLUM
Postvah.nlar obstruction is rare, less than 5%o of al7 aortic pathology. A congenital web in the proximal aorta, a
discrete thickening of the proximal aortic intima, or a
general aortic hypoplasia can be present. Usually these
defects are associated with other cardiac anomalies and
are noted at a very early age.
Presentation
Symptomatic presentation occurs in a bimodal distribution with median ages of 10 and 48 years. Younger
patients usually suffer from undiagnosed congenital
lesions, mostly subaortic, and present with symptoms of
dyspnea, exertional chest pain/discomfort, syncope/near-
syncopal events, and sudden death. Symptoms will

worsen as the child grows and places more demand on a
previously asymptomatic lesion.
Older patients usually have a valr,ular stenosis and present with symptoms of dyspnea, chest pain/angina, syncope, and sudden death. One of the most common presentations in an older adult is new or worsening angina.
Without treatment, symptoms are progressive with exertional fatigue, then dyspnea, and finally CHE.
AS predisposes toward thrombus formation and resulting emboli. Presentation with symptoms of systemic or
CNS emboli should include AS as a precipitating cause in
the differential.
Pathophysiology
The basic pathology ofaortic stenosis is obstruction
of
blood flow to the systemic circulation. To maintain cardiac output, the left ventricle compensates by dilatation
and hypertrophy. The increased LV pressures are transmitted retrograde to the pulmonary vasculature causing
hypertension, congestion, and edema. Noncompliant,
hypertrophic myocardium requires more oxygen while
simultaneously decreasing its supply by compression of
the coronary arteries; subendocardial ischemia, angina,
and ventricular arrhythmias are common. During periods
of increased demand (exercise, infection, fever) the CO
cannot be maintained and syncope is common as blood
flow is directed away from the brain. Sudden decompensation may occur if the patient suffers new atrial fibrillation (AF) as a maximally hypertrophied left ventricle may
require the atrial kick to maintain cardiac output.
The aortic outflow tract is normally 3 to 4 cm2, but
lesions are not symptomatic until the opening is less than
1.0 to 1.5 cm2. Surgical repair is required when the cross
section is below 0.8 cm2 because of the high risk of
arrhythmias and sudden death. An aortic valve with less
than 0.5 cm2 is termed critical aortic stenosis. Aortic
stenosis is progressive, with 50oh to 70%o of patients
developing angina 2 yearc after presenting with exer-
tional fatigue. Exertional syncope occurs 3 to 4 years
after onset of angina. Left ventricular failure is seen

within I to 2 years after development of angina and ventricular arrhythmias are very common, with 3o/o to 5%o of
patients suffering sudden death per year, even if otherwise asymptomatic. CHF is responsible for 50o/oto70%o
of all deaths, but ischemia-induced ventricular fibrillation accounts for 15% to 20%o of deaths. Additionally,
with turbulent high flow across calcified stenotic valves,
there is a high incidence of thrombus formation, subsequent embolization, and endocarditis.
In
discrete and tunnel subvalvular lesions the main
cause of AS is their own obstruction of the outflow tract.
They also cause an increased velocity that damages the

normal native aortic valve. By the time of symptomatic
presentation, these subaortic lesions have a significant
valvular stenosis component.
Idiopathic hypertrophic subaortic stenosis (IHSS) is a
congenital asymmetric thickening of the left ventricle
that affects the anterior mitral valve leaflet. Under conditions of high flow, it creates a negative pressure Venturi
effect that pulls the mitral leaflet into the aortic outflow
tract. The high flow also damages the native valves, fur-
ther worsening the clinical situation. Arrhyhmias are

common with this lesion and a common cause of adolescent exertional syncope, near syncope, and sudden death.
Diagnosis
Signs and symptoms of AS are dependent on which
type ofAS is present and its degree of progression. Blood
pressure is initially normal, but as disease progresses the
pulse pressure narrows and systolic pressure drops.
Carotid pulses have a delayed, stuttering upstroke and
diminished amplitude. The apical impulse is prominent
and displaced inferolaterally. A left chest heave is possible in severe cases of LVH. A systolic thrill may be palpated at the jugular notch or in the carotids.
In vahular AS the murmur is a harsh systolic
crescendo-decrescendo ejection murrnur heard best over
the right second ICS with radiation to the carotid. The
later in systole the murmur peaks, the worse the stenosis.
A midsystolic click sometimes is heard as the stiff aortic
valve snaps fully open with increasing ventricular pressure. As the condition nears a premorbid state, cardiac
output will drop and the murmur will correspondingly
decrease in intensify. A faint munnur may be a very worrisome sign. 51 may be normal or have a paradoxical split
as the ventricular pressure causes a premature closure of
the mitral valve. Sz is delayed and may be diminished. In
severe LVH, the anatomy of the heart is so distorted that
it disrupts the tricuspid and pulmonary valves as well
causes mild regurgitation.
In discrete or tunnel varieties of subaortic stenosis the
murnur is identical to valvular AS. In IHSS there are
important differences. First, hypertrophic stenosis is a
CarulovRscut/,R DTsoRDERS
dynamic pathology, requiring high velocity flow. In a
calm ED setting the murmur may be soft to nonexistent.
Maneuvers that reduce preload (standing, Valsalva),
reduce afterload (amyl nitrate), or increase contraction
(isoproterenol) will increase blood flow and increase this
murnur, the exact opposite to valvular AS, for the above
maneuvers will decrease the murmur. Hypertrophic AS,
since it involves the mitral valve, will have a component
of mitral regurgitation.
ECG findings are usually LVH with strain, possible
ischemia, and ventricular ectopy. Chest radiographs usually show an enlarged heart, pulmonary congestion or
edema, occasionally a dilated aortic root, or a calcified
aortic valve.
o n/
Tre at m e n t
In the early stages of asymptomatic or mild AS, treatment is mainly avoidance of strenuous exercise, antibiotic
prophylaxis, and close follow-up. Serial biannual
echocardiography, with valve replacement recommended
when the cross-sectional areareaches 0.8 cm2 or the pressure gradient across the valve exceeds 50 mm Hg.
Diuresis should be avoide4 as the compensated heart
may require an increased preload to maintain CO. When
patients become symptomatic they usually require admission for monitored diuresis. Beta-blockers and calcium
channel blockers help relieve symptoms but don't alter
progression of disease. Nitrates may help angina but may
worsen syncope and hypotension. Similarly, dobutamine
may increase CO but worsen ischemia.
Valve replacement is definitive treatment. Mortality is
influenced greatly by comorbid disease and left ventricular function. Balloon valyuloplasty and open commissurotomy are better tolerated in a critical patient but have
an extremely high restenosis rate. These procedures are

normally done in younger patients as a temporizing measure until the heart finishes growing, or in women of
childbearing age who want children before valve replacement requires long-term anticoagulation.
Milral Insufticiency (MI) (2.2.5.2)

Etiology
Mitral insufficiency can either be acute or chronic. The
MI are usually ruptured chordae tendinae

or papillary muscle from myocardial infarction or acute
leaflet rupture from infective endocarditis. Chronic MI
can be caused by multiple processes, the most common of
which is mitral valve prolapse (MVP), a congenital condition causing a floppy mitral valve that is thought to
causes of acute
to l}Yo of the population. Other causes of

chronic or intermittent MI include papillary muscle
affect up
ischemia, rheumatic heart disease, hypertrophic and con-
85
gestive cardiomyopathies, left ventricular hypertrophy,

connective tissue disorders,and Marfan syndrome.
Presentation
Acute MI presents with dyspnea, tachycardia, hypotension, and fulminant pulmonary edema. If the MI is due to
myocardial infarction causing papillary muscle rupture,
there may be associated chest pain and ECG findings.
Endocarditis may present with feveq hypotension, or a
history of IVDA.
Chronic or intermittent MI presents with the symptoms
of exertional fatigue or dyspnea, and later the symptoms
of pulmonary congestion such as orthopnea and short-
ness
Int erv enti
of
breath. Very commonly ischemia
or
angina
brought on by increased demand such as fever, exercise,

or infection will mask symptoms of dyspnea. Rarely, the
presentation of chronic MI is the sequela of systemic or
CNS emboli.
Chronic MI secondary to MVP is much more atypical. Females outnumber males 2:l and familial tendencies have been documented. Only a fraction of patients
are symptomatic, however; symptoms are intermittent
and do not correlate with the severity of prolapse on
echocardiography. Common complaints include chest
pain (sharp, localized, nonexertional, and of limited

duration), palpitations, anxiety, fatigue, dizziness, and
syncope. MVP has also been associated with migraines,
transient ischemic attacks (TIAs), and cerebrovascular
embolic events, thought to be secondary to sterile
emboli to the CNS. Rarely, MVP has been associated
with endocarditis, malignant arrhythmias, and sudden
death.
Pathophysiology
In
acute
MI there is a sudden failure of the mitral
valve, usually due to the detachment of the ruptured papillary muscle, chordae tendinae, prosthetic valve, or sudden destruction of the valve leaflet itself. Left ventricular
contraction preferentially forces large amounts of blood

into the low pressure left atrium and pulmonary vasculature. The regurgitant volume may be three to four times
the forward stroke volume, resulting in sudden severe
pulmonary edema, systemic hypotension, and cardiovascular collapse. Mortality in acute MI approaches 60% to
80o% even with immediate intervention.
In intermittent MI, the cause is usually ischemia of the
papillary muscles that in turns behaves like acute mitral
insufficiency. The difference is that with return of perfusion, from rest or nitrates, the valve becomes competent
again and symptoms resolve.
In chronic MI, the regurgitant volume is initially very
small. Cardiac output is first maintained by fluid retention, then by dilation and hypertrophy ofthe left ventri-
86 /
Err4rRceNcy MrorcrNn:
THr Conn Cunnrculul,r
cle. The increased left atrial pressure transmits to the pul-
monary vasculature causing hypertension and edema.

The left atrium dilates in response to the increased pressure. Atrial fibrillation (AF) is very common in later
stages of this process. Onset of AF may precipitate heart
failure as the left ventricle may require the atrial kick for
adequate cardiac output. Like most valvular diseases
chronic MI is progressive and will eventually lead to left
or biventricular heart failure.
MVP is an exception to many of the basic rules of
valve pathology and the exact pathogenesis remains
unclear. It is thought to be a congenital defect causing a
redundant, floppy mitral valve. Associated chest pain is
thought to be from stretching of the papillary muscle.
Often MVP is associated with other connective tissue
diseases such as Marfan and Ehler-Danlos syndromes.
The symptoms are intermittent in nature and usually provoked by stress. It is common for patients to be asymptomatic during examination. Most cases of MVP are not
problematic, nor do they progress in severity.
Diagnosis
Acute MI usually presents in a state of cardiopulmonary collapse. Hypotension is severe and refractory;
CHF is fulminant. The pansystolic murmur is often
masked by tachycardia, tachypnea, and, rales. The ECG
may show left-sided ischemia but no evidence of LVH or
left atrial enlargement (LAE). Radiography shows a normal cardiac silhouette and severe pulmonary edema.
Chronic MI may be mil( presenting with only mild

dyspnea or exertional fatigue. There may be a diminished late carotid upstroke. As congestion worsens,
more respiratory complaints occur. A left parasternal
heave and thrill are common with ventricular hypertrophy. There is a harsh pansystolic murmur heard best at
the apex with radiation to the axilla and back. An Sr and
S+ are common. ECG findings include LAE, LVH with
strain, AF, and evidence of cardiac atherosclerosis (Q
waves, conduction blocks, etc.). Radiographs show an
enlarged left ventricle, pulmonary congestion, and loss
of the posterior hilar window on lateral views from
LAE.
Patients with MVP often have an abnormal body
habitus such as pectus excavatum or scoliosis. There
may be only a faint murmur or no murmur at all.

Maneuvers that decrease end-diastolic volume (standing, Valsalva) will increase the intensity of MVP's classic midsystolic click (the leaflet snaps as it prolapses)
and subsequent late systolic murmur. Like MI, this murmur is also heard best at the apex with radiation to the
axilla and the back. ECG often shows ectopy and prolonged Q-T intervals, as well as nonspecific S-T
changes in the inferior and lateral precordial leads.
Chest radiography is usually noncontributory.
Int ery enti
on / D i sp o s i t i o n
In the setting of acute MI, rapid airway and hemodynamic support is needed as well as oxygen and afterload
reduction with nitroglycerin and nitroprusside, even if the
blood pressure is being supported. These measures

improve forward flow and myocardial function. Dobutamine is helpful but may worsen ischemia. Intraaortic balloon pump is helpful in augmenting cardiac output. Early
consultation with a cardiothoracic surgeon is essential.
In chronic MI, asymptomatic or mildly dyspneic

patients can be referred for outpatient management.
Treatment is limited to diuretics and fluid and salt restric-
tion. If symptoms worsen, the patient will need admission

for diuresis and improvement of ventricular function. AF
may precipitate hypotension, pulmonary edema, ischemia, and confusion. Emergent cardioversion or rate control with diltiazem is appropriate. Surgical valve replacement is definitive.
Mitral valve prolapse, while it may cause many disconcerting symptoms to the small subset of affected individuals, is rarely in need of treatment. Reassurance is
usually effective. Beta-blockers and calcium channel

blockers have been used in some patients to alleviate
symptoms. Those with significant ECG changes or regurgitant flow should be referred to a cardiologist. Rarely,
MVP requires valvular replacement.
Mitral Stenosis (See 2.2.5.2)

Etiology
Almost all (90 to 99%) cases of isolated mitral stenosis (MS) are the result of rheumatic heart disease. Very
rarely others occur including infectious endocarditis,

congenital malformations, idiopathic annular ring calcium deposits, and left atrial myxomas.
Presentation
Usually symptoms begin in the fifth decade of life. The

latent period between the episode of rheumatic fever and
development of symptoms averages 20 to 25 years. Half
of an patients present with a gradually worsening course
of dyspnea, palpitations, or hemoptysis. Untreated, these
symptoms generally worsen until patients present with
CHF. The other half present with new-onset atrial fibrillation. This valvulopathy is progressive with an 85% mor-
tality 20 years after onset of symptoms.

MS is also very prone to thrombus formation and subsequent embolization. Patients may present with a variety
of symptoms such as chest or abdominal pain, findings of
endocarditis, or focal neurologic defects consistent with
embolic events.
Cenuovescur-qn Drsorunns
87
Pathophysiology
In asymptomatic or very mild MS, treatment should be

limited to antibiotic prophylaxis for procedures. Other
Rheumatic fever causes an inflammatory reaction in

the mitral valve leaflets resulting in thickening, loss of
mobility, and, in 75o/o of cases, commissural fusion.
When valvular cross section become one-half normal,
systemic symptoms begin to develop. With the stenotic
mitral valve obstructing blood flow from the left atrium,
pressure rises, causing pulmonary hypertension. Hemoptysis occurs from dilated bronchial venules bursting
under the high pressure.
The left atrium dilates progressively as compensation
until the predominant left heart failure becomes biventricular as the load on the right ventricle increases. Rightsided heart failure produces signs such as JVD, ascites,
and edema. The maximally dilated atrium is also very
prone to arrhythmias, and in later stages atrial fibrillation
is more common than not. AF is also extremely refractory
to conversion to a sinus rhythm. Onset of AF predisposes
to acute decompensation.
With the dual problems of stenosis with its blood stasis andAF, risk of thrombus formation is extremely high.
From 9% to 20o/o of MS patients suffer from embolic
CVAs. The damaged mitral valve is also extremely susceptible to endocarditis from transient bacteremia. Extra
care must be taken to ensure prophylaxis during procedures and to consider endocarditis when patients present
medications may mask worsening symptoms that should

alert the patient and the primary care provider to a worsening condition and the need for valve replacement, balloon vahuloplasty, or commissurotomy. These later procedures have much lower restenosis rates in MS than in
aortic stenosis.
Atrial flutter and fibrillation is poorly tolerated in these
patients. The heart needs a long diastole to allow adequate blood to pass through the narrowed opening into
the left ventricle. AF with rapid ventricular response can
cause significant cardiovascular collapse. Cardioversion
should be avoided unless absolutely necessary due to the
risk of emboli. IV diltiazem is indicated for rate control,
followed by IV digoxin. Venodilators and diuretics should
be avoided or used very cautiously as the ventricle may
require high preload pressures for adequate filling. Significant hemoptysis may require intubation and blood
with fever.
Diagnosis
In the early stages of MS physical examination findings are subtle. There is a diminished or absent apical
impulse from an underfilled left ventricle. Sometimes a
palpable diastolic thrill may be felt at the apex.
MS gives a characteristic early diabolic opening snap
as the stiff valve leaflets open, and a low-pitched, diastolic decrescendo rumbling murmur best heard at the
apex. As the disease progresses, the S1 opening snap may
disappear as the valve becomes too stiff to open quickly.

ECG is notable for LAE, atrial ectopy, and atrial fibrillation. Late in the disease right axis deviation and right
ventricular hypertrophy are noted. Chest radiography

usually shows the loss of the posterior hilar window on
lateral views from LAE. Sometimes calcification of the
mitral leaflets and annular ring can be seen. Acute pulmonary edema is noticeable immediately.
Int erv enti
on /D i sp o s i t i o n
Since rheumatic heart disease is overwhelmingly the
primary cause of MS, the first step in its treatment is the

prevention of rheumatic fever. Proper evaluation and
treatment of streptococcal pharyngitis will largely prevent MS from occurring.
transfusions.
Pulmonary Insufficiency
(2.2. 5.3)
Etiology-
The most common cause of pulmonary insufficiency

(PI) is pulmonary hypertension with subsequent dilation
of the pulmonary annular ring. Other causes include congenital malformations, mitral stenosis, rheumatic fever,
COPD, left ventricular hypertrophy, and infective endocarditis usually from IV drug abuse or tuberculosis.
Presentation
Isolated PI is tolerated for may years without significant pathology or progression. Any symptoms that
patients experience are usually the result of pulmonary
hypertension or underlying cardiac pathologies. Usual
symptoms are fatigue, dyspnea, shortness of breath, or
syncope.
Pathophysiology
Pulmonary hypertension directly causes a gradual

dilatation of the valvular ring, which produces valvular
incompetence and regurgitant flow. Isolated PI is very
rare and rarely causes any significant symptoms in the
low pressure right heart system. More commonly, it is PI
in conjunction with a left-sided vahular lesion (MI, MS,
or AS) that precipitated the pulmonary hypertension.
Those vahular lesions
will
cause significant derange-
ments requiring intervention long before

severe.
PI
becomes
88 /
ErranRcnNcv MrorcrNn:
Tsn Conn Cunnrcurutr
Endocarditis can become problematic, and antibiotic

prophylaxis is recommended.
Diagnosis
Physical findings are subtle. In severe cases there may
be right ventricular hypertrophy (RVH) with a corresponding right chest heave, but usually the only evidence
of PI is a high-pitched, blowing crescendo-decrescendo
diastolic murmur at the left second ICS. There may be a
faint systolic murmur as blood is ejected across the damaged valves.
ECG may be normal or have evidence of RVH in

severe cases. Chest radiography is usually normal or
shows changes consistent with pulmonary hypertenslon.
Int erv enti
on
/D i sp o s i t io n
Isolated PI is usually well tolerated and no intervention

is required. Vasodilators are helpful but should be given
very cautiously as hypotension is common if preload
drops too far. In severe cases, pulmonary hypertension is
treated with diuretics. In rare cases, valvular replacement
is required. More commonly, treatment is based on the
other cardiac abnormalities.
Pulmonary Stenosis (See 2.2.5.3)

Etiology
Like aortic stenosis, the obstruction in pulmonary

stenosis (PS) may be infravalvular, valr,.ular, or
supravalvular. Unlike aortic stenosis, which has a variety
of causes, PS in all positions is usually the result of congenital defects. Often PS is found only during the evaluation of other, more serious congenital defects.
body responds with right ventricular dilatation and hypertrophy, as well as fluid retention to increase preloadind
augment forward flow.
The lesion is graded as mild when the pressure gradient is <65 mm Hg, moderate from 65 to 120 mm Hg, and
severe if >120 mm Hg.
Diagnosis
The physical examination of a patient with pS is characteristic. A prominent a-wave in the jugular veins is
noted. An early right parasternal lift and a systolic thrill
felt in the left second ICS and suprasternal notch are
caused by RVH.
The murmur of PS is classically a harsh, high-pitched
crescendo-decrescendo systolic ejection murmur, best
heard at the left second ICS. There may be an early opening snap as the stiff pulmonic valves open and 52 is
widely split as the right ventricle maintains pressure

longer and delays pulmonic closure. The murmur will
increase with inspiration (Carvallo's sign).
ECG will show evidence of RVH with strain. Chest
radiography will show right ventncular enlargement and
dilation of the pulmonary arrery if the lesion is
supravalvular. The pulmonary vasculature is normal.
In
te
rv en ti o n/D i sp
o s it i o
Treatment in the asymptomatic and mild early phases

should be withheld so as not to mask symptoms. patients
should be monitored with serial ECGs, and valve replace-
ment should be considered if right ventricular pressure

exceeds 70 mm Hg or if the gradient exceeds 50 mm Hg.
If the patient has moderate to severe symptoms, treatment is supportive with oxygen and possibly pressor sup-
port. Vasodilators should be avoided as a decrease in pre-
load may precipitate hypotension. Arrhythmias
and
sudden death have been reported, so ECG monitoring is

Presentation
PS usually remains asymptomatic for many years but
eventually causes fatigue, dyspnea, and syncope. These
symptoms are exacerbated by high-output states such as
fever, exercise, and infection. Sudden death has been
reported in patients with PS. Endocarditis can complicate
this righfsided lesion and patients may present with
fever, hypoxemia, and embolic bacterial pneumonia,

much like IVDA.
Pathophysiology
PS obstructs the blood flow from the right ventricle

into the pulmonary artery. To maintain cardiac oueut the
critical.
Tricu spid In s uffic ie n cy (2. 2.
5.
4)
Etiology
Most commonly, tricuspid insufficiency (TI) is a result
of right ventricular dilation and failure. And since
the
most common cause of right heart failure is left heart failure, evaluation for TI is often a search for left heart
pathology. Other causes include rheumatic heart disease,
infectious endocarditis, congenital valve deformity, endocardial cushion defects, prolapsed leaf syndrome, trauma,
and papillary muscle damage. IV drug abuse is particu-
larly prevalent in tricuspid pathology
as injected particu-
Cenuovescur-AR DrsoRDERs
late and bacterial contaminants impact first against the
tricuspid valve.
89
Presentation
Presenting symptoms are a result of increased systemic venous pressure. Common complaints include
Presentqtion
Symptoms are usually those of rightsided venous

overload such as painful hepatosplenomegaly, ascites,
peripheral edema, ascites, and hepatosplenomegaly. If

the mitral or aortic valves are affected as well, there may
be the presenting symptoms of fatigue, dyspnea, and
and peripheral edema. In cases where the TI is the result

of left heart pathology, these often dramatic symptoms
will predominate.
Pathophysiology
Pathophysiology
With RVH the tricuspid annular ring dilates slowly

causing an increasing stream ofregurgitant blood into the
central venous circulation. This increases the venous
pressure with its associated symptoms, and causes the
right atrium to dilate, predisposing to AF and other
arrhythmias.
Diagnosis
There are prominent signs of right-sided venous hyper-
tension such as jugular venous distention with a pronounced c-v wave; a palpable right ventricular heave; and
a soft, blowing holosystolic munnur best heard over the
left sternal border of the xiphoid. The intensity of the
sound
will
increase
with deep inspiration (Carvallo's
sign).
ECG shows RAE, RVH, and commonly atrial fibrillation. Chest radiography shows altered right heart outlines
from RAE and RVH, with normal pulmonary vascula-
orthopnea.
Rarely does TS cause a problem. The valvular cross

section of the normal valve is 7 cm2 and patients rarely
become symptomatic until the stenosis is <1.5 cm2.
Coexistent aortic or mitral disease usually presents symptomatically much earlier, and TS is found during their
evaluation.
TS can, however, become sy,rnptomatic. With a
stifftriis obstructed to the right ventricle.

Right atrial dilatation occurs to increase forward flow.
cuspid, valve flow
This predisposes to atrial arrhythmias, especially AF,

which with rapid response and other tachycardiac states
are very poorly tolerated by patients with TS. The lowpressure right heart system needs a long diastole for adequate stroke volume, and increased heart rates will
directly decrease cardiac output.
Of secondary concern is the increased risk of endocarditis. Since the most common cause of TS is IV drug
abuse, endocarditis is often the presenting complaint in
patients with TS.
Diagnosis
ture.
I n t erv e n t i on /D i sp o s iti o n
Salt and fluid restriction is the treatment of choice for
mild cases. More severe cases need the addition of diuretics and evaluation for valve replacement. In cases of TI
secondary to left heart problems, correction of the pri-
mary lesion takes precedence.

When the patient suffers fromAF, rate control with diltiazem in the acute phase, or digoxin in the chronic phase,
and long-term anticoagulation are required.
Tricuspid Stenosis (See 2.2.5.4)
Etiology
Tricuspid stenosis (TS) is an uncommon entity, and if
found premortem, usually results from endocarditis secondary to IV drug abuse or rheumatic fever. Isolated
rheumatic involvement of the tricuspid is rare; usually
mitral and aortic pathology coexist and predominate.
Patients with TS exhibit signs of right-sided venous

hypertension; peripheral edema, hepatosplenomegaly,
and ascites are common. fVD and large jugular a waves
may be present. The atrium contracting against the
obstructed valve has the pressure wave deflected up the
superior vena cava, causing thejugular findings.
A low-pitched, rumbling decrescendo diastolic murmur can be heard at the left fourth and fifth parasternal
spaces. Carvallo's sign, an accentuation of the murmur
with inspiration, is common.
ECG shows evidence of RAE and atrial arrhythmias,
especially AF. Chest radiography shows an enlarged right
atrium and possibly a dilated superior vena cave. Pulmonary architecture will remain normal unless there is
mitral or aortic comorbidity.
Int ery enti
on /D i sp o s it i on
In the asymptomatic or mild phase patients may be discharged with close follow-up, and salt and fluid restriction. More serious symptoms need inpatient evaluation
90 /
EN{uncrNcy MsorcrNs:
Tnr Conr CunnrculuM
by echocardiography with possible surgical intervention.

Close evaluation for any left heart valvular pathology is
with history or laboratory evidence of streptococcal

pharyngitis should be assumed to be RF and treated
mandatory.
Rate control of AF is critical. The dilated right atrium
accordingly.
is usually difficult to convert to sinus rhythm, so most

patients will need inpatient admission for digoxin loading
and anticoagulation. Diltiazem is recommended for initial rate control.
Etiologlt
Prior to the invention of antibiotics, rheumatic

fever/heart disease was a leading cause of death in children and the most common cause of heart disease in
adults over 40 years. Now, due to the widespread use of
antibiotics, rheumatic heart disease (RHD) is uncommon
in the U.S., although outbreaks
are still
group
B-hemolytic streptococcal
(GABS) infections of the pharynx. Isolated cases of strep

pharyngitis and all cases of streptococcal skin infection
have not been associated with RF, but the incidence of RF
approaches 3o/o of all untreated epidemic GABS outbreaks.
Children between 4 and l8 years ofage are at the highest risk of GABS infections, although both younger and
older patients have been described. Up to one-third of all
patients do not recall having pharyngitis in the weeks preceding the onset of symptoms of RF. For those that do,
the latency between strep pharyngitis and onset of symptoms averages 18 days but ranges from I to 5 weeks.
The Jones criteria (Table 2-14) were established over
50 years ago, revised in 1965, and remain the hallmark
for both presenting symptoms and diagnosis of RF. Presence of two major or one major and two minor criteria
TABLE 2-14. Jones criteria for rheumatic fever

Major
GABS infection;either
a positive throat culture
or positive ASO titers
Pathophysiology
The exact mechanism of pathology remains unknown
but its relation to GABS infections, and the almost uniform presence of antibodies to valve and joint connective
tissue in affected serum suggests a hyperimmune
response. New research is now finding similar proteins in
GABS and on native heart valves, myocardium, joints,
and skin. Current evidence suggests that antibodies to
GABS cross-react and incite damaging inflammation
affecting native connective tissues.
Presentation
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Plus evidence of recent
ical and laboratory findings can be negative. Erythema

marginatum is painless, nonpruritic erythematous circles
found on the trunk and proximal extremities. Subcutaneous nodules are pea-sized, nontender lumps usually on
bony prominences. And as one would expect, fever is
usually present.
reported.
Rheumatic fever (RF) is more commonly a problem in

developing countries.
RF and its subsequent valvular complications are a
of
of proportion to physical findings. Carditis may be found

in up to 40o/o of patients, and may present as chest pain,
dyspnea, or heart failure, dependent on its severity.
Chorea consists of random, purposeless movements usually ofthe upper body and face. It is frequently the only
symptom and may be seen with carditis, but for some
unknown reason is not seen with arthritis. All other phys-
Rheumatic Heart Disease
direct result
The migratory polyarthritis usually affects the larger

joints (knees, elbows, ankles, and wrists) and has pain out
Minor
Fever
Diagnosis
As stated above, the diagnosis is made by recognizing

fulfilling the Jones criteria. Additional signs and laboratory findings are outgrowths from these. Carditis may
and
present as a new murmur, pericardial effirsion, or congestive heart failure. ECG may show a prolonged PR inter-
val and chest radiography may show evidence of pulmonary congestion or edema and alteration of cardiac
size and shape. The erythrocyte sedimentation rate (ESR)
is nonspecifically elevated and constitutes a minor crite-
rion. Up to half of all patients may also have mild proteinuria. While a third of all patients don't recall a pre-
ceding pharyngitis, and throat cultures done
on
presentation of RF are usually negative, antistreptolysin

antibody (ASO) titers are usually elevated for 4 to 6
weeks and indicate recent GABS infection.
Arthralgias
Elevated ESR or C-reactive
protein
Prolonged PR interval on
ECG
History of previous
rheumatic fever
I nt erv e ntio n/D i sp o s itio n

The best treatment for RF is to prevent its occurrence.
Treatment with antibiotics up to 7 to 9 days after GABS
pharyngitis has been show to prevent development of RF.
Rapid strep tests, commonly used in EDs and clinics, are
Canorovnscur_AR DrsoRDERs
insensitive in finding GABS. A positive result allows for

treatment but a negative result is not helpful and a culture
must be done to rule out GABS infection. Throat cultures
are much more sensitive but much less useful in ED set-
tings since results will not be known for several days,

making follow-up difficult. Clinical judgment as well as
ability to follow-up should guide EPs in whether to treat
pharyngitis with antibiotics.
The treatment consists of either a single IM benzathine
penicillin injection (600,000 units for children under 25
kg, and 1.2 million units in adults) or a full l0-day course
of penicillin V 250 mg. This is usually dosed tid or qid,
but evidence has shown bid dosing is effective but has the
danger of treatment failure if even only one or two doses
are missed. Penicillin allergic patients may take erythromycin estolate 20 mglkg divided bid or erythromycin
ethylsuccinate (EES) 40 mg/kg divided bid for 10 days.
New evidence has shown effectiveness of azithromycin,
clarithromycin, and some second- and third-generation
cephalosporins in treating GABS pharyngitis, but no evidence exists as yet as to whether they prevent RF. patients
should always be instructed to complete the full l0-day

course ofantibiotics, as stopping early has been associated with treatment failures and development of RF.
Treatment for acute RF is mainly supportive. No treatment has yet been shown to reduce the time course or
degree of pathology. Patients should be admitted for supportive and pain control measures. Eradication of any
GABS should be started with parenteral benzathine penicillin (same dose as for pharyngitis, qd for l0 days). In
cases ofcarditis both glucocorticoids and salicylates have
been used with mixed results.
Following recovery from RF, patients need prophylactic antibiotics since repeat infections with GABS and RF
sequelae occur and are usually more severe. The literature
addressing length of prophylaxis is unsettled. Some recommend until age 18, some until age 35, and others
indefinitely regardless of the initial severity. Current
/ gl
ease. Before that, patients had a well-documented, pro_

gressive, and uniformly fatal worsening of their corrAi_
tion, despite all medical management. With modern
valves,
and the ability to monitor the
ology with echocardiograPhY,
ity are reduced substantially. Key to this success, however, is early recognition of
valvulopathies and intervention prior to irreversible damage.
Valve replacement is not without risks. Besides the

risks from open heart surgery there are the complications
of acute valve failure, paravalvular leak, endocarditis,
thrombus formation, hemolysis, valve degeneration, and
problems stemming from long-term anticoagulation.
Presentation
The patient's initial complaints will depend on the

valve involved and method of failure. Acute valve failure, either in an open or closed position, may precipitate
sudden cardiovascular collapse. Failure in the open position causes acute insufficiency, while failuri in the
closed position acutely blocks forward flow beyond the
valve.
mech
cific
embo
va
or
al
focal neurologic symptoms are highly suggestive of this
etiology. Hemolysis may present with orthostasis, jaun-
dice, or feelings of fatigue. Complications from anticoagulation are varied but may present with easy bruising,
mucosal bleeding, painful swollen joints, or mental status
changes in the case of spontaneous CNS hemorrhage.
Pathophysiology
There are two main categories of prosthetic valves,
ecific complicaneed close follow-up with serial physical examinations

and echocardiography to watch for developing valvular
disorders before irreversible myocardial changes have
occurred.
Prosthetic Valves
Etiology
The development of prosthetic heart valves in the
1950s revolutionized the treatment of valvular heart dis-
largely replaced
known.
each should be
The mechanical valves fall into either the caged ball or

tilting disk varieties. The caged ball (Starr-Edwards) consists of a metal cage with a floating ball that forms a one-
way valve.
The tilting
styles but
door. Their
The advantage of mechanical valves is their extremely
long life span of 20 years compared to tissue valves, g to
10 years. Disadvantages are the required need of long-
92 /
ErvmncnNcv Mnorcrur:
Tsr
Conn CunnrculuM
term anticoagulation, predisposition for thrombus formation (2 to 3o/oper. year), and hemolysis by the mechanical
action of opening and closing. Of special note is that the
Bjork-Shiley valve has been associated with an unacceptably high rate of metal strut breakage and acute failure.
Patients with valves from the affected batches may opt for
elective replacement.
Tissue prostheses are either homografts or heterografts. Homografts are human cadaver heart valves, and
heterografts are porcine or bovine valves or pericardial
tissue that are preserved and grafted onto artificial stents
for transplantation into human hosts.
Biological prostheses require no long-term anticoagulation beyond the initial postoperative stage, and hemolysis is rarely a problem. Disadvantages, however, are a
short life span requiring replacement at 8 to 10 years and
the risk ofearly degeneration and valve failure. They are
usually not acceptable for younger patients. Thrombus
formation, while less than mechanical valves, is still a
risk at loh to 2o/o ayear.
Diagnosis
Complications with prosthetic valves are a common
occurrence with devastating consequences. Acute valve
failure, paravahular leak, and thrombus formation present as acute valvular insufficiency or stenosis in the
affected valve. Diagnosis is made clinically by considering symptoms and auscultating a new mu{mur. Emergent
echocardiography or fluoroscopy may be required to
visualize a stuck, incompetent valve, or formed thrombi,
and help in differentiating prosthetic valvular pathology
from mimics such as pulmonary embolism and acute

myocardial infarction.
Endocarditis is diagnosed
by physical examination
positive
coupled with
blood cultures. Blood cultures
should always be drawn and antibiotics started on all
patients with prosthetic valves presenting with fever.
Clinically significant hemolysis is suspected with jaundice, elevated LDH, falling hematocrit, and peripheral
smear showing red cell fragmentation consistent with
mechanical' disruption.
Possible complications from anticoagulation should be
investigated with appropriate clotting studies. Cranial CT
is mandatory with mental status changes or focal neurologic deficits, as intracranial hemorrhage or embolic disease may be present.
I nt em e ntio n/D isp o s itio n
All patients with signs or symptoms suggestive of

valve dysfunction must be assumed to have it until
will need appropriate supportive
and admission for monitoring, imaging,
catheterization, or valve replacement if warranted. In
proven otherwise. They
measures
severe cases with unstable patients, hemodynamic support and emergent cardiothoracic surgery consultation
are required.
Valve replacement patients with fever or other findings

must be assumed to have endocarditis. Blood cultures
should be drawn and the patient admitted for IV antibiotics until culture results are known.
Patients with symptomatic chronic hemolysis may be
treated with blood transfusions and discharged home; but
close outpatient follow-up
is
mandatory because
increased hemolysis is sometimes an early sign of valve

failure. Acute hemolysis should not be presumed to be
valve-related except during the immediate postoperative
period.
SELECTED READING
Aronorv WS, Ahn C, Kronzon I, et al. Prognosis of patients with heart failure and unoperated aortic valvular regurgitation and relation to ejection
fiaction. Am J Cardiol 1994;74:286J88.
Bashore TM, Lieberman EB. Aortic/mitral obstruction and coarctation of
the aorta. Cardiol Clin 1993;l l(4):617-641.
Baxley WA. Aortic valve disease. Carr Opin Cardiol 1994;9:152-157.
Carabello BA. The changing and unnatural history of valwlar regurgitation. Ann Thorac Surg 1992;53:19I-199.
Currie PJ. Valvular heart disease, a correctable cause of congestive heart
faih:;e. Postgrad Med 1991 ;89(6):123-136.
Devlin WH, Starling MR. Outcome of valvular heart disease with vasodilator therapy. Compr Ther 1994;20(10):569-574.
Follman DF. Aortic regurgitation, identifying and treating acute and ckonic
disease. Postgrz d M ed 1993 ;93 (6): 83-88.
Harris JP. Evaluation of heart murmurs. Pedian'Ret 1994:15(12):490493.
Rahimtoola SH. Management of heart failure in valve regurgitation. CIin
C ardio I 1992;1 5 (I) :22-27 .
Rahimtoola SH. Recognition and management of acute aortic regurgitation.
Heart Dis Stroke 1993;2:217-221.
Waller BF, Howard J, Fess S, et al. Pathology of aortic valve stenosis and
pure aortic regurgitation. C lin Cardiol 1994;17 :1 50-156.
Waller BF, Howard J, Fess S, et al. Pathology of mitral valve stenosis and
pure mitral regurgitation. Clin Cardiol 1994;17:395402
Zuppiroli A, Rinaldi M, Kramer-Fox MS, et al. Narural history of mitral
valve prolapse. Am J Cardiol 1995;75:1028-1032.
Myocarditis (2.2.6) (See 13.2.3.3)

Myocarditis is an infrequent etiology of cardiac disease; nevertheless,
it is often associated with serious
sequelae consisting of cardiac failure, significant

arrhythmias, and sudden death. The low incidence of
myocarditis in the population is borne out in several postmortem studies showing a range of I .06% to 5.4o/o. The
most common etiologic agents are the Enterovirus genus,
with coxsackie virus being responsible for the majority of
cases seen in the United States and Europe, accounting
for as many as 50oh of reported cases. Other causative
viral agents include poliovirus and echovirus. However,
the causes of myocarditis are numerous and extremely
variable (Table 2-15),In addition, myocarditis has a high
association with dilated cardiomyopathy (DCM), being
detected in 63% of the patients with DCM who undergo
endomyocardial biopsy.
CerurovesculAR DTsoRDERS
TABLE 2-15. Etiologies of myocarditis
Viral
Coxsackie A and B
Echovirus
Adenovirus
lnfluenza
Varicella
Poliomyelitis
Mumps
Hepatitis B
Epstein-Barr
Cytomegalovirus
Herpes simplex
Protozoan
Malaria
Schistosomiasis
Trypanosomiasis
Toxoplasmosis
Medications
Acetaminophen
Lithium
Doxorubicin
Catecholamines
Cocaine
HIV
Bacterial
Diphtheria
Salmonella
Mycobacterium sp.
Streptococcus
Meningococcus
Clostridium
Legionella
Rickeftsia sp.
Fungal
Aspergillosis
Histoplasmosis
Candidiasis
Actinomycosis
Blastomycosis
Cryptococcus
Chemical
Lead
Arsenic
Carbon monoxide
93
and dyspnea to cardiac failure and sudden death. It is suspected that the majority of cases of myocarditis are sub-
clinical and go unrecognized. A directed history will

often reveal an antecedent or concurrent viral infection.
Typically the time interval between the onset of viral illness and cardiac involvement is 2 weeks. There should be
a high index of suspicion for myocarditis in any patient,

regardless of age, with new onset of cardiac abnormalities, particularly congestive heart failure, and a recent
history of viral infection.

The most common presenting symptoms in the adult
are fatigue, dyspnea at rest and on exertion, palpitations,
pyrexia, and precordial chest pain consistent with pericarditis. Leukocytosis is a common finding along with an
elevated erythrocyte sedimentation rate. Tachycardia is a
frequent finding and is classically out ofproportion to the
of pyrexia. Cardiac auscultation often demonstrates a pericardial rub, an 33 if congestive heart failure
is present, and a decrease in intensity of 51. Often a sum-
Radiation therapy
degree
Systemic disease
Systemic lupus erythematosus
Connective tissue diseases
Sarcoidosis
lnflammatory disorders
mation gallup is noted with biventricular involvement,

and an apical murmur consistent with mitral regurgitation
may be heard. On pulmonary auscultation a pleural rub is
frequently heard, and with severe failure pulmonary rales

are present. Peripheral edema may be present in cases
of
severe myocarditis with accompanying congestive heart
failure.
Pediatric patients, particularly infants, commonly present with a fulminant course. Their symptomatology con-
Pathophysiolog;
The two defining characteristics of myocarditis are

myoc)'te necrosis and interstitial mononuclear cell infiltration. Cardiac involvement is often focal but may be
diffi.rse, involving both ventricles along with the pericardium. The pathogenesis of the disease is not well
defined yet is recognized to have two stages. Viralinduced myocyte necrosis predominates in the acute
stage, and is prevalent in neonatal myocarditis. In the
chronic stage, cytotoxic T lymphocytes infiltrate the
myocardium and mediate destruction of host myocardial
cells in a virus-specific manner. Further destruction of
the ml,ocytes occurs with autoimmune activity directed
against car diac myo sin. Myoc ardial autoantibodie s re sult
from myosin released from lysed myoc).tes. This autoimmune activity persists long after viral particles are no
longer detected. Further complicating the pathogenesis
further are reports finding damage to the coronary vessels with luminal obliteration and thrombus formation.
The ensuing ischemic damage compounds the immune
mediated response, exacerbating the clinical presenta-
tion. Finally, the conduction system of the heart
is
involve4 displaying a wide array of conduction defects.
Clinical Findings
The clinical features of myocarditis are extremely variable, ranging from the nonspecific symptoms of fatigue
sists of pyrexia, respiratory distress, cyanosis, tachycardia, and cardiac failure along with systemic involvement.
The electrocardiographic findings progress serially
and can often help to differentiate acute myocarditis from
acute myocardial infarction. ST:segment elevation without reciprocal ST:depression is one of the most common
findings in acute myocarditis. In addition, the total QRS
amplitudes are significantly decreased in the acute stage.
Transient findings consist of abnormal Q-waves that

quickly resolve along with second degree or higher atrioventricular block. Both left and right bundle branch
blocks may occur and may remain for months. An important note: 40% of patients with any viral infection may
have electrocardiographic changes suggestive of myocardial involvement (Table 2-16).
Chest radiograph initially reveals a normal cardiac silhouette, but with pericardial involvement or cardiac dilation an increase in cardiac'silhouette is seen. Echocardio-
graphy demonstrates impaired systolic and diastolic

function, a decreased ejection fraction, or a pericardial
TABLE 2-16. ECG characteristics of myocarditis
ST elevation without reciprocal ST depression
Decreased QRS amplitudes
Abnormal and transient Q waves (septal Q waves)
Transient Mobitz type I second-degree AV block
Progression to Mobitz type ll second-degree AV block
Third-degree AV block
Bundle branch blocks that may persist for months
94 /
EuencnNcy MnucrNr: TsB Conn Cunnrculurr,r
effirsion. Cardiac enzymes are helpful as they demonstrate a characteristic pattern of slow elevation and fall
over a period ofdays in contrast to the abrupt rise seen in
acute myocardial infarction.
Isolation of the virus from the myocardium is incontrovertible evidence of myocarditis, yet only 30% of
patients with clinical myocarditis have unequivocal

endomyocardial biopsies. Viral isolation from body fluid
cultures is supportive of the diagnosis. Recently, poly-
merase chain reaction (PRC), in which a specific chain of

viral nucleic acid is amplified, has been employed in the
detection ofa specific viral agent, providing a rapid and
sensitive method
for myocardial and body fluid viral
identification.
Therapy
Interventional treatment for myocarditis has consisted
of restricted physical activity and treatment of congestive
heart failure with diuretics and digitalis glycosides. Anticoagulation with coumadin or heparin is initiated given
the increased incidence of thromboembolic complica-
tions that may occur with myocarditis. Captopril, an

angiotensin-converting enzyme inhibitor, has been beneficial when there is significant left ventricular failure and
symptoms of congestive heart failure. Beta-blockers
should be avoided because they increase the extent of
myocyte necrosis and mortality. Sympathomimetic
agents should be used with caution since they can cause
toxic myocarditis.
The efficacy of immunosuppressive therapy with corticosteroids and cyclosporin is controversial. Given in
acute myocarditis, corticosteroids have been shown to
increase viral replication and enhance myocardial necrosis. Cyclosporin has been shown to be deleterious in mice
with acute myocarditis. However, these agents may have
a role in the later stage of myocarditis, though definitive
trials are not yet available and immunosuppressive therapy has never shown proven benefit. Nonsteroidal antiinflammatory agents are contraindicated in early myocarditis because they also enhance myocardial necrosis.
Although there are several promising experimental protocols involving Tcell suppression, antiviral pharmacologic agents, and virus specific immunization, none has
yet shown significant benefit. Cardiac transplantation

may be considered if severe cardiac failure occurs; however, these patients have an allograft rejection rate more
than twice that of recipients who do not have myocarditis
as the underlying cause ofcardiac failure.
SELECTED READING
myocarditis.lrch Pathol Lab Med
1991;115:390-392.
Martin AB, Webber S, Fricker J, et al. Acute myocarditis rapid diagnosis by
PRC in children. Circulation 1994;90:330-339.
Gravanis MB, Sternby NH. Incidence of
McNulty CM. Active viral myocarditis: application of current knowledge to

clinical practice . Heart Stroke I 992; I : I 3 5-140.
Nakashima H, Honda ! Katayama T. Serial electrocardiographic findings
in acute myocarditis. Intern Med 1994;33:659466.
Olinde KD, O'Connell JB. Inflammatory heart disease: pathogenesis, clinical manifestations, and treatment of myocarditis. Annu Rev Med
1994;45:481490.
Peters NS, Poole-Wilson PA. Myocarditis-continuing clinical and pathologic confusion. Am Heart J 199l;121:942-947.
See DM, Tilles JG. Viral myocarditis. Rev Infect Dis 1991;31:951-956.
Smith NM, Bourne AJ, Clapton WK, Byard RWi The spectrum of presentation at autopsy of myocarditis in infancy and childhood. Pathology
1992;24:129-131.
DTSEASES OFTHE PERTCARDTUM (2.3)
Pericardial diseases are becoming more frequently

recognized in emergency medicine. In part this is largely
due to patients with neoplastic disorders, renal failure,
connective tissue disorders, and immunologic disorders
living longer with advanced medical care. In addition, the
rise in specific infectious diseases such as HI! tuberculosis, and lyme disease has made a significant contribution to the occurrence of pericardial disease. Epidemiologic data are scarce concerning pericardial diseases, but
it is estimated that pericardial effi.rsions occur in l2%o of
patients with known cardiovascular disease andthat l5Vo
to 20o/o of patients with HIV have pericardial effirsions.
Similarly, pencardial effirsions have a relatively high

occurrence rate in congestive heart failure (14%), valvular heart disease (21%), and myocardial infarction (15%).
Given the incidence of pericardial disease, the emergency
physician needs to be astutely attuned to the presentation
and management of the various aspects of this increasingly prevalent disease process.
Pathophysiology
The pericardium is composed of two distinct layers, a
collagenous-parietal layer, which surrounds the visceral
pericardium, and the fibrous visceral pericardium, which
lies adjacent to the epicardium. A potential space exists in
which l0 to 15 cc of pericardial fluid is normally present.
This fluid functions to reduce the friction between the
layers of the pericardium as the myocardium contracts.
Interestingly, the pericardial space allows for the accumulation of 200 cc of fluid before intrapericardial pressure rises to a significant level, which results in impairment of cardiac function. If the increase in pericardial
fluid is gradual, much larger amounts of fluid can be
accommodated without major impairment in cardiac
function. Pericardial fluid is usually serous in characteq
but may be inflammatory, purulent, hemorrhagic, or a
combination.
As pericardial fluid increases, the intrapericardial pressure rises in an exponential fashion (Fig. 2-3). This
increase in pressure primarily affects the right atrium and
right ventricle, which depend on systemic venous pres-
Cerurovnscur-rn DrsoRnnns
95
TABLE 2-17. Etiology of pericarditis
Pressure
200m1
100m1
Volume
FlG. 2-3. Pressure-volume curve of increasing pericardial
pressure.
sure as the major filling mechanism. As intrapericardial

pressures increase right atrial transmural pressure
increases, resulting in decreased venous refurn to the
right atrium. When pericardial pressure exceeds right
atrial pressure, collapse of the atrium occurs. The net
effect can be a dramatic decrease in stroke volume of the
nght ventricle. To maintain cardiac output, a compensatory tachycardia and increased systemic vascular resisin systemic resistance
ultimately maladaptive and increases the
afterload, further limiting cardiac output. Eventually, systolic and diastolic collapse of the right ventricle occurs
with subsequent severe hypotension. At this point, if the
tance occurs. However, this increase
is
Etiology
Comments
lnfectious
Viral
Coxsackie A and B
Echovirus type 4
Adenovirus
lnfluenza
Cytomegalovirus
Epstein-Barr virus
Mumps
Varicella
Herpes simplex
Most often associated with

patients less than 30 years
of age
High association with the

immunocompromised
patient
Hepatitis B
Bacterial
Mycobacterium sp.
Borrelia burgdorferi
Staphylococcus
Streptococcus
Gram-negative rods
Legionella
Rickettsia sp.
Systemic diseases
Systemic lupus
erythematosus
Rheumatoid arthritis
Connective tissue
disorders
Sarcoidosis
Myxedema
Uremia
I nf lammatory disorders
Neoplastic disorders
Lung cancer
Breast cancer
Lymphoma
Leukemia
Usually young patients but of

increasing occurrence
Age group is often the older

patient and the pericardial
effusion is often chronic in
character
Predominantly occurring in
the older patient
intrapericardial pressure is not reduced quickly, death

occurs rapidly.
disorders. Uremia is a less common cause of pericarditis
(Table 2-17).
Pericarditis (2.3.1) (See 13.2.3.1)

Although often thought to be idiopathic, the etiology
ofacute pericarditis is age dependent. In patients younger
than 30 years of age, the most common etiology is infectious, primarily viral. Coxsackie A and B predominate,
followed by echovirus, adenovirus, and influenza. Viral
isolation is exceedingly rare. Bacterial causes are next
with Mycobacterium tuberculosis and Borreliq burgdorferi of increasing significance. In the immunocompromised patient, Mycobacterium may be the principal agent
along with varicella, cytomegalovirus, herpes simplex,
and Epstein-Barr virus.
In the older patient the principal etiology is neoplastic:
lung cancer, breast cancer, lymphoma, and leukemia. Following neoplasms, infectious causes predominate, partic-
ularly M. tuberculosis.
In addition, the
etiologies of
chronic pericardial effirsions tend to be the neoplastic disorders and the systemic diseases of systemic lupus erythematous, rheumatoid arthritis, and the connective tissue
Clinical Findings
Historically the patient may relate
a recent
viral illness
in the days to weeks preceding the onset of symptoms.

Often this viral illness is an upper respiratory infection
that typically resolves prior to the onset of symptoms.
The classic presentation is that of severe, nonanginal
chest pain of a pleuritic quality. Frequently this chest pain
is precordial or retrosternal with radiation to the left

shoulder and arm; it often lasts for several days and is
exacerbated
by positional
changes, especially lying
supine. The patient may find relief by sitting up and lean-
ing forward. Associated signs and symptoms consist of

tachycardia, dyspnea, dysphagia, intermittent low-grade
pyrexia, fatigue, and night sweats. The historical finding
that the pain is not exacerbated by physical activity is
important in helping to distinguish between pericardial
96 /
EnrnncrNcy MeucrNr:
Tnr
Conn CunrucuLUM
pain and ischemic cardiac pain, as are the prolonged

nature and positional characteristics ofthe pain.
A pericardial friction rub is often present and of significant clinical importance because it denotes inflammation of the visceral pericardium. Most often this rub is
biphasic with a systolic and diastolic component best
heard with the patient leaning forward. However, the classic rub is triphasic with systolic, diastolic, and presystolic
components. This rub is often described as creaking
leather or the sound of walking on dry snow. It is best
heard with the bell of the stethoscope in the lower left
sternal border and cardiac apex. The rub is typically transient, lasting only a few hours to several days.
The electrocardiographic changes in acute pericarditis
result from superficial inflammation of the epicardium.
They have been described as serial changes consisting
first of widespread" concordant ST:segment elevation
without reciprocal depression, followed by return of the
ST segment to baseline with later T:wave changes, then T:
wave inversion with the ST segment at baseline, concluding with a return to normality. PR-segment depression is
thought to be sensitive and specific. It is important to
note that these changes occur in only 50o/o to 70%o of the
patients with documented pericarditis. In addition,
another helpful finding is significant J-point elevation; if
it exceeds 25oh of T-wave voltage, it is strongly suggestive of acute pericarditis. Sinus rhyhm is present in 90%o
of the cases documented, but occasionally atrial fibrillation and flutter have also been documented. Electrical
alternans is noted infrequently (Table 2-18).
Chest radiographic findings are typically normal
unless an extensive amount of pericardial fluid is present.
With large pericardial effusions, the cardiac silhouette
may be globular, configured like a Spanish water bottle.
In addition, relatively rapid increases in the size of the
cardiac silhouette without evidence of pulmonary congestion should alert the emergency physician to a pericardial effi.rsion. Other radiographic findings may include
a left pleural effr.rsion or bilateral pleural effirsions.
The principal diagnostic study used to demonstrate
pericardial effirsion is two-dimensional echocardiography. Echocardiography can demonstrate as little as 17 ml
TABLE 2-18. Electrocardiographic changes in acute

pericarditis
Concordant ST-segment elevation except leads aVR
and Vr
Absence of ST-segment depression except in lead aVR
and often Vr
PR-segment depression
J-point elevation
Decrease in QRS voltage when pericardial effusion is
present
Sinus rhythm in 90% of documented cases
lnfrequently atrial fibrillation and flutter
lnfrequently electrical alternans
of pericardial fluid. Small effirsions are first seen as a

fluid collection posterior to the left ventricle, and larger
effirsions may be seen anterior to the right ventricle or
surrounding the heart. Echocardiography is extremely
useful in demonstrating cardiac tamponade and guiding
needle pericardiocentesis.
Therapy
Treatment for viral and inflammatory pericarditis consists of nonsteroidal antiinflammatory medication with
indomethacin being recommended most frequently. Bacterial and rickettsial infections may require appropriate
antibiotic therapy for periods of up to 6 months. Pericarditis resulting from M. tuberculosis requires therapy
with isoniazid, rifampicin, ethambutol, and prednisone.
Prednisone is frequently used in cases of pericarditis with
associated protracted pain and a relapsing course. Pericardiocentesis is indicated for cardiac tamponade with
rapid decompensation, suspected purulent pericarditis,
and as a diagnostic procedure in a patient with an unresolved or enlarging pericardial effi.rsion. A pericardial
window may be the treatment
of choice with those
patients with a chronic pericardial effi.rsion.
Pericardial Effusion/Tamponade (2.3,2)

Cardiac tamponade warrants special consideration
because the causes of tamponade are numerous and the
mortality extremely high. Most often cardiac tamponade
results from an increase in pericardial fluid from metastatic tumor. Other leading causes are idiopathic pericarditis, viral and bacterial infections, trauma, connective
tissue disorders, anticoagulant therapy, and as a complication from diagnostic cardiac procedures, particularly
cardiac catheterization.
As the pericardial fluid or blood accumulates around
the heart, there is an eventual equalization of intrapericardial and right atrial pressures. At this point right atrial
collapse begins to occur and becomes complete when the
intrapericardial pressure exceeds the right atrial pressure.
Soon right ventricular collapse follows as the pericardial
pressure becomes equal to the diastolic venous pressure.
This equalization of pericardial and diastolic venous

pressure results in an inability of the right side of the
heart to fill with blood. The net effect is a marked drop in
stroke volume and biventricular output. In turn, heart rate
and systemic and pulmonary venous pressures are significantly increased as compensation occurs
profound and progressing hypotension.
to the often
Clinical Findings
Clinically, the patient with cardiac tamponade presents
with dyspnea, tachycardia, a narrowed pulse-pressure
Cenuovescur-AR DTsoRDERS
TABLE 2-19. Etiology for a lack of pulsus paradoxus in
cardiac tamponade
Left ventricular dysfunction
Regional right atrial tamponade
Positive pressure breathing
Atrial septal defect
Pulmonary arterial obstruction
Severe aortic regurgitation
echocardiographic diagnosis? Circulation 1993;87:
by systolic hypotension, and elevated systemic venous pressure as manifested by distended neck
veins. Other clinical evidence consists of diminished
heart sounds on auscultation and infrequently pulsus
manifested
alterans. A paradoxical arterial pulse (pulsus paradoxus)

is typically found in almost all cases of cardiac tamponade. A paradoxical arterial pulse is defined as an inspiratory decline of systolic arleial pressure exceeding l0
mm of Hg. However, cardiac tamponade is not the only
cause of a paradoxical arterial pulse. Other causes are
chronic obstructive pulmonary disease, severe asthma,
right ventricular infarction, pulmonary embolism,
and
constrictive pericarditis. In addition, several functional

abnormalities can result in the absence of pulsus paradoxus when cardiac tamponade is present. Pulsus paradoxus is absent in the patient with cardiac tamponade in
the presence ofpositive pressure ventilation, left ventricular dysfunction, atrial septal defect, pulmonary arterial
obstruction, severe aortic regurgitation, and regional right
atrial tamponade (Table 2-19).
Definitive diagnosis is achieved with use of twodimensional echocardiography demonstrating a large
pericardial effi.rsion often circumferential to the heart
with right atrial and ventricular diastolic collapse. Other
echocardiographic findings consist of dilated inferior
vena cava without inspiratory collapse, left atrial compression, left ventricular compression, increased right
filling with
inspiration
with
associated
increased blood flow velocity through the tricuspid valve,

and decreased left ventricular filling with inspiration and
TABLE 2-2O. Common echocardiographic findings for

cardiac tamponade
Fluid in the pericardial space anterior to the right ventricle
Right ventricular collapse during diastole
Left ventricular diastolic compression
Right and left atrial compression
Dilated inferior vena cava without inspiratory collapse
Tachycardia
lnspiratory increase in right ventricular dimensions
lnspiratory decrease in left ventricular dimensions
"Swinging heart"
Modified from Fowler NO. Cardiac tamponade: a clinical
or
echocardiographic diagnosis? Circulation 1993;87:
1738-1741, with permission.
220).
Therapy
1738-17 41, with permission.
ventricle
97
associated decreased blood flow through the mitral valve.

An additional finding of a swinging heart may be seen on
echocardiography (Table
Modified from Fowler NO. Cardiac tamponade: a clinical
or
,/
Treatment
of
acute cardiac tamponade
is
emergent,
requiring needle pericardiocentesis. Echocardiography

has the unique advantage of aiding needle pericardiocentesis; however, ifechocardiography is not available, blind
pericardiocentesis should be initiated since any delay in
relieving the tamponade may prove fatal. Without intervention the patient decompensates: the blood pressure
drops rapidly and a fatal ventricular arrhythmia is frequently the terminal event. Depending on the etiology of
the pericardial effrrsion, pericardiocentesis may need to
be repeated, and these patients need to be carefully monitored for recurrence of the tamponade.
Constrictive Pericarditis
Constrictive pericarditis is an inflammatory reaction
involving the lining of the pericardium. Both the visceral
and parietal layers are involved, obliterating the pericardial space. Because the pericardial space is occlude( this
process is distinct from pericarditis. Typically, the inflammatory changes are slow and extend over a period of
months to years. Intrapericardial hemorrhage is thought
to be the most common, etiology followed by radiation

therapy and nonspecific pericarditis. However, almost
any ofthe etiologies responsible for acute pericarditis can
cause constrictive pericarditis. It should be noted that
acute rheumatic fever and myocardial infarction are very
rare causes.
Hemodynamically there is an equal elevation of both

the right and left ventricular filling pressures, resulting in
decreased right ventricular compliance and elevation of
systemic venous pressure. Right atrial and pulmonary
wedge pressures are approximately equal, varying within
only 4 to 5 mm Hg of each other; there is, however, a
preservation of systolic function.

C linical Finding s/Therapy
The clinical presentation is not unlike that of congestive heart failure. These patients may present with dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and lower
extremity edema characteristic of elevated systemic

venous pressure. These symptoms do not improve with
diuresis as would be the case in congestive heart failure.
In the setting of
constrictive pericarditis. The ECG
changes are nonspecific, consisting of decreased voltage

and inverted T:waves. There may be intraatrial conduction
defects resulting in atrial fibrillation or atrial flutter. Evi-
98 /
EuBncsNcy MnucrNn: THn Conn CunrucuLUM
dence of right ventricular enlargement may be noted on

ECG secondarily to localized fibrotic obstruction of the
right ventricular outflow tract.
Chest radiographs reveal pericardial calcifications in
only 40%o of the patients with constrictive pericarditis.
The cardiac silhouette is typically normal or only slightly
increased in size. Echocardiography is most often nonspecific but occasionally may show pericardial thickening. Computed tomographic (CT) scanning is the most
useful diagnostic modality because it can clearly demonstrate the presence of pericardial thickening. Magnetic
resonance imaging (MRI) is also very useful in defining
the extent of pericardial thickening. Infrequently, CT
scanning may fail to demonstrate pericardial thickening,
and the differential diagnosis of restrictive cardiomyopathy must be entertained. In this particular situation, pericardial biopsy becomes a necessary diagnostic adjunct.
Definitive treatment is pericardiectomy in patients with
significant symptoms. However, in those patients in

which the responsible etiology is radiation therapy or
connective tissue disease, pericardiectomy can be ineffective in alleviating symptoms because of the associated
restrictive cardiomyopathy that often accompanies these
two etiologies.
SELECTED READING
RI, Mohanty PK. Diagnosis of
cardiac tamponade after cardiac surgery: relative value of clinical,
echocardiographic, and hemodynamic signs. Am Heart J 1994;127
Chuttani K, Tischler D, Pandian NG, Lee
913-918.
FowlerNO. Cardiac tamponade:

C irculatio
a clinical or echocardiographic diagnosis?

993 ;87 : 17 3 8-17 41 .
Pericardial disease. Heart Dis Stroke 1992;1(2)85-94.
FowlerNO.
Houghton JL. Pericarditis and myocarditis: which is benign and which
isn't? Postgrad Med 1992;91 :27 3182.
Kirkland LL, Taylor RW. Pericardiocentesis. Cril Care Clin 1992;8:
699-712.
Maisch B. Pericardial diseases, with a focus on etiology, pathogenesis,
pathophysiology, new diagnostic imaging methods, and treatrnent. Curr
Op in C ardio I 1 994;9 :37 9-388.
Mazurek B, Jehle D Martin M. Emergency department echocardiography
in the diagnosis and therapy ofcardiac tamponade. J Emerg Med l99I'
9:2711.
Ward D. Pericardial and myocardial diseases. Ptactitioner 1993;237:
929-932.
DISEASES OF THE CONDUCTION SYSTEM

(DTSTURBANCES OF CARDTAC RHYTHM) (2.4)
To aid in the understanding ofdisturbances ofcardiac
rhythm, it is important for the emergency physician to
be familiar with the normal conduction system of the
heart. The primary site of impulse generation in the
undiseased heart is the sinus node, which is located in
the right atrium at the junction of the atrium and the
superior vena cava. In the majority of individuals, the
blood supply to the sinus node arises from a branch of
the right coronary artery; however, in up to 45o/o of indi-
viduals the sinus node is supplied by a branch of the circumflex aftery.

The rate at which the sinus node discharges is controlled by the autonomic nervous system. Sympathetic
nerve endings, mediated by catecholamines, increase the
rate of firing of the sinus node. Parasympathetic nerve

endings, mediated by acetylcholine released by the vagus
nerve, hyperpolarize the sinus node, thus decreasing the
rate of depolarization and the frequency of discharge.
Once the discharge leaves the sinus node it travels
quickly throughout both atria, initiating atrial contraction.
The impulse moves along the anterior interatrial myocardial band directly to the left atrium. Further specialized
tracts (the anterior middle, and posterior internodal
tracts) conduct the impulse directly from the sinus to the
atrioventricular (AV) node.
The AV node is located in the right atrium near the
coronary sinus and tricuspid valve. In the vast majority
(approximately 90%) of individuals, the blood supply to
the AV node arises from the right coronary artery. The
emergency physician should be aware that since both the
sinus and AV nodes receive the majority of their blood
supply from the right coronary artery, decreased flow
through this artery (as seen with an acute inferior myocardial infarction) is associated with disturbances ofnormal conduction. Like the sinus node, the AV node is also
innervated
by both sympathetic and
parasympathetic
nerve endings that serve to increase and decrease conduction of impulses through the AV node, respectively.
There are two major features of the AV node. The first
is to slow the conduction of the impulse from the atria to
the ventricles. This slowing allows time for the atria to
contract, maximizing ventricular filling. The other specialized feature is a long refractory period. The increased
refractory period helps protect the ventricles from overstimulation in situations where the rate of impulses from
the atria are excessively rapid. Other cells within the AV
node can serve as the pacemaker in situations where the
sinus node has failed to fire or is generating impulses at
too slow a rate.
Upon leaving the AV node, the impulse travels to the
bundle of His located in the intraventricular septum. The
common bundle is only approximately 12 mm in length
and then divides into the right and left bundle branches
(RBB and LBB). The RBB travels down the right side of
the septum separating into smaller branches. The LBB
travels 2 to 3 cm down the septum before dividing into
the thinner left anterior superior fascicle and the larger
left posterior inferior fascicle. Both the RBB and LBB
eventually divide into Purkinje fibers, which conduct the
impulses throughout the ventricles initiating depolarization.
Abnormalities in any portion of the normal conducting
system can lead to dysrhythmias. The most common
causes of dysrhythmias are automaticity, reentry, or triggered automaticity. Automaticity refers to the potential of
Camrovescut-{R DTsoRDERS
the pacing cells ofthe heart to spontaneously depolarize
and initiate the impulse for contraction. This may be seen
with damage secondary to myocardial infarction or with
drug toxicities. Reentry is the most common cause of
dysrhythmias and is associated with myocardial damage
or the presence ofan accessory pathway. Accessory pathways, or bypass tracts, are embryologic remnants of myocardium that can conduct impulses between the atria and
ventricles, bypassing the normal conduction mechanism.
Triggered automaticity occurs when an ectopic pacemaker depolarizes and becomes the dominant pacemaker.
This is seen with ischemia, drug toxicities, and electrolyte imbalances.
Diagnosis
History and Physical Examination
As with any patient encounter in the ED, the importance of the history and physical examination cannot be
overstated. Of course, if the patient presents in extreme
distress, the usual order of taking a history, performing a
physical examination, obtaining appropriate diagnostic
tests, and initiating treatment must be modified. Addressing potential life threats and instituting appropriate measures adhering to the ABCs of resuscitation is indicated.
As soon as the patient's condition permits, a history

should be obtained. The history is usually best obtained
from patients themselves; however, additional resources
such as family members, EMS personnel, medical
records, and drugs (illicit or prescribed) should not be
overlooked. Factors such as description and onset of
symptoms should be identified. The presence of associated symptoms, alleviating/exacerbating factors, history
of previous symptoms, as well as identification of cardiac
risk factors and the presence ofpossible allergies should
also be obtained. Simultaneously with obtaining the his-
tory and instituting appropriate resuscitative measures, a

directed physical examination should be quickly performed. The information gathered in the history and
physical examination should dictate the ordering of further tests as well as guide diagnostic and therapeutic decisions.
E lectrocardiogram (EC G)
The ECG is an invaluable tool in the recognition and

diagnosis of cardiac dysrhythmias and should be
quickly obtained on any patient presenting with a complaint of suspected cardiac origin. The ECG is helpful in
diagnosing specific dysrhythmias as well as identifying
the presence of cardiac ischemia/infarction, conduction
delays, and structural anomalies (ventricular hypertrophy, accessory pathways). While it is beyond the scope
ofthis chapter to give a detailed explanation ofelectro-
99
cardiography, a brief description of the important complexes and intervals and how they relate to conduction is
indicated.
The P wave represents atrial depolarization and is best
identified in leads II and Vr. The normal duration of the

P wave is less than 0.10 seconds. The absence of a
defined P wave indicates that the impulse did not generate within the sinus node and is seen with various dys-
rhythmias (e.9., afral fibrillation/flutter, junctional

rhythms, ventricular dysrhythmias). Atrial repolarization
is usually obscured by the QRS complex and not identi-
fied on the ECG.
The PR interval is used to estimate the conduction time
within the AV node. The normal PR interval is between

0.12 and 0.20 seconds. Prolonged PR intervals may be
seen with AV blocks and drug toxicities, while shortened
PR intervals may represent accessory pathways (WolffParkinson-White syndrome).
The QRS complex represents ventricular depolarization and normally ranges from 0.04 to 0.10 seconds. Prolongation of the QRS complex may represent an intraventricular conduction delay (bundle branch block) or an
impulse that originated below the bundle of His premature ventricular contraction (PVC).
The ST segment indicates the plateau phase of ventricular depolarization. Abnormalities of the ST segment
may represent ischemia, infarction, or drug effects.
The T wave represents ventricular repolarization. The
height and width of the T wave is variable and is affected
by multiple factors such as ischemia, infarction, hypertrophy, drugs, and abnormal repolarization.
The QT interval is an indication of the total time for
ventricular depolarization and repola ization. The normal
QT interval commonly ranges between 0.33 and 0.42 seconds; however, this measurement must be corrected for
heart rate. This is done by dividing the QT interval by the
square root of the RR interval. Variation in the QT segment often represents drug toxicities or metabolic abnormalities.
Cardiac Monitoring
While the ECG is a valuable adjunct, it is important to

remember that it represents the electrical activity of the
heart at a single point in time. Many dysrhythmias may be
intermittent; therefore, continuous cardiac monitoring of
the patient while in the ED is essential. A low threshold
for obtaining repeat or serial ECGs should be maintained,
particularfy if dysrhyhmias occur or there is a change in
the patient's condition.
Other diagnostic modalities such as laboratory testing,
radiography, echocardiography, cardiac stress testing,
cardiac catheteizations, and electrophysiologic examinations may all be indicated. Their use must be individualized to specific patient needs.
100 /
EvrncoNcy MnorclNn: Tnn Conn CunnrculuM
Therapeutic Adjuncts
Medicutions
The most common, and unfortunately sometimes most
overlooked, "medication" used in the care of the patient
with a cardiac dysrhythmia is oxygen. Many dysrhythmias can be caused or exacerbated by hypoxemia. Since
myocardial ischemia/infarction may also precipitate dys-
rhythmias, the empiric administration of supplemental

oxygen to all patients with suspected dysrhythmias,
whether or not they are exhibiting overt signs of respiratory distress, is indicated. The use of specific antidysrhythmic medications will be discussed in the context of
the specific dysrhythmias.
Vagul Maneuvers
Measures
to increase vagal tone have been shown to
decrease conduction and prolong the refractory period of

the AV node. These maneuvers can be valuable in the
diagnosis and treatment of certain supraventricular tachycardias. Potential maneuvers include carotid sinus massage, the Valsalva maneuver, gagging, and facial immersion in ice cold water (the diving reflex). The success of
these maneuvers in the termination of specific dysrhyth-
mias is variable; but, since they are generally safe and

noninvasive, they are often useful tools for the emergency
physician.
Electrical Therapy
Electrical therapies are useful adjuncts in the treatment
of the energy delivered to avoid the vulnerable period

during ventricular repolarization where the electrical
impulse may initiate ventricular fibrillation. Synchronized cardioversion utilizes lower energy levels and is
used with unstable tachydysrhythmias when a pulse is
present.
Transdermal and transvenous pacemakers are commonly utilized in the ED in the management of hemodynamically unstable bradydysrhythmias. Both types of
pacing involve the generation of a current to the heart in
order to stimulate myocardial contraction. Transcuta-
neous pacemakers have the advantage of being more eas-
ily
applied and initiated. Transcutaneous pacemakers

should be prophylactically applied to patients with significant bradydysrhythmias, AV blocks (Mobitz II, thirddegree), and patients with new bi- or trifascicular blocks
in the setting of an acute myocardial infarction so that
they can be quickly activated should the patient become
unstable. Pacemakers may also be utilized to terminate
certain tachydysrhythmias [supraventricular tachycardias
(SVTs), torsades de pointes]. By pacing the heart at arate
faster than the tachydysrhyhmia and then stopping, overdrive pacing allows the heart's intrinsic pacemaker to take
over.
Dysrhythmias (2.4.1) (See 13.2.1)
In this discussion of specific dysrhythmias and their

recognition and treatment, it is assumed that the basic
tenets of emergency care such as stabilization (the
ABCs), supplemental oxygen, intravenous access, continuous cardiac monitoring, assessment/monitoring of the
vital signs, and obtaining an ECG will be applied to all
of cardiac dysrhythmias. Indeed" they are often the treatment of choice in certain situations. Defibrillation and
cardioversion are utilized to terminate certain unstable
patrents.
tachydysrhythmias. The process involves passing a direct

current across the myocardium in an effort to depolarize
Sinus Bradycardia
all of the cardiac cells. After repolarization, a dominant

pacemaker, such as the sinus node, can take over and
transmit impulses in the normal manner. Defibrillation is
associated with higher energy requirements and is
reserved for ventricular fibrillation and pulseless ventricular tachycardia. C ardioversion invo lve s synchroni zation
Sinus bradycardia is characterized by a decrease in the

rate of atrial depolarization secondary to a slowing of discharges from the sinus node. The ECG findings of sinus
FlG.
2-4.
bradycardia include an atrial rate less than 60 beats per

minute (bpm), normal P waves and PR intervals, and oneto-one AV conduction (Fig. 2-4).
Sinus bradycardia.
CRnorovescur-AR DTsoRDERS
TABLE 2-21. Treatment of sinus bradycardia
101
Sinus Tachycardis
Rarely requires treatment

Search for underlying causes (e.9., drug effects,
hypothyroidism, acute Ml) and institute appropriate
treatment.
lf hemodynamically unstable
Transcutaneous pacemaker
Atropine 0.5-1.0 mg lV (may repeat q 5 min to total dose
Sinus tachycardia occurs when there is an increase in

atrial depolarization secondary to an acceleration of discharges from the sinus node. The characteristics of sinus
tachycardia seen on ECG are an increased atrial rate (usually between 100 and 160 bpm), one-to-one AV conduction, and usually normal P waves and PR intervals (Fig.
of 3.0 mg)
Epinephrine 0.5-1.0 mg lV (may repeat q 5 min as
needed)
lsoproterenol drip 2-10 mcg/min (drug of choice in
patient status post-heart transplant)
Dopamine drip 2-20 mcg/kg/min
2-s).
Like sinus bradycardia, sinus tachycardia may represent
pathologic conditions or drug effects, or be physiologic.
There are many etiologies of sinus bradycardia.

Some are representative of pathologic causes such as
acute inferior myocardial infarction, hypothyroidism,
and increased intracranial pressure. Other etiologies
may be the result of certain medications, for example
Pathologic conditions associated with sinus tachycardia

include hyperpyrexia, pulmonary embolus, hypovolemia,
hypoxia, hyperthyroidism, and congestive heart failure
(both high- and low-output states). Medications that can
cause sinus tachycardia include cocaine, atropine, epinephrine, Bz-agonists, xanthenes, and sympathomimetics.
Physiologic etiologies of sinus tachycardia are exercise,

emotional stress, and fear. Sinus tachycardia is a normal
finding in resting heart rates of infants and small children.
digoxin, beta-blockers, calcium channel blockers, seda-
Sinus tachycardia rarely requires treatment. Initial
tives, or narcotics. Furthermore, sinus bradycardia may

occur in settings unrelated to pathologic processes such
as during sleep, vagal maneuvers, or in conditioned ath-
measures should be aimed at eliminating the underlying

cause(s). In the setting of suspected ongoing myocardial
letes.
Clinical manifestations vary based on the etiology. The

need for teatment, if any, is based on the patients condition. If the patient is asymptomatic, addressing possible
pathologic causes and continued monitoring are indicated. In the rare instance where sinus bradycardia causes
hemodynamic compromise, treatment should begin with

transcutaneous pacing. If a pacemaker is not immediately
available, agents such as atropine, epinephrine,

dopamine, and isoproterepol may be utilized (Table
t tt\
FlG.
2-5.
ischemia or infarction, treatment may be required with

administration of beta-blockers. In the setting of cocaine
toxicity, benzodiazepines may be useful (Table 2-22).
Atrial Flutter
(2.4. 1. 1)
Atrial flutter is a dysrhythmia that usually originates in

the right atria. Although felt by some to be the result of a
reentry circuit, the exact mechanism is unknown. ECG
characteristics ofatrial flutter include a regular atrial rate
ranging from220 to 350 bpm (usually around 300 bpm),
and absence of normal P waves (the "sawtooth" flutter
Sinus tachycardia.
TABLE 2-22. Treatment of sinus tachycardia

Rarely requires treatment
Search for underling cause (e.9., pulmonary emboli, drug effects, fever) and institute appropriate
treatment
lf unstable or suspect ongoing cardiac ischemia
Beta-blockers-propranolol 1.0-3.0 mg lV, atenolol 5.0 mg lV (may repeat up to 15.0 mg total
dose)
Benzodiazepines-diazepam 2.0-5.0 mg lV, lorazepam 1.0-2.0 mg lV (especially useful in setting
of cocaine toxicity)
102
EnmncrNcy MnorcrNs: THs Cons CunrucuLUM
FlG.
2-6. Atrial flutter.
waves are best seen in leads II, III, and aVF) (Fig. 2-6).
The PR interval and QRS complexes are usually normal,
but may be variable. The ventricular response depends on
the degree of block at the AV node. The block may be
constant (usually 2:l) or variable. When 2:1, the patient
frequently presents with a ventricular rate of 150 bpm,
and the rhythm may initially be mistaken for a sinus

tachycardia. Vagal maneuvers or an intravenous dose of
adenosine may be helpful in making the diagnosis by
decreasing the ventricular response and exposing the flutter waves.
The possible etiologies for atrial flutter are many; how-
eveq
it
seldom presents in patients without underlying
heart disease. Causes of atrial flutter include ischemic

heart disease, acute myocardial infarction (AMI),
hypoxia, hypokalemia, pulmonary embolism, thyrotoxicosis, myocarditis, congestive cardiomyopathy, valvular
disease, and drug toxicity (digoxin).
Clinical manifestations of atrial flutter are dependent
on the etiology, the patient's existing medical condition,
and the ventricular response. The patient may complain
of minimal symptoms such as palpitations or mild shortness of breath or may present with severe respiratory distress, hypotension, and ischemic pain.
Treatment of the patient with atrial flutter is based on

the clinical presentation. If an etiology listed above is
identified" measures to correct that condition should be
instituted. If the patient is tachycardiac and hemodynamically unstable, immediate synchronized cardioversion is
indicated. The majority of cases of atrial flutter respond
to low-energy cardioversion (25-50 J). If unsuccessful,
repeated cardioversion at progressively higher energy
levels may be necessary.
In the stable patient, control ofthe ventricular response
is the immediate goal. Many agents such as diltiazem,
verapamil, digoxin, and beta-blockers may be utilized.

Verapamil and beta-blockers should be used with caution
in patients presenting u'ith congestive heart failure (CHF)
as they may exacerbate rhis condition. If the patient is
presently taking digoxin, blood levels should be obtained
prior to administenng additional digoxin as atrial flutter
is a rare consequence of digitalis toxicity. The above

agents are used primarily for rate control; however, they
do exhibit a low incidence (20-30%o) of conversion of
atrial flutter to a sinus rhythm. Once rate control has been
achieved, a type I antiarrhythmic such as procainamide or
quinidine can be administered to help convert the dysrhythmia and prevent recurrence (Table 2-23).
TABLE 2-23. Treatment of atrial flutter
Synchronized cardioversion 25-50 (if unsuccessful, reattempt at progressively higher energy
levels)
lf stable, initial goal is rate control

lf diagnosis is unclear, vagal maneuvers or adenosine 6.0 mg rapid lV push may slow ventricular
response enough to establish dysrhythmia identification
Diltiazem 0.25 mg/kg lV (may repeat at 0.35 mg/kg if no response); if successful, may begin
continuous lV infusion at 10 mg/hr
Verapamil 2.0-5.0 mg lV"
Digoxin 0 4
.0.6 mg lV (if patient presently on digoxin, consider digitalis toxicity as potential cause
of dysrhythmia)
Beta-blockers-propranolol 1.0-3.0 mg lV, atenolol 5.0 mg lV (may repeattototal dose of 15.0 mg
tv)'
When rate control is achieved

Procainamide 50 mg/kg/day po
Quinidine 1-2 tabs q po 8-12 hr
aUse with caution in the setting
of CHE
Cenolovascur-qn Drsononns
FlG.
103
2-7. Atrial fibrillation.
Atrial Fibrillation (See 2.4.1.1)
Atrial fibrillation results from multiple areas within

the atria continuously discharging. This may be caused by
many ectopic foci or multiple areas of reentry. The result
is a lack of orderly depolarization and effective contraction of the atria. While the rate of atrial electrical discharges typically ranges from 400 to 700 per minute,
decreased conduction by the AV node results in an average ventricular response of 160 to 180 bpm. Characteristics of atrial fibrillation seen on the ECG include no discernible P waves, a chaotic baseline of fibrillatory waves
best seen in leads Vr, Vz, V3, and aVF, and an "irregularly
irregular" ventricular response usually at 160 to 180 bpm
(Fig. 2-7). The ventricular rate may be faster or slower
based on preexisting cardiovascular status and the presence of certain drugs, especially digoxin. The QRS width
is usually unaffected unless aberrant conduction exists.
Atrial fibrillation may be constant or intermittent; therefore, continuous monitoring is indicated.
The most corunon conditions associated with atrial fibrillation are rheumatic heart disease, ischemic heart disease, hypertension, and thyrotoxicosis. It may also be seen
with pericarditis, atrial septal defects, acute myocardial
infarction, and acute alcohol intoxication (holiday heart).
As with atrial flutter, the clinical manifestations seen
with atrial fibrillation are variable. In patients with compromised cardiac output, the loss of eflective atrial contractions may precipitate congestive heart failure.
Angina, respiratory distress, and hypotension are also
common presentations with acute atrial fibrillation, par-
ticularly with a rapid ventricular response. Patients with

long-standing atrial fibrillation may remain relatively
asymptomatic, if the ventricular rate is controlled.
Chronic atrial fibrillation predisposes the patient to arterial embolic events (up to l5% of patients per year). If the
onset of the atrial fibrillation is not clear, it is not recommended to convert the patient to a sinus rhythm without
first instituting anticoagulants. Administration of heparin
or warfarin for a period of I to 3 weeks prior to cardioversion will help prevent the sequelae of arterial
embolism of an intraatrial thrombus.
While it is ideal to first anticoagulate patients in atrial
fibrillation of chronic or uncertain duration prior to
attempting conversion of the rhythm to sinus, if the
patients condition warrants, the treatment of choice is
synchronized cardioversion. Atrial fibrillation usually
requires higher energy levels for conversion to a sinus
rhythm than does atrial flutter. Starting at 100 J and escalating doses as needed is recommended. If the first
attempts at cardioversion are unsuccessful, administration of intravenous procainamide may enhance success. If
the patient is hemodynamically stable, control of the ventricular response is desirable. Many agents may be used.
Digoxin has long been used and is effective; however, the
onset of action may be slow (mean time to conversion of
approximately 11 hours). Diltiazem is very effective in
slowing the ventricular response, often in less than l0
minutes. Once rate control has been establishe4 attempts
to pharmacologically convert the atrial fibrillation to a
sinus rhythm may be attempted with the use of procainamide, quinidine, or verapamil (Table 2-24).
TABLE 2-24. Treatment of atrial fibrillation

Synchronized cardioversion, 100 (if unsuccessful, reattempt at progressively higher energy levels)
lf stable, but tachycardic, attempt to control ventricular response
Digoxin 0.4-0.6 mg lV (if patient presently on digoxin, consider digitalis toxicity as potential cause
of dysrhythmia)
When rate control is established

Procainamide 50 mg/kg/day po
Quinidine 1-2 tabs po q 8-12 hr
Verapamil 24O-32O mg/day (divided tid or qid)"
aUse with caution in the setting of CHF.
704
EunncrNcy MnorcnE,: TFtr Conn CunrucuLUM
FlG.
Supraventricular Thchycardiu
2-8.
Supraventricular tachycardia.
(2.4. 1.4)
Supraventricular tachycardias (SVTs) are the result of

a reentry circuit or an ectopic foci occurring above the
bundle of His. A reentry mechanism accounts for approximately 80% of SVTs, with three-fourths of those occurring in the AV node. The other 25Yo involve the presence
of bypass tracts. Bypass tracts, as seen in Wolff-Parkinson-White (WPW) syndrome, are accessory pathways
located outside the AV node (e.g., Kent's bundle) that can
conduct impulses into the ventricle.
The ECG typically demonstrates a regular rate of 160
to 200 bpm; however, ranges between 100 and 250 bpm
have been reported. The P wave is often obscured by the
QRS complex and difficult to identify (Fig. 2-8). Patients
with WPW may exhibit a shortened PR interval <0.12

seconds) and a slurring of the upstroke of the QRS complex (delta wave). The AV conduction in SVTs is usually
1:1. The QRS complexes are usually narrow even in the
setting of bypass tracts. Wide QRS complexes may be
rate related, resulting from abnormal conduction through
a bypass tract or seen with preexisting bundle branch

blocks. Aberrantly conducted SVTs are often mistaken
for ventricular tachycardia.
Reentry SVTs can be seen in patients with normal
hearts or may be associated with rheumatic heart disease,
pericarditis, myocardial infarction, mitral valve disease,
stimulant use, or in the presence of an accessory pathway.
Acute paroxysmal episodes (PSVTs) are more commonly
associated with a reentrant phenomenon than with
ectopic foci. Ectopic SVTs are commonly associated
with digitalis toxicity, myocardial infarction, hypoxemia,
COPD, and alcohol intoxication. Symptoms range from
mild palpitations to severe respiratory distress, hypotension, and anginal-like chest discomfort.
Treatment is based on suspected etiology and patient
condition. Regardless of suspected cause, if the patient
presents with severe distress and hemodynamic insta-
bility, synchronized cardioversion is the treatment of

choice. Like atrial flutter, PSVT usually responds to
low energy levels and it is recommended to begin at
50
J.
TABLE 2-25. Treatment of supraventricular tachycardia

Synchronized cardioversion 50 J (if unsuccessful, reattempt at progressively higher energy levels)
Reentrant SVT in a stable patien
Vagal maneuvers (e.9., Valsalva, carotid sinus massage)
Adenosine 6.0 mg rapid push (if unsuccessful, may repeat at 12.0 mg for 2 doses)
Verapamil2.0-5.0 mg lV
of dysrhythmia)
Beta-blockers-propranolol 1.0-3.0 mg lV, atenolol 5.0 mg lV (may repeat to total dose of 15.0 mg)
Overdrive pacing (rarely necessary)
lf ectopic SVT
of dysrhythmia)
Verapamil2.0-5.0 mg lV
continuous lV lnfusion at 10 mg/hr
Beta-blockers-propranolol 1.0-3.0 mg lV, Atenolol5.0 mg lV (may repeat to totaldose of 15.0 mg)
lf SVT thought to be secondary to digitalis toxicity
Digitalism specific antibodies (Fab)
Phenytoin 15.0 mg/kg (infuse no greater than 25-50 mg/min)
Cardioversion ineffective
Carurovescur-AR DTsoRDERS
Vagal maneuvers are often helpful to both diagnose
and treat SVTs. As vagal maneuvers aie generally safe,
effective, and noninvasive, they should be attempted first
in the stable patient. By slowing the ventricular response,
the underlying rhythm is much easier identified in cases
when the diagnosis is unclear. With reentry SVI vagal
maneuvers can terminate the circuit by increasing the
refractory period in the AV node.
If vagal maneuvers
agent
if
105
TABLE 2-26. Treatment of multifocal atrialtachycardia

Treat underlying hypoxia (oxygen, bronchodilators)
Control ventricular rate
Magnesium sulfate 1.0 g lV
Verapamil 2.0-5.0 mg lVa
Beta-blockers-propranolol 1.0-3.0 mg lV, atenolol 5.0

mg lV (may repeat to total dose of 15.0 mg)a
aUse with care in the setting of CHF.
are unsuccessful, adenosine should
be administered as the drug of choice. Adenosine will

produce a transient AV block that is successful in terminating approximately 90% of reentrant SVTs. Verapamil
is as effective as adenosine, but due to its mechanism of
action as a calcium channel blocker, it has been associated with more adverse effects, particularly hypotension.
However,
adenosine is ineffective, verapamil is the next
of choice. Other medications
such as diltiazem,
beta-blockers, and digoxin may also be utilized if necessary. In rare cases, overdrive pacing may be used. Once
the acute event has been terminated, referral to
an
internist or cardiologist may be indicated. Patients with

WPW have been successfully treated with radiofrequency
catheter ablation oftheir bypass tracts.
If
the SVT is thought to be of an ectopic nature

(excluding digitalis toxicity), medications that may be
helpful for rate control include digoxin, verapamil, diltiazem, and beta-blockers. Long-term antidysrhythmic
therapy with either procainamide or quinidine may be
ent morphologies, variable PR and RR intervals, and a

ventricular rate usually between 100 and 180 bpm. As the
initiation of the impulse is in the atria, the QRS complex
is usually of normal width unless a bundle branch block
exists. Due to the abnormal P wave morphologies and the
irregular ventricular rate, it is often mistaken for atrial
fibrillation.
MAI
occurs primarily
in patients with chronic lung
disease. Hypoxemia associated with the chronic lung dis-
ease appears to be the major initiating factor. Theophylline and rarely digitalis toxicity are also possible etiologies.
Clinical response is variable; however, the tachycardia

may exacerbate associated congestive heart failure. Treatment is directed toward correcting hypoxemia with oxy-
gen and bronchodilators. Rate control has also been

accomplished with magnesium, verapamil, and betablockers. Cardioversion is ineffective (Table 2-26).
necessary. Ectopic SVT in the presence of digitalis toxic-
ity warrants the use of digoxin-specific antibody fragments (Fab). Phenytoin has been traditionally utilized as
the antiarrhythmic of choice with varying success. Lidocaine and magnesium may also be used. Cardioversion in
the presence of digitalis toxicity is generally ineffective
and may precipitate more serious dysrhythmias; therefore, it is not recommended (Table 2-25).
Mu
lffi c al Atria I Tac hy c ardia
Atrial Ectopy (2.4. 1.2)

Premature atrial contractions (PACs) arise from ectopic
foci within the atria. PACs are very common and are seen
in patients of all ages in the absence of heart disease. ECG
features include an abnormal P wave (P') that occurs earlier than the next expected sinus P wave. The P' has a different morphology than the preceding sinus P waves, and
the interval to the next sinus P wave following a PAC is
longer but less than fully compensatory when compared to
Multifocal atrial tachycardia (MAT), otherwise known

as "wandering atrial pacemaker," is an irregular dysrhythmia caused by multiple atrial ectopic foci. ECG
characteristics include P waves with three or more differ-
FlG.
2-9.
normal (Fig. 2-9). The P' may be difficult to identiff if it

occurs early enough to be obscured by the preceding T
wave. If the PAC occurs too early during the absolute
refractory period of the AV node, it will not be conducted
Premature atrial contractions.
106 /
ElmnctNcy Mr,orcrNr: Tsn Conn CunrucuLUM
TABLE 2-27. Treatment of premature atrial contractions
seconds, this specialized tissue can fire, initiating ajunc-
tional escape beat.
Treat underlying cause

Discontinue medications (digitalis)
Avoid stimulants (caffeine, tobacco)
Decrease fatigue and stress
If
the sinus node continues to fail to

if the sinus initiated impulse is
blocked from reaching the AV node, repeated impulses
from the tissue surrounding the AV can establish ajunctional rhythm. Junctional rhythms are usually regular and
between 40 and 60 bpm; however, accelerated junctional
rhythms (60 to 100 bpm), and junctional tachycardias
(>100 bpm) can occur. P waves may be inverted (retrograde) in leads II, III, and aVF, and may precede, follow,
or be obscured by the QRS complex. The PR interval is
often shorter than the normal preceding PR interval. The
QRS complexes are usually normal (Fig. 2-10).
Junctional rhythms may occur with severe bradycargenerate an impulse, or
or "blocke(" exhibiting a P' with a QRS. The majority of

PACs are conducted normally through the AV node, resulting in a normal-appearing QRS complex.
Multiple etiologies include fatigue, alcohol, tobacco,
caffeine, COPD, and emotional stress. Digitalis toxicity is
a potential cause and if seen in this setting, the PACs may
be a precursor to SVT. Myocardial ischemia/infarction
and distention of the atria as seen with CHF are also

potential causes.
Clinical effects are usually minimal. Some patients

may note the sensation of a "skipped beat" as a result of
increased ventricular filling after the PAC. PACs have
been shown to precipitate SVI atrial flutter, and atrial
fibrillation.
Treatment of PACs is directed toward eliminating any
underlying etiology (discontinuing drugs, avoiding stimulants). If the PACs trigger sustained tachycardias, antidysrhythmics such as quinidine, procainamide, and betablockers are sometimes used (Table 2-27).
Junctional Rhythms
dias, AV blocks, CHF, myocarditis, hypokalemia, and

digitalis toxicity. Accelerated junctional rhythms are seen
with myocardial ischemia/infarction, rheumatic heart disease, and digitalis toxicity.
Clinical manifestations may include CHF and worsening of ischemic symptoms, particularly if the ventricular
response is slow. Treatment may not be necessary in the
stable patient. If digitalis toxicity is suspected, it should
be treated with Fab antibodies. Atropine may be useful to
increase the discharges from the sinus node in an effort to
initiate a sinus rhythm. Transcutaneous pacing should be
standing by
in the rare event of
severe decomposition
(Table 2-28).
Premature Junctional Contractions
While the conducting tissues surrounding the AV node

and bundle of His above the bifurcation can serve apace-
maker for myocardial contraction, the sinus node normally serves this function. In situations when no impulse
from the sinus node reaches the AV node for 1.0 to 1.5
Premature junctional contractions (PJCs) occur when an

ectopic pacemaker around the AV node initiates the
impulse for ventricular depolarization. ECG findings
include a P' wave of differing morphology than the sinus P

wave (as with junctional rhyhms, the P' may be retrograde
FlG. 2-10. Junctional rhythm.
TABLE 2-28. Treatment of junctional rhythm

May not be needed in stable patient
lf bradycardic and symptomatic
Atropine 0.5-1.0 mg lV repeat q 5 min to total of 3.0 mg
Pacemaker
lf digitalis toxicity is suspected
Digitalis-specific (Fab) antibodies
Cenuovesculq-n DrsonorRs
FlG.
707
2-11. Premature junctional contractions.
TABLE 2-29. Treatment of premature junctional complexes

Generally asymptomatic
Treatment is directed at alleviating precipitating causes (CHF, Ml, digitalis toxicity)
and occur anytime in relation to the QRS complex); a QRS

complex that is earlier than expected and usually of normal
configuration; a shortened P'R interval; and a fully compensatory pause before the next sinus beat (Fig. 2-l 1).
PJCs are rare in an undiseased heart. Etiologies include
myocardial ischemia/infarction, CHE, and digitalis toxic-
ity. PJCs are generally asymptomatic. Treatment
is
directed at correcting any underlying causes and continued observation for the appearance of other dysrhythmias
(Table 2-29).
Ventriculsr Fibrillation
(2. 4. 1.
5)
Ventricular fibrillation (VF) results from multiple

areas within the ventricles spontaneously depolarizing
and contracting. There is no organized ventricular depolaizalion, hence no effective contraction occurs. The
ventricle appears to quiver and produces no cardiac out-
put. The ECG displays an erratic baseline without

defined P waves, QRS complexes, or T waves (Fig.
2-12). The amplitude of the baseline may vary from very
coarse deflections to an almost flat line that can be mistaken for asystole.
VF may occur without warning (sudden death) with or
without associated acute myocardial infarction. VF may
FlG.
also be the result of trauma, hypothermia, drug toxicity
or electrolyte
abnormalities. Iatrogenic causes include direct myocardial stimulation during transvenous pacemaker or central
line placement. Furthermore, unsynchronized cardioversion of a tachydysrhythmia is also a potential iatrogenic
(digitalis, quinidine), electric shocks,
cause.
Clinically, since there is no cardiac output, the patient

be without pulse or blood pressure. The patient will
will
be apneic; however, early in the course of the dysrhythmia, ineffective agonal respirations may be present.
If a defibrillator is not immediately available, airway
control and support (ideally through endotracheal intubation and bag-valve-mask), cardiopulmonary resuscitation
(CPR), and intravenous access should be initiated. The
treatment of choice for VF is immediate defibrillation.
The initial three defibrillations should be delivered at 200
J,200 Io 300 J, and 360 J. Ifthe patient does not respond
to the initial defibrillation, the above resuscitative measures should be started and medications given, keeping in
mind that the major resuscitative medications can be

administered both intravenously and endotracheally.
After each medication administration, an attempt to circulate the drug should be made through continued CPR
for 30 to 60 seconds. At this point, the patient should be
2-12. Ventricular f ibrillation.
108
EnrnncrucyMrorcrNn: THr Conn CunnrculuM

TABLE 2-3O. Treatment of ventricular fibrillation
lf defibrillator immediately available
lmmediate defibrillations (200 J, 200-300 J, 360 J)
Airway control-endotracheal intubation
Support ventilations-bag-valve-mask
Cardiopulmonary resuscitation
lnitiate intravenous access (if access not immediately available, several resuscitative drugs can be
delivered via the endotracheal tube)
Epinephrine 1.0 mg lV (may repeat q 5 min as long as the dysrhythmia persists); some authors
recommend intermediate dose (3.0 mg) or high dose (b.0 mg)
After every medication administration, circulate drug via CPR and repeat defibrillations at 360 J
Lidocaine 1.5 mg/kg bolus (may repeat at 0.75 mg/kg bolus q 5 min to total dose of 3.0 mg/kg)
Bretylium 5 mg/kg bolus (after 5 minutes may repeat at 10 mg/kg boluses q 5-30 min to total-ol
35 mg/kg)
Magnesium sulfate 1.0 lV
Procainamide 20-30 mg/min (to total dose of 17 mg/kg)
Consider termination of resuscitative efforts
if VF is persistent, repeated defibrillations at 360 J should be delivered. The first drug that
should be administered is epinephrine, which makes VF
more susceptible to defibrillation. Following epinephrine,
reassessed, and
antidysrhythmic agents such as lidocaine, bretylium, procainamide, and magnesium may all be utilized per the
American Heart Association's Advanced Cardiac Life
Support guidelines (Table 2-30). Sodium bicarbonate

may be used to alleviate the acidosis that inevitably
occurs with VF. However, since the acidosis is a result of
metabolic by-products of hypoxic lactic acidosis, it is recommended that acidosis first be treated by hyperventilation and that sodium bicarbonate be utilized with caution.
While defibrillation is effective in some cases of VE, particularly if delivered early, VF is often a preterminal
rhythm, and the decision concerning when to terminate
resuscitative measures will need to be addressed on an
individual basis.
Ventricu
lar
Tachy
cardia
(2. 4. 1. 6)
Ventricular tachycardia (VT) occurs when there
are
three or more consecutive beats from an ectopic ventricular focus firing at arale greater than 100 bpm. The ECG
FlG.
will typically
display wide QRS complexes; a usually

regular ventricular rate between 100 and 220 bpm; ST
segments and T waves of opposite polarity to the QRS;
and a usually constant QRS axis (Fig. 2-13). Because
there is often AV dissociation, the sinus node may still be
firing and depolarizing the atria. Therefore, a P wave can
sometimes be seen between the QRS complexes; however, there will not be a fixed relationship between the P
waves and the QRS complex.
VT is rarely seen in the setting of a normal heart. VT

most commonly occurs in the presence of myocardial
ischemia and/or infarction. Other causes include mitral
valve prolapse, drugs (quinidine,
procainamide),
hypoxia, alkalosis, electrolye abnormalities, and cardiomyopathy.
VT is often difficult to distinguish from an SVT with

aberrant conduction, and clinical presentation is of little
help since both can present with similar symptomatology.
A QRS width of greater than 0.14 seconds suggests VT;
however, it is not absolute. It is generally thought best to
assume that all wide complex tachycardias are VT. It
would be uncommon for VT treatment to cause harm to
the patient with SVT. In the stable patient, vagal maneuvers or adenosine administration are felt to be safe and
may occasionally demonstrate an underlying SVT.
2-13. Ventricular tachycardia.
Cenotovescur-qn DrsoRnnRs
109
TABLE 2-31. Treatment of ventricular tachycardia

lf pulseless, treat as VF (Table 2-30)
lf a pulse is present but the patient is hemodynamically unstable
Synchronized cardioversion 100 J (if unsuccessful, reattempt at progressively higher energy levels)
lf a pulse is present, and the patient is hemodynamically stable
Lidocaine 1.5 mg/kg bolus (may repeat at 0.75 mg/kg bolus q 5 min to total dose of 3.0 mg/kg);
if successful, may begin continuous infusion ol 2.O-4.O mg/min
Bretylium 5 mg/kg in 50 cc NS run in over 8-1 0 min; repeat dose of 5-1 0 mg/kg may be given
in 10-30 min; if successful, may begin continuous infusion of 2.0 mg/min
Procainamide 20-30 mg/min (to total dose of 17 mg/kg); il successful, may begin continuous
infusion of 1.0-4.0 mg/min
The clinical manifestations of VT are varied and the

treatment is dependent on the clinical presentation.
Polymorphic Vl or VT presenting without a pulse, is
treated as VF. If a pulse is present but the patient
exhibits significant symptoms or hemodynamic compromise, synchronized cardioversion is the treatment of
choice. An energy level of 100 J is generally effective.
As with all cardioversion attempts, if the patient's condition permits, administration of sedation andlor analgesia is desirable. If the patient is hemodynamically
stable, then medication administration is indicated.
Lidocaine is the first agent of choice followed by
bretylium and procainamide (Table 2-31). Patients who
survive episodes of VT or VF should be evaluated as to
the potential need for automatic implantable cardio-
defibrillators (AICDs).
Ventricular Ectopy
(2, 4, 1, 7)
Premature ventricular contractions (PVCs) may arise

from ectopic foci located in either ventricle, which cause
depolarization ofthe ventricle prior to the next expected
sinus beat. Since the depolarization does
not
occur
through normal pathways, the resulting QRS complex is

often bizarre in appearance and abnormally widened
(>0.12 seconds). Another ECG characteristic is a missing
or abnormal P wave. The next sinus P wave is usually hidden within the PVC's QRS complex; however, occasionally the PVC causes retrograde depolarization of the atria
FlG.
resulting in an abnormal P wave (Fig. 2-l4). Most PVCs

are associated with a fully compensatory pause before the
next sinus beat; however, PVCs may occur between sinus

beats without associated pauses (interpolated PVC).
Since PVCs may arise from separate foci, the resulting
QRS complexes are of differing morphologies (multifocal PVCs). Due to the prematurity of the PVC, the ventricular rhythm is usually irregular. PVCs may occur
alone, in pairs (couplets), or in groups of three or more
(VT). Certain nomenclature has been developed for

PVCs occurring in regular patterns after every sinus beat
(bigeminy), after every two sinus beats (trigeminy), and
after every three sinus beats (quadrageminy, i.e., every
fourth beat is a PVC).
PVCs are common and often occur in the absence of
heart disease. Common causes of PVCs include myocardial ischemia/infarction, hypoxemia, CHF, drugs (stimu-
lants, digoxin), and electrolyte abnormalities. Patients

experiencing PVCs may be asymptomatic or may complain of palpitations.
The need to treat PVCs is based on multiple factors
such as their frequency, timing, number of ectopic foci,
and the clinical condition ofthe patient. In the setting of
acute myocardial ischemia or infarction, the goal should
be to treat the underlying ischemia and not simply to suppress the PVCs.
PVCs seen with ischemia may represent potential

instability and be a precursor ofVT orVF. If the PVCs do
not resolve with measures to restore myocardial perfusion, treatment may be indicated. PVCs that occur during
2-14. Premature ventricular contractions.
110
EnrRcrNcyMnorcrNr: Tnn Conn Cunruculuu

TABLE 2-32. Treatment of premature ventricular contractions
Often do not require treatment
lf treatment is indicated, first priority is to restore adequate oxygenation and perfusion
Supplemental oxygen
Support blood pressure
lf significantly bradycardic, increase heart rate (pacemaker, atropine)
Lidocaine 1.5 mg/kg bolus (may repeat at 0.75 mg/kg bolus
q 5 min to total dose of 3.0 mg/kg); if successful, may begin continuous infusion ol 2.04.0 mg/min
Procainamide 20-30 mg/min (to total dose of 17 mg/kg); if successful, may begin continuous inf-usion
of 1.0-4.0 mg/min
Bretylium 5 mg/kg in 50 cc NS run in over 8-10 min; repeat dose of 5-10 mg/kg may be given
in 10-30 min; if successful, may begin continuous infusion of 2.0 mg/min
or immediately after the T wave (ventricular repolarization) carry a risk of precipitating VT or VF (R on T phenomena). If treatment is indicated, lidocaine is the first
drug of choice; if unsuccessful, procainamide or
bretylium may be utilized (Table 2-32).
Pulseless Electrical Activity
Pulseless electrical activity (PEA), otherwise known
(EMD), occurs in settings other than VT or VF where there is electrical activity noted on the cardiac monitor or ECG without an associated palpable pulse. The appearance on the ECG may
range from normal appearing complexes to chaotic,
bizarre complexes with no uniformity.
In the setting of cardiac arrest associated with acute
myocardial infarction, PEA often represents profound
dysfunction of the myocardium ("pump failure") and is
as electromechanical dissociation
generally resistant to treatment. Potentially treatable

causes of PEA include tension pneumothorax, hypovolemia, hypoxemia, severe acidosis, cardiac tamponade,
ventricular rupture, drug overdoses [tricyclic antidepressants (TCAs), digoxin, calcium channel blockers], pulmonary emboli, hypothermia, and hyperkalemia.
Treatment is directed at the identification and alleviation of the underlying causes. Epinephrine and atropine (if the rate is less than 60 bpm) are also utilized
(Table 2-33).
Asystole
Ventricular asystole occurs when there is an absence of
ventricular electrical activity. Since no electrical activity
TABLE 2-33. Treatment of pulseless electrical activity

Search for and correct underlying causes
Epinephrine 1.0 mg lV (may repeat q 5 min if dysrhythmia
persists)
lf rate is < 60/min
Atropine 0.5-1.0 mg lV (may repeat to total dose of
3.0 mg)
is present, ventricular depolarization cannot occur, and

thus there is no ventricular contraction or cardiac output.
The ECG generally demonstrates a "flat line" (Fig.
2-15), although occasionally P waves or wide, irregular
complexes (agonal beats) may occur. Asystole should be
documented in at least two different leads because fine
VF may be mistakenly identified as asystole. Asystole is
the most common dysrhythmia seen in patients sustaining
cardiac arrest of greater than 10 minutes.
Etiologies of asystole include hypothermia, hypoxemia, severe acidosis, hypo/hyperkalemia, electrical

shocks, and drug overdoses. The patient is unresponsive
and pulseless.
Treatment of asystole consists of addressing potential
treatable causes. Additionally, transcutaneous pacing,
epinephrine, and atropine are utilized. However, it should
be noted that asystole is a preterminal rhythm that rarely
responds to treatment. Cessation of resuscitative measures should be considered (Table 2-34).
QTI nterval
Sy n dro m
(2: 4. 1.
8)
Torsades de Pointes
Torsades de pointes is an atypical form of VT in

which the QRS axis appears to be constantly changing.
The ECG generally demonstrates a rate of 200 to 240
bpm with the QRS axis alternating between positive and
negative in the same lead. Torsades de pointes usually
occurs in short bursts (<15 seconds). The QT interval of
the beats preceding torsades de pointes is usually prolonged.
Torsades de pointes is felt to be secondary to a triggered automaticity mechanism. Drugs that may precipi-
tate torsades de pointes are fype IA antidysrhythmics

(quinidine, procainamide, disopyramide), TCAs, phenothiazines, and organophosphates. Electrolyte imbal-
ances such as hypomagnesemia, hypokalemia, and

hypocalcemia may also contribute to the onset of torsades
de pointes. Other etiologic factors are myocardial ischemia/infarction, hypothyroidism, myocarditis, rnitral valve
prolapse, left ventricular failure, and severe bradycardias.
Cenorovescur-q-n Drsonosns
/ lll
FlG.2-15. Asystole.
TABLE 2-34. Treatment of asystole

ldentify and treat potential correctable causes
Transcutaneous pacemaker
Epinephrine 1.0 mg lV (may repeat q 5 min if dysrhythmia persists)
Atropine 0.5-1.0 mg lV (may repeat to total dose of 3.0 mg)
Consider termination of resuscitative measures
Initial treatment consists of removing or alleviating

underlying causes. Accelerating the heart rate to a rate
greater than the torsades de pointes (overdrive pacing) is
the most effective treatment. Magnesium sulfate has also
been demonstrated to be effective (Table 2-35).
Conduction Blocks (2.4.2)

Sick Sinus Syndrome (2.4,2.2)
Sick sinus syndrome (SSS), also known as tachy-brady
syndrome, is a clinical entity that involves both intermittent tachydysrhyhmias and bradydysrhythmias. Common tachydysrhythmias include atrial fibrillation, atrial
flutter, reentrant SVTs, and junctional tachycardias. The
bradydysrhythmias commonly seen include severe sinus
bradycardias, and sinus and AV blocks.
The etiology of SSS includes rheumatologic diseases
(e.g., sarcoidosis), rheumatic heart disease, ischemia,
myocarditis, pericarditis, cardiomyopathy, and cardiac
surgery. A variety of medications (digoxin, quinidine)
and medical conditions (hyperthyroidism, electrolyte

imbalances) can potentiate the tachy- or bradydysrhythmlas.
Symptoms such as palpitations, syncope, dyspnea, and
chest discomfort may be precipitated by the dysrhythmias. As the dysrhythmias are intermittent, continuous
cardiac monitoring either in the inpatient or outpatient
TABLE 2-35. Treatment of torsades de pointes

Alleviate underlying causes
Overdrive pacing
Magnesium 1.0 g lV
setting may be necessary to make the diagnosis. Treatment is directed at rhythm control. Patients often require
pacemaker placement to prevent untoward sequelae from
the bradydysrhythmias. It is recommended that pace-
maker placement be considered prior to treatment of

tachydysrhythmias as the treatment may exacerbate the
bradycardias.
Atrioventric ular
B lo
ks
(2. 4. 2.
3)
Atrioventricular (AV) blocks are divided into first,

second, and third degree. The block may occur in the AV
node itself or may be infranodal. A first-degree AV

block represents a delay in AV conduction; however, all
impulses are conducted. A second-degree AV block is
characterized by an intermittent lack of conduction
through the AV node and is further divided into Mobitz
I and II AV blocks. A third-degree AV block represents
complete interruption of AV conduction (AV dissocia-
tion).
First-Degree AV Block
With
a first-degree AV block, there is a delay in normal
AV conduction. ECG characteristics of first-degree AV

block include a prolonged PR interval (>0.20 seconds);
one-to-one conduction of P waves to QRS complexes;
usually a regular rhythm; and normal-appearing QRS
complexes (Fig. 2-16). The PR interval is usually constant but may be variable.
First-degree AV blocks may be the result of myocardial
infarction, myocarditis, or drug effects (digitalis). They

may occur in normal individuals and are not associated
with increased mortality.
712 /
EurncnNcy MrtrcrNn: THn Conn CunrucuLUM
FlG.2-16. First-degree AV block.
TABLE 2-36. Treatment of first-degree AV block

No treatment needed, except identification and correction of precipitating
etiology
The patient is typically asymptomatic. There is usually

no need to initiate treatment other than correcting underlying causes (Table 2-36).
Second-Degree AV Block, Mobitz
It is often difficult to initially
differentiate
a 2:l
Wenckebach from a second-degree AV block, Mobitz

II. However, Wenckebach's is more commonly associ-
Second-degree AV block, Mobitz I, also known as

Wenckebach's disease, is a condition where there is
progressive delay in AV conduction until there is complete block of the sinus impulse. The ECG demonstrates a progressively lengthening PR interval until a P
wave is seen without a corresponding QRS complex.
The P waves and QRS complexes are usually of normal
morphology. The R-R intervals shorten prior
interval lessens and the pattern is repeated (Fig.2-17).

Wenckebach's may occur in patterns such as 4:3, 3:2, or
2:1. The pulse will be irregular unless the pattern is 2:1.
to the
dropped beat. Once the complete block occurs, the PR
ated with a normal QRS length than is a Mobitz
II
block.
Wenckebach's disease is often transient and intermittent. Common causes include acute myocardial infarc-
tion, cardiac surgery, and myocarditis. Drugs such as dig-
italis, verapamil, and propranolol can also produce a

second-degree Mobitz I AV block.
Patients are usually asymptomatic and do not require
treatment. If there is associated symptomatic bradycardia, atropine or pacing may be utilized (Table 2-37).
FlG.2-17. Second-degree AV block, Mobitz I (Wenckebach).
TABLE 2-37. Treatment of second-degree AV block, Mobitz I (Wenckebach)

Generally no treatment needed; consider discontinuing drugs that may have initiated the block
lf bradcycardic and symptomatic
Pacemaker
CenorovRscur-cR DrsoRDrRs
773
FlG.2-18. Second-degree AV block, Mobitz ll.

TABLE 2-38. Treatment of second-degree AV block, Mobitz ll
I
ncrease ventricular rate

Pacemaker
Second-Degree AV Block, Mobitz
II
Mobitz II AV blocks occur infranodally, usually at the

bundle branches and less commonly at the bundle of His.
ECG characteristics include a normal P wave with more
P waves than QRS complexes; a PR interval that may be
prolonged from normal but remains constant; and an
irregular ventricular rate associated with a regular atrial
rate (Fig. 2-18). Mobitz II AV blocks are usually seen in
association with bundle branch or fascicular blocks;
therefore, the QRS complexes are usually wide.
Mobitz II AV block generally represents structural
damage to the conducting system below the AV node.
There is a potential for the block to progress to complete
heart block, particularly in the setting of myocardial
ischemia/infarction.
Treatment involves increasing the ventricular rate with
either atropine of pacing. Since patients with Mobitz II
AV blocks can precipitously decompensate, it is advisable to have transcutaneous pacing pads in place even
with the stable patient (Table 2-38).
Third-Degree AV Block
In third-degree AV block there is no AV conduction.

The block may occur at the AV node, the bundle of His,
or the bundle branches. Since no atrial impulses reach the

ventricles, the ventricular response is controlled by a ventricular ectopic focus. ECG findings include a ventricular
response that is slower than the atrial rate and usually
regular; an atrial rate that is typically normal with normal
P waves; and since the atria and ventricles are being

depolarized at different rates from different foci, the PR
interval is variable (Fig. 2-19). If the ventricular pacemaker is located near the junction, the rhythm is typically
between 40 and 60 bpm and may have a normal QRS configuration. If the ectopic pacemaker is located lower in
the ventricles, the intrinsic rate will more likely be slower
<40 bpm) and be associated with widened QRS complexes.
Third-degree AV blocks occurring at the AV node may

result from acute inferior myocardial infarction, structural damage to the AV node, and drug toxicities (digi-
talis, propranolol). Infranodal blocks are most commonly

associated with acute anterior myocardial infarction.
Clinical manifestations depend on the level of the
block and the resulting bradycardia. Patients with blocks
occurring at the AV node may initially present with stable
vital signs secondary to the junctional escape rhythm,
which may maintain perfusion. These patients should
have transcutaneous pacemakers at the bedside as they
may decompensate, particularly in the setting of ongoing
FlG.2-19. Third-degree AV
block.
ll4 /
ErrrencrNcy MnlrcrNr: Tnn, Conn CunrucuLUM
TABLE 2-39. Treatment of third-degree AV block

I
ncrease ventricular rate

Pacemaker
Atropine 0.5-1.0 mg lV (may repeat to total dose of
3.0 mg)
dysrhythmias may demonstrate little to no clinical effects

or may present with lifethreatening symptoms. Understanding of potential causes as well as the ability to properly identifli and treat cardiac dysrhythmias are essential
skills for all emergency physicians.
SELECTED READING
ischemia/infarction. Patients presenting with infranodal
blocks generally necessitate emergent pacing as the
intrinsic rate of their ectopic pacemaker is generally too
slow to maintain perfusion. Atropine and catecholamines
may be utilized as needed; however, pacing is preferred in
the unstable patient. All patients with third-degree AV
blocks should receive an evaluation by a cardiologist to
determine the need for a permanent pacemaker (Table
2-3e).
Bundle Branch Blocks (2.4.2.4)

Bundle branch blocks can occur in any of the three
major ventricular conduction pathways: the right bundle
branch (RBB), the left anterior superior fascicle (LASF),
or the left posterior inferior fascicle (LPIF). The block
may involve one (unifascicular), two (bifascicular), or all
three (trifascicular) pathways.
ECG characteristics of right bundle branch blocks
include increased QRS width (>0.12 seconds), RSR' in
lead Vr, and wide S waves in leads I, V5, and Vo. Blocks
involving the LASF demonstrate QRS axis of<-45
degrees, R wave in lead I greater than that in leads II or
III; a normal QRS width; deep S waves in the inferior
leads; and a qR complex in lead AVL. LPIF blocks pre-
with a normal QRS duration; QRS axis
>110
degrees; deep S and small R in lead I; R wave in lead III
greater than that in lead II; and a qR complex in lead III.
sent
Left bundle branch blocks (LBBB) have the following

ECG findings: a wide QRS (>0.12 seconds); large R
waves in leads I, aVL, V5, and Vo; small r and deep S
waves in leads II, [I, aVF, Vr, and Vl] and no q waves in
leads I, aVF, Vs, and Vo.
Bundle branch blocks may be the result of ischemia,
myocarditis, surgery, cardiomyopathy, valvular disease,
and congenital conditions. Bifascicular and trifascicular
blocks are associated with severe heart disease. In the
presence of ongoing ischemia/infarction, patients with
these blocks are at increased risk of developing thirddegree heart block and should be treated with pacemakers.
Summary
Diseases of the cardiac conduction system, manifested
as cardiac dysrhythmias, are commonly encountered
clinical entities in the ED. Patients presenting with cardiac
Aghababian R\ ed,. Emergency management of cardiovascular disease.

Boston: Butterworth-Heinemann, 1994.
American Heart Association. Textbook of advanced cardiac life support.
Dallas: American Heart Association, 1994.
Gorgels AP, Vos MA, Smeets JL, Wellens HJ. Ventricular arrhyhmias in
heart failure. Am J Cardiol 1992;70(10):3743.
Li HG, Morillo CA, Zardini M, et al. Effect of adenosine or adenosine
triphosphate on antidromic tachycardia. Am J Cardiol 1994;74(4):
401404.
Lowenstein SR, Halperin BD, Reiter MJ. Paroxysmal supraventricular
tachycardias. J Emerg Med 1996;14(1):39-51.
McMurray J, RankinA. Treatment of heart failure and atrial fibrillation and
arrhythmias. Br Med J 1994;309(6969):1 63 1-l 635.
Roden DM. Risks and benefits of antiarrhythmic therapy. N Engl J Med
199 4;33 I (.12):7 85-7 9
l.
Singh BN. Choice and chance in drug therapy ofcardiac arrhyhmias: technique vs. drug-specific responses in evaluation of efficacy. Am J Cardiol
1993;72(16):It4-124
LA, Huang SK. Management of cardiac arrhythmias

with radiofrequency catheter ablation. Arch Intern Med 1995;155(2):
Wagshal AB, Pires
137-147.
Waldo AL. Wit AL. Mechanisms
341 (8854):1
of cardiac arrhythmias. Lancet 1993;
89-l 1 93.
DISEASES OF THE CIRCULATION, ACQUIRED

(2.s)
Arterial (2.5.1)
Acquired arterial disease includes a variety of conditions. Due to the smaller caliber of peripheral vessels,
their involvement tends to be more symptomatic than
major vessel disease. Although the greatest number of
conditions are related to atherosclerotic disease, problems due to inflammatory disease, spasm, thrombosis, or
emboli may also require urgent or emergent evaluation.
Atherosclerosis/Insufficiency
(2. 5. 1.
1)
Atherosclerotic arterial disease is a complex problem

due to a variety offactors, including tobacco use, genetic
predisposition, hypertension, hyperlipidemia, diabetes

mellitus, vascular flow characteristics, and aging. Arterial
lesions develop gradually, initially with intimal thickening, followed by macrophage foam cell migration and
phagocytosis of extracellular lipid, with subsequent intimal fibrosis and calcific deposition, and ultimately hemorrhage, hematoma and thrombus formation, with intimal
erosion and plaque fissure. The intra-intimal depositions
cause compromise of the lumen; initially, some compensatory dilatation can occur to maintain lumen size, but
fibrosis and stenosis eventually limit this capability.
Cenlrovescuran DrsoRorRs
it is possible for collateral vascularization to occur around small and some

medium-sized vessels, to augment the slowly decreasing
flow from the primary vessel. It most commonly affects
the vessels of the lower extremities, the aorta, and the
carotid and coronary vessels.
Because of its gradual progression,
Arterial Insufficiency
Lower extremity arterial insufficiency, or chronic arte-
rial occlusive disease (AOD), is present in approximately

15o/o to 20%o of patients over the age of 55. It may be
asymptomatic, present with symptoms including claudication and rest pain, or present as frank occlusion with
ischemia. The strongest risks for arterial occlusive disease are tobacco smoking, hypertension, diabetes mellitus, hypercholesterolemia, history of coronary artery dis-
ease (CAD) (especially dysrhythmias or valvular

disease), and family history of AOD. It is more corunon
in males, with a gender ratio of 2 to l0:1. This variability
may be due to higher incidences of male smokers, males

with CAD, and an increased likelihood of males to seek
medical attention for these disorders. Although the
majority of patients with arterial occlusive disease have a
stable chronic condition, approximately 20% to 25o/o of
these patients eventually develop acute arterial occlusion.
The main causes of acute occlusion are cardiogenic

thromboembolism, thrombosis of a previously compromised area, or arterial stasis from hypotension. In lowflow states, multiple organ systems, rather than an isolated extremity, are usually involved.
Patients with lower extremity AOD will generally present with exertional pain of the buttock, hip, thigh, or
calf. More proximal symptoms imply a more proximal
site of atherosclerosis.- Diagnosis of peripheral vascular
disease can be suspected based on a history of limb pain
with exertion in patients with known risk factors, particularly smoking tobacco.
It is essential that the examination of the patient
include a careful cardiovascular assessment: a cardiac
examination, palpation of all peripheral pulses, blood
pressure measurements in all extremities, and evaluation
for bruits in all major vessels. The ankle-brachial index
(ABI) should be obtained. This is an objective measurement comparing the brachial artery blood pressure to the
dorsalis pedis blood pressure. The ABI should normally
be slightly greater than 1 because of decreased vascular
compliance and the effects of gravity and the static fluid
column. An ABI of 0.9 or less should be considered evidence of arterial occlusive disease and an ABI 0.4 or less
should be considered limb-threatening ischemia, requiring early, aggressive intervention. Ifonly one arterial segment is diseased, the ABI is usually greater than 0.5, but
will decrease with increasing arterial involvement.
Patients with claudication usually have an ABI between
175
0.5 and 0.8. The location of disease can be determined by
checking systolic blood pressure with an inflatable blood

pressure cuffat the high thigh, above the knee, below the
knee, and at the ankle. A pressure gradient ofgreater than
20 mm Hg usually indicates the diseased segment. This
method is
of limited use in diabetic or renal
disease
patients with noncompressible vessels, and there may be

factitious elevation of blood pressure in patients who are
obese, if a small blood pressure cuff is used. The ABI is
also unhelpful in patients with severe vasoconstriction or
hypotension.
The affected limb should be compared with the contralateral limb, and to the other extremities. The overlying
skin should be examined for slowly healing wounds and
areas of obvious vascular stasis, ulceration, infection, or
gangrene. Dermal atrophy, with decreased hair and

slowly growing, thickened nail plates may be present.
Skin temperature may be decreased, increased, or nor-
mal compared to surrounding areas, depending on the

degrees of vascular compromise and compensatory
vasodilatation. Skin temperature will increase proximally,
but one should note whether the temperature change
occurs gradually or whether there is a sharp demarcation
of temperature change.
Skin color may be pale from lack of blood supply,
cyanotic, or have dependent rubor ifthe blood supply is
inadequate. In addition, color changes may occur from
venous distention, cellulitis, or hemosiderin deposition.
Claudication is a chronic manifestation of limb ischemia. It is defined as calf pain that occurs only when walking, and resolves'*'ithin l0 minutes of resting. Its prevalence is approximately 2Yo in patients over the age of 60
years. Patients with claudication may present to the ED
when the pain occurs with less exertion than previously,
or when the symptoms progress for a given amount of

exertion.
In many cases, patients with claudication can be managed conservatively. When treating patients with claudication, the primary goal is modification of the atherosclerosis risk factors, including cessation of smoking,
blood pressure, glucose and cholesterol control, and

gradual improvement of exercise tolerance, as with a
planned exercise walking program. In all patients with
peripheral vascular disease, vasoconstrictive medications
should be avoided whenever possible because of their
potential to compromise blood supply to the extremities.
The role of medications in the treatment of claudication is controversial. Before medical treatment for claudication should be considered, the patient needs to meet the
following criteria: (1) they cannot walk more than 500 m
without symptoms; (2) the symptoms are present and stable for at least 6 months; (3) any medications that might
worsen their claudication should be discontinued.
As with coronary artery disease, aspirin appears to be
of some benefit in slowing the progression of arterial disease in general, including peripheral disease, through its
116 /
EurRcnNcyMnorcrNr: THs CoRE Cunnrculuv
inhibition of thromboxane Az and its inhibition of prostacyclin, therefore inhibiting platelet activation and aggregation. The optimal dose of aspirin is still uncertain,
although, as in coronary artery disease, as little as 80 mg
may be effective. Based on current studies, use of aspirin
alone appears to be as effective as aspirin combined with
other antiplatelet agents. Other antiplatelet agents, such
as ticlopidine and clopidogrel, also inhibit platelet aggregation but can produce neutropenia and are much more
expensive than aspirin.
Medications affecting the microcirculation by improv-
ing blood viscosiry such as pentoxi$rlline, may help

improve patients exercise tolerance, although there is no
evidence that it prevents progression of the disease.
Antioxidants, such as vitamins E, C, and beta-carotene,
may slow atherosclerosis and may be of benefit in
patients with peripheral arterial occlusive disease.

Vasodilators, such as calcium channel blockers and
angiotensin-converting enzyme (ACE) inhibitors, have
not been shown to be ofany benefit, since vessels already
appear to vasodilate in order to compensate for atherosclerotic disease.
Limb{hreatening arterial insufficiency presents
as
ulceration or rest pain. In these cases, blood flow at rest

is insufficient to meet the basal tissue demands of the
limb. Ulceration occurs primarily in patients with diabetes mellitus, because neuropathy predisposes to minor
trauma to the foot or leg. The traumatized tissue heals
slowly due to poor tissue perfusion, which makes it susceptible to secondary infection. These patients must be
careful to wear well-fitting shoes at all times, use emollients to keep the skin well hydrated cut toenails carefully
to avoid ingrown toenails, and pad between the toes with
cotton or lamb's wool to prevent pressure ulcers. They
should inspect their feet and legs often for insignificant
injuries, since neuropathy may prevent their early detection. In these patients, systgmic anticoagulation may prevent thrombosis, although it does not appear to have any
effect on progression ofthe disease. Prostaglandin derivatives are being investigated for their antiplatelet effects
and effects on the microcirculation, although specific
agents are not yet available. Approximately 30o/o of
ischemic ulcers heal with careful, aggressive wound care,
including hyperbaric oxygen therapy.
Aneurysm (2.5.1,2)
Arterial aneurysms are outpouchings of the vessel

walls due to dilatation or endothelial disruption. They
may be classified by shape, etiology, location, or structure. True aneurysms are irreversible localized dilatations
(1.5 to 2 times normal size) of an intact vessel wall

caused by hemodynamic forces and focal weakness. False
aneurysms (or pseudoaneurysms) occur after disruption

of the vessel wall with containment of blood by sur-
rounding tissue, with formation of a fibrous sac. The

main risk of aortic and visceral aneurysms is rupture with
exsanguination. Peripheral aneurysms rarely rupture, but
are complicated by thrombosis or embolism with distal
ischemia.
Aortic/Iliac
Abdominal aortic aneurysms (AAAs) involve the subdiaphragmatic aorta, and affect approximately 2o/o of the
population. They are true aneurysms, since all three layers of the vessel are intact, and are characteized by a
fusiform shape. Although the majorify of patients with
AAA are asymptomatic, AAAs are the most common
type of aneurysm that cause patients to seek medical care.
Symptomatic AAAs result from dilatation and rupture,
compression of surrounding structures, thrombosis,
embolization, inflammation, infection, or erosion into
surrounding structures. Ruptured AAA is the l3th most
common cause of death in this country. Few patients with

ruptured AAA survive to reach the hospital; of those that
do, mortality is approximately 45Yoto 50o/o. Clearly, early
diagnosis and treatment are of the utmost importance in
decreasing mortality.
Anatomic Classification. MostAAAs (97%) are infrarenal, involving the segment of the aorta between the
takeoff of the renal arteries and the aortic bifurcation.
Suprarenal aneurysms, between the diaphragm and the
renal arteries, are rare; they are usually due to extension
of a thoracic aneurysm.
Etiology and Pathophysiology. The main risk factors
associated with the development of A AA are age over 60
years, smoking, male gender, chronic obstructive pulmonary disease, hypertension, atherosclerotic peripheral
vascular disease, and family history. While most

aneurysms are seen in the presence of other atherosclerotic disease, most patients with advanced peripheral atherosclerotic disease don t develop aneurysms.
The exact role that atherosclerosis plays in the etiology
of aneurysm formation is unclear. It is suspected that atherosclerotic deposits trigger endothelial remodeling, with
increases in collagenase and elastase activity. In AAAs,
elastin concentrations may be decreased by as much as
92o/o, with a compensatory increase in collagen. This
derangement of the elastin-to-collagen ratio may be a
predisposing factor. Abnormalities of either collagen or
elastin, as in Ehlers-Danlos or Marfan's syndrome, also
are more prone to aneurysmal changes. Animal studies
have suggested that deficiency of copper, which is a
cofactor in the cross-linking of elastin and collagen, is
also a predisposing factor.
Additional causes ofAAA include blunt trauma, infec-
tion, and inflammation. Significant blunt trauma can

result in intimal tears with false aneurysm formation.
Mycotic aneurysms (infections of the aorta) are due to
Cer,orovescur-{R DTsoRDERS
bacterial endocarditis with septic emboli, with staphylococcal or streptococcal organisms. The term inflammatory abdominal aortic aneurysm is used to describe those
aneurysms in which the aortic wall is unusually thickened, possibly due to an autoimmune reaction to atherosclerotic plaque, with extensive reactive fibrosis.
Initial Presentqtion Many AAAs are found incidentally on examination when patients are being evaluated
for other complaints. However, diagnosis of patients with
symptomatic, leaking, or rupturing abdominal aortic
aneurysms can often be a diagnostic challenge.
Presenting symptoms may include localized or diffirse
abdominal pain often radiating to the back, chest, or
flank, nausea, vomiting or frank hematemesis, syncope,

dizziness, neurologic deficits, claudication or limb ischemia, jaundice, and weight loss. Patients may also be
aware of a throbbing sensation in their abdomen, and of
the presence of a mass or fullness
The most important initial assessment is of hemody.
namic stability. If patients are tachycardiac, hypotensive,

or have a history of syncope, they should be treated emergently, with fluid resuscitation, central monitoring, type
and cross-match of packed red blood cells, and surgical
consultation.
In
addition
to eliciting the above
symptoms, the
patient's past medical history, medications, surgical history family history, and social history should be obtained
to assess for risk factors for AAA.
Vital signs should be checked immediately on presentation, and frequently thereafter if AAA is suspected.
Blood pressures should be checked in both arms and both
legs to look for differential pressures.
A complete cardiovascular examination should be performe4 looking specifically for signs of ischemia and
atherosclerosis. The major vessels should be auscultated
for the presence of bruits. A careful abdominal examination should be performe4 to determine the presence of a
pulsatile mass and to exclude other intraabdominal
pathology. When palpating the abdomen, it is important
to palpate the lateral aspects of the aorta; if the examiner's hand is placed directly anterior to the aorta, pressing it against the spinal column, there will be increased
transmission of aortic pulsations, and a normal aorta may
be falsely perceived to be an AAA. The renal arteries
arise from the aorta at the level of Ll, which is in the
transpyloric plane, and the aortic bifurcation occurs at the
L4 level, along a line drawn between the iliac crests,
slightly below the umbilicus. Therefore, suprarenal and
visceral aneurysms are not palpable. Obesity or hypotension may make it impossible to palpate a pulsatile abdominal mass, so failure to palpate a mass does not rule out
A.rd{.
The flanks should be examined for ecchymosis (Grey
Turner's sign), which is evidence of retroperitoneal hemorrhage. Rectal examination should be performed to help
rule out gastrointestinal hemorrhage as a cause of abdom-
777
inal pain and hypotension. A careful neurologic examination, especially of the extremities, should be performed to
look for deficits due to nerve ischemia or compression,
particularly in the femoral or sciatic distribution.
Dffirential Diagnosis. Because of the variety of presentations, misdiagnosis of ruptured or expanding AAA
is common. When abdominal or back pain with hypotension and a pulsatile abdominal mass are present, diagnosis is readily apparent. Unfortunately, a ruptured AAA
may mimic myocardial infarction, diverticulitis, renal

colic, musculoskeletal back pain, and gastrointestinal
hemorrhage. The first evidence of AAA may be newonset renal insufficiency or failure, limb ischemia, or
neurologic deficits. Patients with syncope may appear to
have a primarily cardiac or neurologic event, or may fall
or be involved in motor vehicle crashes, and present to
the ED with multiple trauma.
Ancillaryt Tests. Laboratory and radiologic evaluations
of unstable patients with symptoms highly suspicious for
ruptured AAA waste valuable time; those patients should
immediately be taken to the OR. However, if the diagnosis is uncertain or the patient is stable, diagnostic studies
may avoid unnecessary emergency surgery.
An electrocardiogram should be ordered to evaluate
for myocardial infarction, although a normal ECG does
not rule out MI. Hematocrit, platelet count, and coagulation studies should be obtaine{ and a white blood cell
count should be checked if infection is suspected. Blood
urea nitrogen
@U$
and serum creatinine should be
obtained to evaluate renal function. Serum electrolytes

and glucose can be evaluated as necessary
ifthe patient is
diabetic or is on medications that might affect these values. A urinalysis should be checked ifthe patient reports
flank pain, urgency, frequency or hematuria. The erythrocyte sedimentation rate (ESR) tends to be elevated in
patients with inflammatory aneurysms, although it is not
pathognomonic. Depending on the level of suspicion for
AAA, a type and screen or type and cross-match should
be obtained.
Ultrasound is very reliable in the diagnosis of AAA,

with accuracy approaching 100%.It is noninvasive, rapid,
and can readily be performed in the ED. It is best at determining aortic diameter; it is not useful in distinguishing
whether an aneurysm has ruptured.
Computed tomography (CT) can accurately determine
the presence of an aneurysm, its extent, the presence of
thrombus, and whether the aneurysm has ruptured. CT
can also evaluate fibrotic adherence to adjacent structures
or fistula formation. Spiral CT further enhances the ability of CT to identify branch vessel involvement; although
image quality is improved, image processing takes more

time than conventional CT. The main disadvantages of
CT include inaccessibility of the patient, and allergy to or
nephrotoxicity from contrast medium.
Angiography is useful for the preoperative evaluation
of aortic diameter in AAA, although mural thrombi may
118 /
Err,rencnuctr
MerrcINr: Tns Conn CunrucuLUM
lead the examiner to underestimate lumen size. It also

requires the use of radiopaque contrast medium, with its
inherent risks.
MRI may be used in the outpatient setting for evaluation of AAA. It is comparable to ultrasound and CT in
assessing aneurysmal diameter and better at assessing
branch vessel involvement, but lack of immediate availability limits its use in the emergent setting.
Abdominal films (flat, upright, cross-table lateral) are
of limited use in the diagnosis of AAA. Calcification of
the aorta wall can be identified in -600/o of patients with
AAA, but no other relevant information can be obtained
from plain films.

Treatment.
Management
of
abdominal
aortic
aneurysms in the ED depends on the case presentation.

Continuous cardiac and vital sign monitoring and pulse
oximetry should be initiated. Diagnostic studies should
be undertaken as outlined above.
Symptomatic patients, whether stable or unstable,
require aggressive preoperative management and monitoring. Two large bore IVs should be placed and blood
drawn for blood type, cross-matched for six units of

packed red blood cells, and baseline CBC, electrolytes,
renal function, and coagulation studies should
be
obtained. An arterial line should be placed for continuous
blood pressure monitoring, and an indwelling urinary

catheter should be placed to monitor urinary output, a
good indicator of tissue perfusion. Blood pressure needs
to be monitored carefully. If the patient is hypotensive,
fluids should be given to maintain a blood pressure of 90
to 100 mm Hg systolic, and mental status and urine output should be monitored. Vasoconstrictors should be
avoided" since they do not treat hypovolemia, and they
contribute to tissue ischemia and metabolic acidosis.
Hypertensive patients require blood pressure control with
short-acting agents such as labetalol hydrochloride, or a
combination of a beta-blocker and nitroprusside. The
goal is to decrease stress and shearing forces on the aortic lumen; rapid changes in blood pressure are more likely
to contribute to rupture.
Symptomatic stable patients may have a slowly leaking
aneurysm
or a ruptured aneurysm contained in
the
retroperitoneal space, but ifthese are not suspected" ultrasound or CT should be considered for definitive diagnosis. The patient should never be left unattended, since
the
rapid decompensation and death can occur
is
made,
As
as
the
diagnosis
ruptures.
soon
aneurysm
there should be emergent surgical consultation; any delay
in surgery greatly increases risk ofdeath. Ifthere is a high
suspicion of AAA, surgery should be consulted immediately, to decide whether operative management should
take place on the basis ofclinical diagnosis alone.
Incidentally found asymptomatic AAAs require
prompt diagnostic evaluation. If they are truly asymptomatic, these patients should be given surgical referral on
an urgent basis. Ultrasoun4 CT, or MRI may be obtained
on an outpatient basis to confirm the diagnosis and to
if
decide on the best therapeutic course. Aneurysms less

than 4 cm in diameter should be followed by serial imaging every 6 months. Asymptomatic aneurysms greater
than 4 cm in diameter may be treated operatively if the
patient is otherwise medically fit; coexisting conditions
such as cerebrovascular disease, and coronary artery disease
will
need to be addressed prior to aneurysm repair.
Contraindications to aneurysmectomy include expected

life span less than 2 years, and severe coronary, pulmonary, or renal insufficiency. Aneurysms greater than 6
cm in diameter are managed operatively in almost all
patients, unless contraindicated.
Visceral Aneurysms
Visceral aneurysms usually involve the splenic,

hepatic, or superior mesenteric artery. Although they are
uncommon, there is a small chance of rupture with exsan-
guination.
Splenic artery aneurysms are the most common visceral aneurysms and occur most commonly in females,
often in pregnancy due to increased splenic arteriovenous
shunting. Only 2% of splenic aneurysms rupture, but of
those, 950lo occur in pregnant females. Often these

aneurysms are asymptomatic but may present as left
upper quadrant pain. Occasionally an acute rupture may

be confused with an ectopic pregnancy. Diagnosis is
often as an incidental finding on ultrasound or MRI, but
angiography confirms the diagnosis. Symptomatic
aneurysms require immediate surgical management.

Asymptomatic aneurysms require surgical consultation.
Hepatic artery aneurysms can be a result of trauma,
infection, or atherosclerosis, and commonly affect elderly
males. Most hepatic artery aneurysms are not symptomatic; symptoms may include right upper quadrant or
epigastric pain, occasionally severe. Bruits or palpable
masses are rarely detectable. They may be detected incidentally with ultrasound or Cl but angiography is the
most reliable diagnostic test. Mortality is high for ruptured aneurysms and surgical resection should be performed on those who are operative candidates.
Superior mesenteric artery aneurysms are the least
common visceral aneurysms. The majority of these
aneurysms are infected aneurysms from bacterial endocarditis, in middle-aged males and females. Patients often
complain of intermittent upper abdominal pain and the
physical examination reveals a palpable pulsatile mass
approximately 50% of the time, so that it may be confused with AAA. Angiography confirms the diagnosis.
Treatment should include management of infection, with
surgical repair after the infection is eradicated.
Peripheral Arterial
Unlike aortic aneurysms, aneurysms of peripheral

arteries seldom rupture. Dilatation often results in mural
Cenorovescur.AR DTsoRDERS
thrombi, which can embolize or progress to thrombosis.
In addition, aneurysms tend to be multiple. Nearly onehalf of patients with bilateral popliteal aneurysms have
AAAs.
Upper Extremity Aneurysms
Upper extremity aneurysms are relatively rare. Localizedtrama is the most common cause. Aneurysms of the
subclavian artery are often due to postischemic dilatation
in patients with cervical rib or thoracic outlet syndrome.
Patients may present with chest, neck, and shoulder pain
from acute expansion. Emboli may cause distal ischemia.
Radial artery pseudoaneurysms are an uncommon complication of radial artery cannulation. Industrial workers
may rarely develop an ulnar artery aneurysm from

repeated trauma to the hypothenar eminence. Depending
on location, symptoms can include pain, coolness, paresthesias, and cyanosis ofthe fingers.
/ ll9
arteritis, such as endocarditis, syphilis, Lyme disease,
HI! toxoplasmosis, malaria, sickle cell

anemia, disseminated intravascular coagulopathy, thromboangiitis obliterans (Buerger's disease), protein C or S
deficiency, thrombotic thrombocytopenic purpura, neoplastic diseases (particularly renal cell or lung carcicytomegalovirus,
noma), lymphoma and myelodysplasia, allergic drug

reactions, amyloidosis, sarcoidosis, multiple sclerosis,
heavy metal poisoning, systemic lupus erythematosus,
and rheumatoid arthritis.
When a patient presents with symptoms suggestive
arteritis,
it is important to determine if
of
other organ sys-
tems are involved. The patient should be asked questions

regarding symptoms such as hemoptysis, dyspnea, chest
pain, and myalgias to evaluate pulmonary cardiac, and
muscle involvement. Any history of sickle cell disease,
connective tissue disease, and neoplasia should be

elicited.
There are no pathognomonic signs, symptoms, or diagnostic studies for arteritis, although the erythrocyte sedimentation rate will be often be elevated as a nonspecific
Lower Extremity Aneurysms
indicator of inflammation. Depending on the type of

arteritis, the organs involved" and the presenting symp-
Lower extremity aneurysms most commonly affect the

popliteal arteries, folloled by the femoral arteries. They
almost always occur in older males with advanced atherosclerotic disease. Expansion may compress adjacent
nerves, resulting in pain or paresthesias. Patients may
experience claudication, thrombosis, embolic events, or
toms, other studies may be necessary.
gangrene.
Physical Examination Depending
on
aneurysmal
location, palpation of local arterial enlargement is generally adequate for diagnosis. Systolic bruits or a palpable
thrill are often present.
Diagnostic Studies. Arteriography provides definitive
diagnosis of arterial aneurysms, although mural thrombus may reduce the apparent diameter of the lumen.
Ultrasound and CT scan can provide further useful informatlon.
Treatment. Acute thrombosis associated with severe

ischemia is an indication for immediate surgical treatment. Early operation is advised with recurrent peripheral
embolization. In asymptomatic patients, prompt surgical
follow-up is necessary.
Small
Vessel
Arteritis
Small vessel arteritis is often due to hypersensitivity,

and the patient will eventually require a workup to identify an antigenic trigger. It most commonly affects the
skin, which will show purpuric lesions, which are reddish
purple, slightly raised papules approximately I to 3 mm
in diameter. They are nonblanching and may be tender.
Lesions are more common
in gravity
dependent-areas
and at pressure points, and there may be ulceration, localized edema, or involvement of mucosal surfaces.
Behget s Disease
Behget's disease is a vasculitis involving both arterial
and venous vessels. It is characterized by recurrent

mucosal ulceration, ophthalmic, dermal, and vascular
involvement. This is most commonly manifested as
recurrent, superficial, or deep thrombophlebitis, although
caval occlusion and aneurysms, particularly

Arteritis (2.5.1,3)
The arteritides are abnormal reactions of the blood
vessels to inflammatory processes. Some of these
responses to inflammation include increased vascular
permeability, vessel weakening with aneurysm formation, and intimal proliferation with thrombosis. The
arteritis syndromes vary with pattern of organ involvement, severity, and associated conditions; there may be a
predilection for small, medium, or large vessels. It is

important to rule out other conditions that can simulate
of
the
femoral, popliteal, pulmonary, brachial, and ulnar arteries, also occur. There may be transmural necrosis of the
walls of large muscular arteries and degradation of elas-
tic fibers with scars and
inflammatory infiltration.
Pseudoaneurysm formation can also occur as can perforation of the vessel wall. Although surgical repair is often
necessary, this is often difficult due to the friable nature
of the vessels, which may result in further vessel injury
inflammation and scarring.
If
repair is necessary, it
should be done when the disease is not active, if possible.

Venous obstruction is often refractory to treatment; med-
120 /
EnmncsNcv MEotctNn:
Tss Conn CumrcuLUM
ical therapy using anticoagulation, corticosteroids, and

immunosuppressive agents may alleviate symptoms, but
it is not clear whether they slow the course of the disease.
Elevation and compression of affected extremities may
provide symptomatic relief.
after signs and symptoms have resolved and the ESR has
refurned to normal, usually over 6 to 24 months, depending on patient response.
Takayasu Arteritis
Temporal Arteritis
Takayasu arteritis is a chronic, nonspecific inflammamay

include the pulmonary and coronary arteries. This disorder is most common in young females and is character-
tion of the aorta and its main branches, which
Temporal arteritis is a granulomatous arteritis of

unknown etiology. It appears most frequently in Caucasians over the age of 50, with a female to male ratio of
2 to 3:1. The incidence appears to be highest in populations of Scandinavian decent. Headache is the most common symptom and may be accompanied by scalp tenderness, facial pain,jaw claudication, and./or visual changes.
The onset of temporal arteritis is often gradual with nonspecific constitutional signs and symptoms, including
malaise, fever, weight loss, and depression. The symptoms often wax and wane regardless of therapeutic intervention. If untreated, the most common complications are
blindness from ischemic optic neuritis and cerebrovascular accident.
Diagnosis of temporal arteritis depends on several fac-
tors. Clinically, temporal artery tenderness, nodulariry

thickening, or swelling may be noted, in some cases with
overlying erythema due to a local inflammatory response.
Initially the temporal pulse may be more prominent than

usual, but eventually decreases or is obliterated. Visual
deficits are usually a sign of advanced disease and may
be irreversible.
No laboratory studies are pathognomonic, although the
erlthrocyte sedimentation rate is markedly elevated, at

least 50 mm per hour, and is often greater than 100 mm
an hour. Other nonspecific findings include normochromic or slightly hypochromic anemia, mild elevation of liver function tests, particularly alkaline phosphatase, and elevated serum immunoglobulin G (IgG),
ized by constitutional symptoms, such as fever, weight

loss, fatigue, anorexia, night sweats, arthralgias, anemia,
and tenderness over affected superficial arteries. As the
with increasing inflammation and

fibrosis (the pulseless phase), the patient may develop
limb claudication, renovascular hypertension, cerebrovascular ischemia, visual changes, pulmonary hypertension
with hemoptysis, heart failure, angina, discrepancies in
blood pressure in different limbs, retinopathy, aortic
disease progresses
regurgitation, and aortic aneurysms.

Diagnosis is based on arteriography, which can show
vascular irregularities, including stenosis, aneurysms,
dilatation, and development of collateral circulation. If a
vascular biopsy is done, there may be mononuclear cell
infiltration, with skip lesions similar to those with temporal arteritis. Other diagnostic studies are similar to
those for temporal arteritis, including ESR, and hemoglobin or hematocrit to assess for anemia. Additional
studies include chest x-ray, electrocardiogram, and

echocardiogram if coronary or aortic involvement are
suspected. Treatment during the acute stage involves
high-dose steroids to decrease the amount of inflammation and subsequent fibrosis. In advanced cases valve
replacement and vascular replacement or bypass may be
necessary.
Emboli (2.5.1.4)
complement, and circulating immune complexes.
Definitive diagnosis is made by temporal artery

A segment of artery on the more symptomatic
be evaluated and a sufficient specimen,
should
side
approximately 3 to 5 cm, should be obtaine4 because of
the high incidence of skip lesions. If the biopsy is nega-
biopsy.
tive, the other side should be biopsied, since involvement

is bilateral approximately 90% of the time. Doppler flow
studies can be useful to determine optimal biopsy sites,
but are not sufficiently sensitive nor specific for diagnoSIS.
If temporal arteritis is suspected, the patient should
be
started on high-dose corticosteroid therapy immediately,

without waiting for a definitive diagnosis. This consists
of prednisone,40 to 60 mg a day in divided doses, which
should reduce the inflammatory response. The patient
should be referred to a rheumatologist for further evaluation and monitoring. Prednisone can be gradually tapered
Acute arterial ischemia is due to embolic or thrombotic

occlusion. Etiologies of emboli include atrial fibrillation,
valvular vegetations in endocarditis, intravenous foreign
bodies, such as particulate contaminants of intravenous
illicit drugs, iatrogenic foreign bodies, such as catheter
tips, septic emboli, or passage of a venous thrombus
through a patent septal defect into the arterial circulation.
Acute arterial occlusion can occur anywhere in the circulation, although compromise is noted primarily in areas
supplied by smaller caliber vessels or areas with little or
no collateral blood flow.
Acute limb ischemia is characterizedby the five P's:
pain, pallor, pulselessness, paresis, and paresthesias. The
limb distal to the occlusion is critically compromised
due to little or no collateral blood flow, and skeletal mus-
cle and nerve fibers are very sensitive to
ischemia.
Immediate diagnosis and intervention are necessary to
Caxuovescur-AR DTsoRDERS
prevent progression to gangrene with need for amputation.
Diagnostic methods used in limb ischemia depend on
patient presentation. Bidirectional
continuous-wave
Doppler ultrasonography can analyze blood flow through

a diseased vessel. Although duplex ultrasonography combines real-time B mode and pulsed Doppler ultrasound,
and provides excellent assessment of blood flow patterns
and mural plaques within the vessel, areas of hemorrhage
or intraluminal thrombus may be difficult to detect unless
color-coding of blood flow is used. In acute occlusion,
the gold standard is arteriography. In addition to diagramming the site of occlusion, arteriography will also
help assess the presence or absence of collateral flow

around the site of obstruction. The main disadvantages of
angiography are the risks of using radiopaque contrast,
such as renal compromise or allergic reactions, exposure
to radiation, and its invasiveness. Other diagnostic techniques include magnetic resonance angiography, and spiral CT with imagery construction in three-dimensions.
These techniques are noninvasive; however, availability
of these studies and of personnel to evaluate them may be
limited. In addition, these studies would both be compro-
mised in patients with metallic foreign bodies, such as

surgical clips from previous operations; MRI could not
be done in these patients, and artifact during tomography
might compromise visualization of nearby vessels.
Once the lesion has been identified" several options
may exist, depending on the duration and location of the
occlusion. The previous gold standard oftherapy was surgical intervention, including thrombectomy, angioplasty,
vascular bypass graft, or intravascular stents.
Currently, the use of thrombolytic agents for acute
occlusion has been undergoing greater study. Thrombolysis is less invasive than surgery, although it does carry
with it complications of major hemorrhage, and if unsuc-
cessful, delays time to definitive revascularization.

Thrombolysis appears to be most effective when the
catheter is placed within the thrombus, and therefore is
only indicated during angiography or intra- or postoperatively. Although catheter location is the major predictor of
success, other factors affecting its success include duration of thrombosis, and degree of underlying atherosclerosis.
Contraindications to thrombolysis include patients in
whom (1) the distal vessel continues to be severely compromised, due to atherosclerosis or other microvascular
disease; (2) there is irreversible distal ischemia; (3) there
is no future mobility; and (4) there exist absolute or relative contraindications to thrombolytic therapy. Absolute
contraindications to thrombolysis include cerebrovascular accident within the past 6 months or history of cerebral neoplasm, aneurysm, arteriovenous malformation or
hemorrhage, major gastrointestinal hemorrhage within
the past 3 months or active internal hemorrhage, major
surgery within the past 2 weeks, known bleeding diathe-
121
sis, puncture of a noncompressible vessel within the past
l0 days, pregnancy, severe persistent hypertension, and

renal insufficiency (serum creatinine >2.5 mg/dl). Relative contraindications include peptic ulcer disease, significant liver disease, hemorrhagic retinopathy, thrombocytopenia, septic thrombophlebitis, and surgery, trauma,
vascular puncture, or hemorrhage prior to the guidelines
given as absolute contraindications.
Once thrombolytic therapy has been performed" additional agents, such as aspirin and heparin may help
improve vessel patency. If no improvement occurs in the
initial
to 2 hours of infusion, repositioning of
the
catheter may be necessary, and if no reperfusion has

occurred after 12 to 18 hours, it is unlikely that the vessel can be revascularized.
The primary complications
of
thrombolysis are
intracranial hemorrhage, retroperitoneal hemorrhage, and

massive gastrointestinal hemorrhage. If any of these
occur, thrombolytic infusion should be stopped immediately, and e-aminocaproic acid (EACA), fresh frozen
plasma, cryoprecipitate, or platelets should be administered. The l-year survival rate of patients undergoing
thrombolysis is higher than that of patients undergoing
surgery. This is related to the greater stresses and poten-
tial complications of undergoing a surgical procedure

rather than being related to rates of limb salvage from
thrombectomy/angioplasty versus thrombolysis. When
comparing thrombolytic agents, tPA has the greatest rate
of initial patency, although at 30 days there is no difference in patency between patients treated with tPA or
urokinase, and there appears to be an increased incidence
of bleeding complications with tPA. New thrombolytics

are being developed that may increase the halflife of
plasminogen activators, as well as increasing their specificity. Other anticoagulants, such as hirudin and other
antiplatelet agents, are being developed, although at this
point the new antiplatelet agents have not been proven to
work better than aspirin.
Spasm (2.5.1.5)
Raynaud s Phenomenon
Raynaud's phenomenon
is a
relatively common
vasospastic disorder ofthe hands and feet. It affects 5%

to l0o/o of the general population and is more common in
females than in males. It is usually bilateral and is more
common in patients with connective tissue disorders or

other evidence of vascular reactivity. Raynaud's phenomenon can be primary or secondary, although most
cases are idiopathic. Patients with hyperviscosity syndromes, such as polycythemia vera or cryoglobulinemia,
and endocrine disorders, such as hypothyroidism, also
have a higher incidence of Raynaud's phenomenon. It is
manifested as episodes of blanching due to vasoconstric-
722 /
ErunncrNcy MnucrNn:
THr Conn CunnrculuM
tion, cyanosis during a period of deoxygenated blood

flow, and then reactive hyperemia. The majority of these
episodes last less than 15 minutes. Sixty percent of
patients with this condition have these triphasic color
changes; the remaining 40% may show only pallor,
cyanosis, or two ofthe three changes.
In approximately 20o/o of the cases, ulceration or tissue
necrosis may occur. The etiology of Raynaud's phenomenon is uncertain, although conditions such as cold temperatures, emotional stress, increased central sympathetic
tone, increased ctz-adrenergic receptors, and increased
responsiveness of vascular smooth muscle to sympathetic
mediators are all thought to be contributing factors. Circulating vasoconstrictors have not been proven to act as
factors in Raynaud's phenomenon in laboratory investigatlon.
orthostatic hypotension. Other agents, such as nicardopine or diltiazem, can also be used. Verapamil has
minimal peripheral activity, and therefore is not recommended in the treatment of Raynaud's phenomenon.
Nitroglycerin has also been used because of its effects
as a vasodilator, although it is necessary to use topical
ointment rather than systemic agents. In acute attacks
intraarterial nitroglycerin has been shown to be of some
benefit; however, side effects, such as headache and
hypotension, are generally pronounce4 and it cannot be
used in the outpatient setting. Sublingual nitroglycerin
has not been shown to be of any use. Prostaglandins,
prostacycline, aspirin, and dipyridamole have not consistently been proven to be of any benefit. Serotonin
antagonists are being investigated" but, again, resuits are
inconclusive. Reserpine and guanethidine, sympatholytic agents, have been used for many years as treat-
There are no pathognomonic tests for Raynaud's phenomenon, although CBC, platelet count, and ESR may
help to evaluate if there is a primary underlying hematologic or inflammatory disorder. The majority of patients
presenting to the ED with Raynaud's phenomenon report
onset of an episode after stress or exposure to cold temperatures, and symptoms may have resolved by the time
the patient is seen by the physician.
If the diagnosis of Raynaud's phenomenon is made,
medication or aggressive therapy is usually not required.
The most important factor is patient education to prevent
episodes of vasospasm. Patients should be taught to avoid
abrupt temperature changes, cold temperatures, and emo-
Buerger's Disease
tionally stressful situations, with advice to keep the hands

and feet warm through the use ofgloves and to keep core
temperature warm as well in order to avoid peripheral
vasoconstriction. Bathing the hands and feet in warm
water may help treat mild attacks. Smoking should be
Buerger's disease (thromboangiitis obliterans) is a

nonatherosclerotic, inflammatory vaso-occlusive disorder of small and medium vessels, possibly related to
tobacco smoking. It starts distally and progresses proxi-
avoided because it potentiates vasoconstriction, and sympathetic stimulants, such as caffeine, decongestants, and
mally. Fibrous and granulomatous changes in the vessel

walls can result in claudication, rest pain. and ultimately,
antihistamines, should also be avoided. In some cases

job-related trauma, such as use of laboratory tools, may
contribute to Raynaud's, and if this is the case, patients
may need to modify the use of these tools. If a patient
presents with moderate to severe Raynaud's phenomenon
with tissue ischemia, ulceration, or necrosis, wound care
to minimize tissue loss or secondary ischemia is neces-
acute ischemia.
sary, as are analgesics.
In the majority of cases nonsteroidal antiinflammatory agents can be used, although narcotics may be
required for more severe pain. Drug therapy has been
tried in patients with moderate to severe Raynaud's,
although only approximately half respond well to medical therapy. Calcium channel blockers have been used
due to their vasodilatory effects. Nifedipine is the calcium channel blocker of choice because it also has
antiplatelet activity and better peripheral vascular

effects than other calcium channel blockers. Patients
need to be warned of side effects of calcium channel
blockers, such as headache, light-headedness, and
ment for Raynaud's, with highly variable results. They

have considerable side effects, including depression,
peptic ulcer disease, orthostatic hypotension, diarrhea,
and impotence. Other treatments, such as plasmaphere-
sis, hyperbaric oxygen, terbutaline, steroids, alcohol,

and surgical sympathectomy, have all been used with
highly variable degrees of success and are not currently
recommended.
Thrombosis (2.5.1.6)
Diagnosis is made based on findings of limb ischemia,

particularly upper limb ischemia, in patients under the
age of 50, with no other risk factors for atherosclerosis
other than smoking. Although not needed for diagnosis,
arteriography can be performed, and will not show the
calcifications or irregular plaques one would see with
atherosclerotic vascular disease.
Cessation of smoking is the cornerstone of treatment.
Vasodilatory agents are not useful because of vascular
fibrosis. Antiplatelet agents may help prevent acute

occlusion, although they have little or no effect on disease
progression. Sympathectomy, vascular bypass grafts, or
amputation may be necessary in advanced stages of the
disease.
Aortic Dissection
(2. 5.
I.
7)
Aneurysms of the thoracic aorta are most common in

the descending aorta but may involve any segment.
Cenorovescut-A,R DTsoRDERS
723
Anatomic Classification
right intercostal space, bounding or water-hammer (Cor-
Classification is based on etiology, pathophysiology,

and probability of dissection. Aneurysms of the ascending aorta dilate the aortic annulus resulting in aortic
rigan's) pulses, left-sided heart failure, pistol-shot

femoral pulses, and capillary pulsations (euincke,s
pulse), may be present. If there is bronchial compression,
the patient may have wheezes or decreased breath
sounds. Patients with hoarseness due to recurrent laryn-
insufficiency, and can dissect proximally, occluding coro-
nary arteries. Aneurysms of the aortic arch have the

potential to occlude the subclavian and carotid arteries
with limb and cerebrovascular compromise. Descending
thoracic aneurysms originate distal to the left subclavian
artery, at or near the ligamentum arteriosum, and extend
distally. Thoracoabdominal aneurysms are rare, arising in
the descending thoracic aorta and extending to the
abdominal aorta, involving the visceral vessels.
Etiology and Pathophysiology
geal nerve involvement
will
have unilateral vocal cord
paresis or paralysis, which can be visualized by direct or
indirect laryngoscopy. The neurologic examination

should reveal any presence of ischemic or compressive
neuropathy.
The patient should also be examined for signs of Mar-
fan's syndrome, such as arachnodactyly, chest wall

deformities, kyphoscoliosis, joint hypermobility, and
lens subluxation, for signs of Ehlers-Danlos syndrome
such as joint hypermobility and bruising, and for signs of
AAA.
Most thoracic aortic aneurysms (TAAs) are atheroscle-
rotic. Other causes include progressive degenerative diseases, such as Marfan's syndrome or cystic medial necro-
sis and, rarely, syphilis. Ascending aortic aneurysms are

commonly due to medial degeneration as in Marfan's
syndrome. Those involving the aortic arch are usually
secondary to atherosclerosis, as are descending thoracic
aneurysms. Syphilitic aneurysms are now rare. As with
all aneurysms, TAAs tend to progress. According to the
law of LaPlace, at a given pressure wall tension increases
in direct proportion to its diameter. Therefore, in a weakened vessel, the greater the diameter, the greater the risk
of dissection.
Compressive symptoms may be caused by mediastinal
abscesses, or neoplasms. Pain associated with bony erosion may mimic
or other thoracic strictures, cysts,
cardiac, musculoskeletal, or neoplastic chest or back

pain. Hemoptysis can be caused by numerous upper and
lower airway disorders such as bronchitis, neoplasm, and
tuberculosis, as well as by nasopharyngeal bleeding or
aspirated hematemesis.
Aortic regurgitation is also due to rheumatic heart dissyphilitic aortitis. Left-sided heart failure can be
ease and
due to congenital or acquired cardiac disease.
Initial Presentation
Thoracic aortic aneurysms are often asymptomatic,
found incidentally on a routine chest radiograph. Symptoms are often related to size and location. Aneurysms of
the ascending aorta may involve the aortic valve or coronary arteries, resulting in dyspnea or chest pain. If the
subclavian artery is compromised, the patient may present with symptoms of cerebrovascular accident or limb
ischemia. Stretching of the recurrent laryngeal nerve may
cause hoarseness, and esophageal compression may
result in dysphagia. Descending TAAs may present with
back pain secondary to spinal erosion, or cough, dyspnea,
or hemoptysis from bronchial compression or erosion.
Patient history may reveal the gradual onset of compressive symptoms, as well as risk factors.
Physical signs in TAAs are more subtle than those of
AAAs. They are not palpable, so signs are related more
to size and location, and the effects on adjacent structures. All major vessels should be auscultated for bruits,
and blood pressures should be checked in all extremities.
Stigmata of aortic regurgitation, such as a widened arte-
rial pulse pressure, a high-pitched blowing decrescendo

diastolic murmur heard best at the third left or second
Ancillary
Tests
Radiographically, a widened superior mediastinum

(mediastinal width-to-chest width ratio > 0.25), tracheal
or esophageal deviation to the right, left mainstem
bronchus depression, and difficulty visualizing the aor-
tic knob or descending aortic outline are all suggestive,

although not pathognomonic, of TAA. In nonemergent
patients, CT or MRI can provide useful information.
Aortography is the definitive study to verify the
aneurysm.
Treatment
Treatment of ascending TAA is surgical, whereas initial management of descending TAAs is usually medical.
Treatment of hypertension, often with use of a betablocker, may prevent, or decrease the rate of, expansion
and risk of rupture. Surgical consultation should not be
delayed in either symptomatic or asymptomatic patients.
Overall, prognosis is poor in untreated patients. Five-year
survival ranges from 15% to l9Yo.
124 /
EurRceNcv MrorcrNr: THn Conn Cunruculul,t
Thoracic Dissection
into the pericardium with pericardial tamponade or hypovolemia secondary to rupture.
The most common catastrophe involving the thoracic

aorta is acute dissection. Untreated patients have a mortality of over Io/oper hour delay. However, survival can be
improved by early diagnosis and rapid institution of medical and surgical therapy.
Pulse deficits and blood pressure discrepancies

between limbs are characteristic signs. These are usually
noted in the upper extremities due to involvement of the
subclavian arteries. Howeveq pulse deficits in the lower
Etiology
Dissection results from endothelial disruption with
leakage of blood into the media, due to degenerative
weakness of the muscularis layer of the aorta. This degen-
eration may be secondary to atherosclerosis, or cystic

medial necrosis as in Marfan's syndrome. Medial necrosis may also occur with coarctation, pregnancy, or congenital abnormalities. Blood tracks between the media
and the adventitia, resulting in true and false lumina,
although communication between the two may be present. Distal extension usually occurs very rapidly because
of little tissue resistance in the potential space. Retrograde dissection may occlude coronary arteries, and rupture into the pericardium may cause cardiac tamponade.
Risk factors associated with dissection include large
aneurysm diameter, hypertension, smoking, and age.
extremities may occur if dissection involves the iliac or

femoral arteries.
Involvement of the aortic annulus may produce CHF
and signs and symptoms of aortic insufficiency. The combination of hypotension, muffled heart sounds, and jugular venous distention (Beck's triad) indicates pericardial
tamponade.
The symptomatology of acute thoracic aortic dissection may mimic a number of pathologic processes. Chest
pain may be similar to that of acute MI or pulmonary
embolism. Distal extension of a thoracic dissection may
of
with symptoms typical

acute surgical
abdomen. When the blood supply to the brain or spinal
cord is compromised" neurologic findings typical of acute
stroke or paraplegia may result. Pulse discrepancies from
occlusion of subclavian, iliac, or femoral arteries may
present
simulate an acute thromboembolic disease.

Anatomic Clas sifi cation
Ancillary
Classification is based on location of the dissection,

not on the site of origin of the tear. There are two widely
accepted systems of classification, DeBakey and Stan-
ford. Stanford type
aortic dissection involves
the
ascending aorta and is frequently described as a proximal
dissection. Type A includes DeBakey types I and II

(ascending only or ascending and involving the arch,
respectively) and occurs in approximately 67oh of

patients. All aortic dissections not involving the ascending aorta are defined as Stanford type B. These include
DeBakey type
III
and are referred to as distal dissections.
Initial Presentation
Typically, patients experience abrupt onset ofa learing,
excruciating chest pain that radiates to the back. Pain may
also be reported in the epigastrium, flank, abdomen, or
any extremity. If the dissection severely limits or obliterates flow to the major vessels of the brain, abdominal
organs, or extremities, evidence of early ischemia or
infarction will be seen. Thus, a patient may present with
syncope, acute stroke, paraplegia, visceral or renal ischemia, or acute pulselessness in an extremity.
More than 75o/o of patients have a history of chronic
hypertension, although blood pressure can be elevated,
normal, or low. Hypotension may result from dissection
Tests
The ECG may indicate chronic hypertension, myocardial ischemia or infarction, or heart block if the coronary
vessels and conduction system are involved. ECG findings indicative of myocardial ischemia are present in 10o/o
to 40o/o of patients.
Echocardiography is noninvasive, quick, widely available, easily performed at the bedside, and requires no
contrast or radiation. However, the sensitivity of transthoracic echocardiography is only -60%.In addition, image
quality is adversely affected by obesiry mechanical ven-
tilation, and emphysema.

Transesophageal echocardiography has replaced aortography as the gold standard for detection of thoracic
aortic dissection. It overcomes many of the obstacles to
transthoracic echocardiography, and provides detailed
anatomic information about the dissection, including
coronary involvement, and functional information. It is
contraindicated in patients with esophageal disease,
including varices, tumors, and strictures, and may not be
tolerated by up to 3% ofpatients.
Computed tomography with contrast medium is minimally invasive with a sensitivity and specificity equal to
aortography. Spiral CT adds the advantages of increased
scan speed and the ability to provide three-dimensional
images. Only intravenous contrast is necessary, decreas-
ing the risk of nephrotoxicity. Disadvantages include
Cemlovescur-{R DTsoRDERS
increased image processing time for more sophisticated
images, increased signal noise, and decreased intravascular detail. CT has the disadvantage ofpatient transfer and
restricted access to the patient while being scanned.
MRI is appealing because it is noninvasive, requires no
contrast medium, and can visualize the aorta and its
branches in detail, but its limited availabiliry high cost,
and inaccessibility to the patient limit its use in the emergent settmg.
Aortography remains one of the best methods to assess
the anatomy ofthe aorta and its branches, but disadvantages include risk of aortic rupture from catheter manipulation, and nephrotoxicity or anaphylaxis from contrast
media.
Treatment
Arterial pressures, urine output, level of consciousness, and neurologic status should be closely monitored.
Hypotension usually responds to fluid resuscitation with

crystalloid or blood products. Immediate surgery is necessary when the ascending aorta is involved in the dissection (Stanford classification type A), because of the
risks of acute aortic regurgitation and coronary sinus
occlusion. Dissections of the descending aorta (type B)
are initially treated medically and repaired later unless
evidence of continued dissection is present. Medical
treatment consists of aggressive blood pressure control
with beta-blocking agents and nitroprusside. Narcotics
and short-acting benzodiazepines may be used to alleviate pain and anxiety.
SELECTED READING
Bradbury AW, Milne AA, Murie JA. Surgical aspects of Behget's disease.
Br J Surg 1994;81(12):1712-1721.
Browne BJ, Jotte RS, Rolnick M. Raynaud's phenomenon in the emergency
department. J Emerg Med 1995;13(3):369-378.
Chirillo F, Cavallini C, Longhini C, et al. Comparative diagnostic value of
transesophageal echocardiography and retrograde aortography in the
evaluation of thoracic aortic dissection. Am J Cardiol 1994;74(6):
590-595.
Cook JB Ma AO. Medical therapy of peripheral arterial occlusive disease.
Surg Clin North Am 1995;75(4):569-579.
Curci JJ. Modes of ilresentation and management of inflammatory

aneurysms of the abdominal aorta. J Am Coll Surg 1994;178(6):
573-580.
Dapunt OE, Galla JD, Sadeghi AM, et al. The natural history of thoracic
aortic aneurysms. J Thoracic Cardiovascular Surg 1994;107(5):
1323-1332; discussion 1332-1 333.
Gloviczki P. Ruptured abdominal aortic aneurysms. In: Rutherford RB, ed.
Vascular surgery, 4th ed. Philadelphia: Saunders, 1 995; 1060-1069.
Grayor RA, Comerota AJ, Douville I et al. (the STILE Investigators).
Results
of a prospective randomized trial evaluating
surgery versus
thrombolysis for ischemia of the lower extremity. The STILE Tial. Ann
Surg 1994;220(3):25 1 166; discussion 266168.
Hollier LH, Taylor LM, Ochsner J. Recommended indications for operative
treatment of abdominal aortic aneurysms. J Vascular Surg 1992;15(6):
1046-1056.
Kerr GS, Hallahan CW, Giordano J, et al. Takayasu arteritis. Ann Intern
M e d 1994;120(1 1):9 19-929.
Kuzu MA, Ozaslan C, Koksoy C, et al. Vascular involvement in Behget's
disease:
/ I25
8 year audit. World J Surg 1994;t8(6):948-953; discussion
9s3-954.
Laissy JP, Blanc F, Soyer i et al. Thoracic aortic dissection: diagnosis with
transesophageal echocardiography versus MR imaging. Radiology
1995;194(2):331-336.
Lavanier GL, Sacks Q Robinson ML. Acute limb ischemia. Emerg Med
Clin NorthAm 1992;10(l):103-1 19.

Mandell BF, Hoftnan GS. Differentiating the vasculitides. Rheumatic Dis
C lin Nor th Am 199 4 ;20(2) :409 442.
Mitchell MB, Rutherford RB, IGupski WC. Infrarenal aortic aneurysms.
In: Rutherford RB, ed. Vascular surgery, 4th ed. Philadelphia: Saunders,
1
995; I 032-1059.
Ouriel K, Comerota AJ. Thrombolytic therapy and the management of

peripheral arterial occlusion. In: Ouriel K, ed. Lower extremity vascular
disease. Phlladelphia: Saunders, 1995;295-320.
Ouriel K, Shortelt CK. Popliteal and femoral aneurysms. In: Rutherford
RB, ed. Vascular surgery, 4th ed. Philadelphia: Saunders,
1995;
l 103-1 I 12.
Riggs P, Ouriel K. Thrombolysis in the treatment of lower extremity occlusive disease. Surg CIin NorthAm 1995;75(4):633-645.
Roggo A, Brumre U, Ottinger LW, Largiader F. The continuing challenge
of aneurysms of the popliteal artery. Surg Gynecol Obstet 1993;
t 77(6):565-s72.
Rothrock SG, Green SM. Abdominal aortic aneurysms: current clinical
strategies for avoiding disaster. Emerg Med Rep 1994;15(14):125-136.
Rubin GD, Zarins CK. MR and spiral,/helical CT imaging of lower extremity occlusive disease. .lurg Clin North Am 1995;75(4):607-620.
Siegel CL, Cohan RH. CT of abdominal aortic anetrysms. Am J

Roentgenol 1994;163(l):17 -29.
Siegel CL, Cohan RH, Korbkin M, Alpern MB et al. Abdominal aortic
aneurysm morphology: CT features in patients with ruptured and non-
ruptured aneurysms. Am J Ro entgen o I I 99 4;1 63 (5) : 1 123-1 I 29.

Soh D-W, Shin G-J, Oh JK, et al. Role oftransesophageal echocardiography in hemodynamically unstable patients. Mayo Clin Proc 1995;70:
925-931.
Tilson MD, Gandhi RH. Arterial aneurysms: etiologic considerations. In:
Rutherford RB, ed. Vasailar surgery,4th ed. Philadelphia: Saunders,
1995;253-263.
Venous (2.5.2)
The most common venous disorders presenting to the
ED are due to insufficiency, inflammation, or thrombosis.
These are best managed conservatively, and rarely require
surgical intervention. The primary goals of therapy are to
alleviate symptoms and to prevent the complications of
ulceration, systemic sepsis, and thrombosis.
Ve n o u
s I n s ullicien cy/Varic
os
itie s (2. 5. 2. I )
Venous insufficiency is a debilitating, although not a

limb- or life-threatening, disorder. Its main presentations
include telangiectasia and reticular or varicose veins,
although cutaneous changes including ulceration and cellulitis may develop. This venous dysfunction does sometimes occur after venous thrombosis, although the stigmata may be present with no evidence of phlebitis or
thrombosis, and patients who have had venous thrombosis do not necessarily show subsequent evidence of
venous dysfunction.
Varicose veins are the most common manifestations of
venous dysfunction. Varicosities occur as a result of several factors, including venous hypertension, venous valve
insufficiency, and degree of calf muscle contraction,
126 /
EunRcnNcy MnnrcrNr:
Tnr Conr
Cunnrculurvr
which is the primary pumping mechanism to assist blood

flow from the distal venous system. Calf muscle contraction primarily augments blood flow from the deep veins.
In venous insufficiency, blood refluxes via perforator
veins to the superficial veins, which have much less soft
tissue support. Therefore, patients most likely to have
varicose veins are those with elevated venous pressure,
such as patients who stand for prolonged periods of time,
and inactive patients with decreased muscle contraction.
There also appear to be hormonal influences because of
the high incidence of varicose veins early in pregnancy,
thought initially to be triggered by progesterone, although
estrogen also relaxes smooth muscle fibers. There may
also be familial inheritance of simple dominance. Varicose veins may be due to alterations in vein wall, collagen, and/or elastin characteristics.
The most common presenting symptom is diffirse ache
secondary to stretching of or pressure on somatic nerve
fibers adjacent to dilated veins. Patients will also show
concern for the cosmetic appearance of dilate4 tortuous
veins, even if asymptomatic.
Physical examination of the patient with venous insufficiency includes careful examination of the patient's
extremities from the groin to the toes, looking for reticular veins, telangiectasia, and varicose veins, which are
most evident along the greater saphenous circulation. The
varicosities should be easily compressible with no palpable cords. There may be chronic skin changes, such as
hyperpigmentation secondary to hemosiderin deposition;
however, ulceration or cellulitis can develop in these tisSUES.
Presence of reflux can be assessed using Doppler or

duplex examination. Continuous wave Doppler ultrasound can be used to detect reflux, and is one ofthe best
ways for assessing venous insufficiency by looking for
venous obstruction secondary to thrombosis.
The initial treatment of venous insufficiency is modification ofrisk factors, ifpossible, followed by conserv-
ative therapy. The patient should be discouraged from

standing for long periods of time, encouraged to increase
calf muscle use, which enhances muscular pump action,
and evaluated for application of external compression
therapy, including support stockings, elastic supports,
elastic bandages, cast boots, or semirigid support appliances. The elastic supports should have maximal pressure
distally, decreasing more proximally to encourage flow

from the periphery to the central venous circulation.
Venous stasis ulcers require bed rest, leg elevation, and
aggressive wound care; infected ulcers and those with
surrounding cellulitis require appropriate antibiotic therapy. There are no specific medications at this time for
venous insufficiency, although methylxanthines may
improve blood cell deformability and may inhibit interleuken-2 alterations of the microvasculature. Oxypentoxifulline may also promote healing of venous ulcers,
although results ofthese studies are inconclusive.
In patients who fail external compression therapy, surgical therapy may need to be considered, including superficial venous sclerotherapy or disruption of perforating
veins. Valvuloplasty and venous segment transfer may
also need to be considered.
Thromboembolism
(2.
5.
2.2)
Thromboembolism or deep venous thrombosis (DVT)

is a common condition. Its true incidence is difficult to
determine, because the majority of cases are not clinically significant. Its diagnostic significance is due to the
risk of pulmonary embolus if a clot dislodges and travels
to the pulmonary vasculature; therefore, early diagnosis
and treatment are essential to avert this potentially catastrophic complication. Deep venous thrombosis has
many etiologies, which can be divided into clinical factors and coagulopathies. This section reviews the pathophysiology, etiologies, and epidemiology of deep venous
thrombosis, and its clinical presentation, differential
diagnosis, current diagnostic methods, and recommended
treatment.
E ti
ology and Pathop hy s i o I o gy
Venous thrombosis occurs as a result of hemostatic

abnormalities involving the vessel wall, the coagulation
cascade, or blood flow characteristics. When abnormalities of one or more of these components occur, there is a
high risk of thrombosis.
Veins are components of a low-flow system, not
exposed to significant hydrodynamic stresses; as a result,
they have a poorly developed adventitia and media. Oneway valves in the superficial and deep systems, assisted
by the pumping action from calf muscle contraction,
facilitate venous return from the periphery to the central
circulation. Proximal to the valves there is relative stasis
that increases with valve damage; vessel trauma with
endothelial damage activates the coagulation cascade
with platelet activation and aggregation, resulting in a
thrombus.
Normal blood flow is nonturbulent with little contact

between the cells and the endothelium. When blood is
more viscous, there is greater interaction between the cellular elements of the blood and the endothelium, leading
to increased risk of thrombosis. Enhancement of the procoagulable state or deficiencies of the anticoagulable
state, such as antithrombin C, protein C, and protein S
deficiencies, also result in increased thrombosis or
decreased fibrinolysis.
Most commonly, DVTs occur due to an acquired,

rather than congenital, condition. Some of these include
pregnancy, malignancy, burns, trauma, sepsis, hemiplegia, the post-operative state (particularly pelvic surgery
or orthopedic procedures), and cardiac disease. Other
Cennrqvescut-AR DTsoRDERS
contributing factors include sedentary lifestyle, the use of
estrogen-containing compounds, and a history of previous thrombotic events. Cardiovascular disease and shock
states are thought to be predisposing factors due to
hypoperfusion, resulting in stasis and thrombosis. Malignancies are well documented as creating a hypercoagulable state. Congenital or hereditary blood defects, such as
antithrombin III, protein C, protein S, plasminogen,
heparin cofactor 2, and tPA deficiencies, dysfibrinogenemia, and other hereditary coagulopathies, as well as factor XII deficiency, contribute to a hypercoagulable and
ultimately thrombotic state. Acquired blood defects, such
as polycythemia, thrombocytosis, lupus anticoagulant,
anticardiolipin antibodies, and acquired coagulopathies
also create a hypercoagulable state.
Initial Presentqtion
The presentation of deep venous thrombosis varies
greatly. Patients often present with a complaint of recentonset unilateral leg pain and swelling. There is generally
no history of trauma or infection, although there may be
a history of recent immobilization, such as recent hospitalization, a sedentary lifestyle, or a history ofprolonged
sitting, such as an automobile or plane trip. DVTs are
much more common in the lower extremities than in the
upper. The majority of DVTs occur in infrapopliteal
veins, although proximal DVTs are more likely to result
in embolization. The location of swelling depends on the
location of the thrombus, with venous congestion and
swelling distal to the thrombus. There may be localized
pain secondary to focal inflammation, although this is
nonspecific and is often present with other disorders.
Patients may present with concurrent dyspnea related
to pulmonary thromboembolism, and the first indication
that a patient has a DVT may be cardiopulmonary arrest
due to pulmonary embolism (PE). The latter is known as
the "great imitator," with some patients presenting with
chest pain, shortness of breath, tachycardia, diaphoresis,
and anxiety, although few or none of these symptoms
may be present.
Past medical history is important to determine if the
patient has had previous evidence of embolic or thrombotic events, rheumatic heart disease, ischemic heart disease, malignancies, infections, or trauma or surgery to the
pelvis, abdomen, or lower extremities. Significant family
history includes autoimmune disease, malignancy, hyperlipidemia, or coagulopathy. Other significant factors
include use oftobacco, use oforal contraceptives, sedentary lifestyle, and obesity.
The physical examination of patients with DVT is variable and often misleading. In addition to the patient's
vital signs (to assess for fever, tachypnea, and tachycardia
in particular), it is important to determine the presence or
absence of palpable cords, erythema (90oh specificity),
127
warmth of the extremity or part of the extremity (90%

specificity), superficial venous dilatation (80% specificity), tenderness, and the comparative size of the
extremity (taking measurements at several locations on
the extremity). The classic Homan's sign with pain of the
calf on dorsiflexion with a palpable cord in the calf has a
75Yo specificity and a40Yoto 50oh sensitivity. Stasis der-
matitis and venous stasis ulcers can be present, without

evidence of preceding or concurrent DVT. It is important
to evaluate the patient for signs and symptoms of PE,
such as sudden onset of dyspnea with tachypnea, tachycardia, pleuritic chest pain, and hemoptysis.
The differential diagnosis of DVT includes varicose
veins, muscle strain, hematoma, venous reflux, Baker's
cyst, cellulitis, lymphangitis, malignancy with venous or
lymphatic obstruction, exacerbation of rheumatoid arthri-
tis, contact dermatitis, gout, erythema nodosum, and

superficial phlebitis.
Ancillary Studies
Due to the 50oh error rate in diagnosis of DVT based
on history and physical, diagnostic studies have been
developed to improve the accuracy of diagnosis. The gold
standard
is contrast venography, with the injection of
radiopaque contrast into the peripheral veins of the

affected extremity. Flow can be assessed from a distal to
proximal direction, with visualization of the vessels and
any vascular lesions, including obstruction. Chronic
occlusion can be differentiated from acute occlusion.
Although highly specific and sensitive, it is also invasive.
Risks of this technique include inability to cannulate a
distal vein, allergy to contrast media, renal compromise
from contrast media, and potential for thrombogenesis by
the contrast media. This technique also requires the
appropriate radiology suite and staffto perform the procedure.
Studies have been developed that are less risky, with

very good sensitivity and specificity. One of the most
popular techniques is venous impedance plethysmography (IPG). This is a noninvasive test that analyzes flow
characteristics of the venous system. Recent studies
demonstrate a sensitivity of 6lYo to 9loh, a specificity of
45Yo to l00o/o, a negative predictive value of 68% to 960/o,
and a positive predictive value of 48oh to 1000/0, compared to venography in symptomatic patients. Because of
these wide variations, other tests should be considered in
patients with high clinical suspicion.
Other tests showing higher sensitivity and specificity
include Doppler ultrasound, B-mode compression ultrasound (preferably with color Doppler), fibrinogen labeling, and ascending contrast venography. Doppler ultra-
128 /
ErvmRcENcy MnorcrNr,:
Tnn ConB CuRnrculunr
sound is a noninvasive method that is considered 94%

accurate. The diagnosis is made by assessing the venous
velocity signal. It is most useful in symptomatic patients
with proximal DVT and is not sensitive in patients who
are asymptomatic or in patients with calf DVT. Real time
B-mode ultrasound is also useful for the diagnosis of

proximal DVT with a sensitivity of 90o/o and specificity
of 97% in symptomatic patients. Again, sensitivity is
decreased in asymptomatic patients or patients with calf
DVTs. B-mode combined with duplex scanning Doppler
is faster and more accurate than each of the other studies
individually. The color-enhanced Doppler flow can examine both deep and superficial veins and the color
enhancement allows venous flow assessment, with and
without compression of the vessels. Calf veins can be
assessed by this method and thrombus can be visualized,
and the age ofthe thrombus can be assessed. Its accuracy
in symptomatic patients is 73% to 92oh sensitivity with
860/o to 100% specificity. Limitations of ultrasound and
Doppler techniques also include availability of equipment and personnel to do the procedures, and risks of
interobserver inaccuracies.
D-dimer assay is currently being touted as an easy
adjunct
in the
diagnosis
of DVT. D-dimer is a fibrin
degradation product found to be elevated in patients with

DVT and PE. The blood test, using a highly specific monoclonal antibody against D-dimer, induces red-cell agglutination in the presence of elevated D-dimer levels. Early
studies suggest that a positive test is 93% sensitive for the
diagnosis of proximal
DVI
and 70% sensitive for calf
DVI with 77o/o specificity. This low specificity is due to

other conditions with elevated D-dimer levels, such as
surgery or trauma within 10 days, acute infection, pregnancy or recent delivery, active collagen vascular disease,
or metastatic cancer. Since all of these are DVT risk fac-
tors, D-dimer is currently being recommended only in

conjunction with another study, such as IPG, to improve
the positive predictive value of both studies.
There are no other specific screening studies available,
although a prothrombin time and a partial thromboplastin
time are recommended as baseline studies prior to therapeutic anticoagulation. Other baseline studies should
include hemoglobin and hematocrit, and a platelet count.
Specialized tests, such as factor assays and antithrombin
III levels, are not part of the screening procedure; however, they may be indicated in the workup for the etiology
of the DVT. Pulse oximetry, arterial blood gases, electrocardiogram, and chest x-ray should be obtained if there is
any suspicion of pulmonary embolism.
as the use
Thrombophlebitis
is currently
being
(2.
5. 2.
3)
Thrombophlebitis is usually a self-limited disorder of

superficial veins. It is localized inflammation and thrombosis of a vein, often precipitated by trauma. The majority of cases are reported after venous cannulation; vasculitis and use of oral contraceptives are also associated
with its development. Endothelial damage triggers an
inflammatory response, manifested by tenderness and
overlying erythema. Secondary infection by skin organisms such as Staphylococcus epidermidis can occur with
rare progression to suppurative phlebitis.
Complications include involvement of the deep venous
system with subsequent deep venous thrombosis and pulmonary embolism, septicemia, and postphlebitic ulcera-
tions. Diagnosis is primarily clinical, although duplex

scanning is advised to determine if the thrombus has
extended to the deep venous system.
Treatment is conservative, with use of analgesics, heat,
elevation, and elastic support bandages. Antibiotics are
not indicated unless an organism is isolated from blood
cultures, and anticoagulation is necessary only ifthe deep
venous system is involved. In cases ofextensive phlebitis,
recurrence, or suppurative disease, phlebectomy may be
necessary.
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Carter CJ. The pathophysiology ofvenous thromb osis. Prog Cardiovasc Dis
1994;36(6):439446.
Colucciello SA, Jones JJ Stewart C. Evaluation and management of deep
venous thrombosis. Emerg Med Rep 1996;17(9):89-100.
Johnson G. Superficial venous thrombosis. In: Rutherford RB, ed,. Vascular
s urgery, 4th ed. Philadelphia: Saunders, I 995; I 696-1 698.
Koopman MM. Diagnosis of deep vein thrombosis. Prog Cardiovasc Dis
1994;37
Traditional management of DVT is initial anticoagulation with intravenous heparin, followed by continued
anticoagulation with warfarin. Alternative therapy, such
agents,
mined if possible. Other treatments have included prophylactic heparinization in high-risk patients. In patients
who have contraindications or failure of anticoagulant
thenpy, an inferior vena cava filter should be placed to
prevent the occurrence of pulmonary embolism. Patients
also need to be evaluated for the risk of paradoxical
embolism, with migration of venous clot through a septal
defect resulting in cerebrovascular thromboembolism.
Markel
Treatment
of thrombolytic
investigated as are alternative heparin and warfarin regimens. The etiology of the thrombosis should be deter-
(t):1-12
A, Weich X Gaitini D. Doppler ultrasound in the diagnosis of
venous thrombo sis. Angi o I o gy 199 5 ;46(l) :65 -7 3.

Rosenow EC. Venous and pulmonary thromboembolism: an algorithmic
approach to diagaosis and management. Mayo CIin Proc 1995;70(I):
4549.
Weinmann EE. Salzman EW. Deep-vein thrombosis. N Engl J Med 1994;
331(24):1630-1641 .
Wells PS, Brill-Edwards B Stevens P, et al. A novel and rapid whole-blood
Cenorovescur-c,R DISoRDERS
assay for d-dimer in patients with clinically suspected deep vein thrombosis. Circulation 1995 ;91(8):2184-2187.
Wells PS, Ginsberg JS. DVT and pulmonary embolism: choosing the right
diagnostic test for patients at isk. Geriatics 1995;50(2):29-32, 35-36.
Wheeler HB, Anderson FA. Diagnostic methods for deep vein thrombosis.
Haemostas is 1995 ;25(1 -2):6-26.
Lymphatics (2.5.3)
Lymphatic vascular disease is not commonly seen as a
presenting symptom in the ED. The two most frequent
conditions, although rare, that are most likely to be seen
are lymphedema and lymphangitis.
729
appearance. Excoriation may occur, although ulceration

is rare. Ischemic changes are also rare. Some patients
may have small vesicles on the skin draining clear lym-
phatic fluid.
The differential diagnosis of limb swelling is extensive. Systemic causes often result in bilateral exftemity
swelling. These include cardiac failure, hepatic failure,
renal failure, hypoproteinemia, myxedema, allergic disorders, hereditary angioedema, and some medications,
including methyldopa, nifedipine, hy dr alazine, estrogen,
and monoamine oxidase inhibitors. Local or regional
causes are more likely to cause unilateral disease. These
include chronic venous insufficiency, lipedema, congenital vascular malformation, arteriovenous fistula, trauma,
Lymphedema
Lymphedema, the condition of lymph fluid extravasation into the surrounding tissues, occurs most commonly
in the extremities. This is usually secondary to an underlying disorder, such as lymphatic dissection during surgi-
cal extirpation of a malignancy, or obstruction of lymphatic vessels by a mass proximal to the lymphatic
vessels.
The most common surgeries resulting in lymphedema

are surgery of the breast, cervical cancer, soft-tissue
tumors, and malignant melanomas of the leg. Other
causes of secondary lymphedema include lymph node
incision with fibrosis secondary to radiation; tumor invasion into the lymphatic vessels; infection with inflammation and occlusion of the lymphatic vessels; abscess causing extrinsic compression of lymphatic vessels; traumatic
disruption; and parasitic infection or lymphatic filariasis
caused by Wuchereria bancrofti and other wormJike parasites. Filariasis is the leading cause of lymphedema
worldwide, although in North America and Europe surgical intervention is the most common cause of lymphedema.
Primary lymphedema may present as a familial disorder, as in Meige's or Milroy's disease, a congenital disorder in children associated with genetic syndromes, or as
a component of lymphatic dysplasia. Depending on the
etiology, it can be unilateral or bilateral. Other causes of
perceived lymphedema are urticarial manifestations of
dermatitis, factitious edema after prolonged application
of a tourniquet, or prolonged positioning of the extremity
in a dependent position, which impedes venous as well as
lymphatic return.
After the initial fluid extravasation, with recurrent prolonged episodes, gradual fibrosis and scarring in the tissues occurs so that eventually the edema becomes irreversible. Chronic lymphedema is generally painless and
progressive. It starts distally with proximal extension as
the fluid builds up in the exkemity. The skin may appear
slightly hyperemic and have slightly increased warmth,
due to increased vascularity; however, there is eventual
fibrosis and scarring of the skin with a peau d'orange
snake or insect bite, infection or inflammation,

hematoma, dependency, rheumatoid arthritis, and hemihypertrophy. However, it is important to remember that a
localized intraabdominal process can result in bilateral
leg swelling, and a thoracic problem is more likely to
cause bilateral upper extremity, as well as possible lower
extremity, swelling.
The primary symptoms of lymphedema causing distress to patients are the sensation of extremity heaviness
due to the accumulated lymphatic fluid, decreased mobility due to the edema, and, less commonly, complications
such as ulceration or infection. When assessing a patient
who has apparent lymphedema, it is important to determine if there is a family history to determine whether this
is a primary or a secondary disorder. It is also important
to obtain a medical history, including history of prior

malignancies, surgeries, lymph node dissections, irradiation, trauma, or infection, as well as travel to countries
where filariasis is common. Most cases of lymphedema
presenting
to the ED are an
acute exacerbation of
chronic, secondary lymphedema.

Conservative management will usually address the
acute symptomatology. The main treatment for acute
lymphedema is reducing the size of the limb, which is
particularly effective in early cases. This can be attempted
by several means. Elevation is the simplest metho4 with
positioning of the limb at approximately 45 degrees or
more over the level of the heart. Size reduction generally
occurs with 2 to 5 days of continuous bed rest. Massage
of the limb can help promote drainage, with stimulation
of lymphatic flow. Initially, the adjacent limb should be
massaged to stimulate its flow and then in the affected
limb, from distal to proximal, to try to allow some of the
lymphatic fluid to move to the normal limb.
Extremity compression, using a variety of techniques,
is employed often, using intermittent pneumatic com-
pression, such as stockings with sequentially inflated

chambers, graduated elastic support stockings, and elas-
tic bandages wrapped distally to proximally to help prevent fluid reaccumulation. Heat therapy, which is
thought to help mobilize fluid and soften the tissues, is
also used.
130 /
EunncnNcy MnucrNn: THr Conn CunnrculuM
Medications are not routinely used in the treatment of

lymphedema, although diuretics have been used to help
remove excess fluid in lymphedema of recent onset. In
cases of chronic lymphedema, where there is more fibrosis, diuresis is much less effective. Other agents, such as
benzopyrones, are thought to increase macrophage activity and decrease proteinaceous fluid, and steroids have
been tried with varying degrees ofsuccess to decrease the
inflammation in long-standing cases of lymphedema.
Beyond elevation and compression, it will usually not
be necessary in the ED setting to initiate other measures.
Patients should be referred to their primary care physician or a vascular surgeon for follow-up and further evaluation as needed, particularly ifthe patient does not have
a medical, surgical, or occupational history suggesting a
clear-cut etiology for the edema. Studies, such as lymphoscintigrams, can then be performed on an elective
basis as necessary. If malignancy is suspected, CT may be
indicated.
Select syndromes and commonly associated

CHD
Syndrome
Congenital rubella
PDA
Down syndrome
Fetal hydantoin
Fetal alcohol syndrome
Marfan's syndrome
AVSD, VSD
Maternal diabetes
Turner's syndrome
VSD, ASD, PDA, COA

VSD, ASD, TOF
Aortic and mitral insufficiency,
MVP
TGA, hypertrophic
cardiomyopathy
COA, AS
PDA, patent ductus arteriosis; AVSD, atrioventricular septal defect (endocardial causing defect);VSD, ventricular septal defect; ASD, atrial septal defeat; CCA, coarctation of the
aorta; TOF, tetralogy of Fallot; MVP, mitral valve prolapse; AS,
aortic stenosis.
240). Advances in surgical and medical management of these patients result in an 85% survival into
Lymphangitis
is infection of the lymphatic
vessels,
most commonly caused by beta-hemolytic streptococci. It

is usually secondary to another infectious focus, such as
cellulitis, insect bite, ulceration, or trauma. Antibiotic

therapy is essential, with coverage of beta-hemolytic
strep, as well as staphylococci. If there is no obvious etiology, the patient's nutritional and immunologic status
should be evaluated" and occult malignancy should be
considered.
SELECTED READING
Gloviczki P. Lymphedema: introduction and general considerations. In:
Rutherford RB, ed,. Vascular surgery,4th ed. Philadelphia: Saunders,
1995;1 883-1 888.
Joos E, Bourgeois P, Famaey JP. Lymphatic disorders in rheumatoid arthri-
tis. Sem Arthritis Rheum 1993;22(6):392-398.

Rooke I Gloviczki P. Nonoperative management of chronic lymphedema.
In: Rutherford RB, ed. Vascular surgery,4th ed. Philadelphia: Saunders,
1995;1920-1927.
Wright NB, Carty HM. The swollen leg and primary lymphedema. Arch Dis
C h i I d 199 4;7 | (r):4449.
Young JR. The swollen leg. Clinical significance and differential diagnosis.
Clin
24O.
(Table
Lymphangitis
C ardiol
TABLE
199
;9(3):443456.
adulthood. Today early discharge from the newborn nursery is common and acutely ill neonates may present to the
ED for treatment.
Pathophysiology
Fetsl to Neonstal Circulution

The placenta provides for gas and metabolite exchange
in the fetus and the ventricles exist in a "parallel" circuit.

The umbilical vein carries oxygenated placental blood,
half of which is delivered to the inferior vena cava via the
ductus venosus while half enters the hepatic circulation.
Inferior vena caval blood enters the right atrium and is
preferentially directed to the left atrium via the foramen
ovale. This relatively oxygen-rich blood then flows into
the left ventricle and is ejected into the ascending aorta to
supply the fetal upper body. Relatively oxygen-poor

blood flows from the superior vena cava to the right
atrium and enters the right ventricle instead oftraversing
the foramen ovale. This blood is then ejected into the pulmonary artery but most of it flows through the ductus
arteriosus into the descending aorta, supplying the fetal
lower body and returning to the placenta via the umbilical arteries.
CONGENITAL ABNORMALITIES OF THE

CARDTOVASCULAR SYSTEM (2.6)
Congenital heart disease (CHD) is a group of conditions in which abnormalities in cardiovascular anatomy
or function are present at birth or develop during normal
maturation. Approximately eight of every 1,000 live
births will be affected by CHD, and the ventricular septal
defect (VSD) is the most common CHD. Up to 20%o of
patients with CHD have associated congenital anomalies
At birth, pulmonary vascular
resistance rapidly
ofthe lungs and a rise
in arterial oxygen tension, while systematic vascular
resistance increases in response to removal of the low
resistance umbilical circulation. Increased pulmonary
venous return raises left atrial pressures, and, combined
with decreased right atrial pressure from cessation ofplacental flow through the ductus venosus, the septum pridecreases in response to expansion
mum is pressed against the septum secundum. The result
is functional closure of the foramen ovale. Over the
Cennrovescur-{R DTsoRDERS
of several days, and in response to high oxygen

saturation and bradykinins, the ductus arteriosis constricts and eventually becomes the ligamentum arteriosum. These changes transform the fetal circulation to the
neonatal circulation and pair left ventricular outflow with
the high resistance systemic circulation and right ventricular outflow with the low resistance pulmonary circulation. In certain CHD, fetal circulatory pathways persist,
resulting in either life-sustaining flow or additional stress
to the circulation. In addition, decreasing pulmonary vascular resistance during the first few weeks of life influences the timing of clinical signs and symptoms from
CHD that rely on relative differences between systemic
and pulmonary vascular resistance to sustain life.
course

Neonates who are intolerant to the changes in the tran-
sitional circulation present early with cyanosis, most

often in the newborn nursery. Other infants with CHD
typically present in the first few weeks of life, while the
emergency physician may also be confronted with an
older child with an undiagnosed murrnur. A detailed history, including maternal and perinatal information, coupled with a careful physical examination and selected
ancillary studies will help establish a diagnosis and treatment priorities. Infants or children presenting with
cyanosis should be triaged to receive immediate care and
supplemental oxygen. The response to supplemental oxy-
gen can guide the physician in determining whether or

not the cyanosis is resulting from a cardiac or pulmonary
cause. The child with CHD often assumes a position that
reduces the work of breathing (e.g., knee-to-chest), and
should be allowed to maintain that position if possible.
Cardiac and pulse oximetry monitoring should be continuous in the cyanotic child. Advanced airway management
may be necessary in the critically ill with respiratory
decompensation.
FamiliaVGenetically Transmitted Disorders (2.6.1)

History
A birth history should be obtained, including any presence of cyanosis and any oxygen therapy and subsequent
response. Maternal complications, such as diabetes, substance abuse, or medication exposures, should be elicited.
A growth history may reflect delays due to heart disease.
Infants with CHD may exhibit feeding difficulties such as
or diaphoresis. Children may

exhibit poor tolerance of exercise compared to their
peers, and specific questions should be asked regarding
play habits, stair climbing, bicycle riding, and performance in physical education. Cyanosis at rest may be
overlooked by parents while cyanosis during exercise or
easy fatiguing, dyspnea,
/ l3l
crying is often readily noted. Chest pain is usually not
manifestation of cardiac disease in the pediatric patient
but
circumstances surrounding the pain should be

detailed as should any episodes of syncope. Because
extracardiac manifestations may be noted in about 25Vo
of patients with CHD, one should inquire about the presence of a congenital malformation syndrome (Table
240).
Several historical clues can be helpful in diagnosing
CHD and differentiating organic from innocent murmurs.
A positive family history of CHD, especially in firstdegree relatives, is more common with ventricular septal
defects (VSD), patent ductus arteriosis (PDA), atrial septal defects (ASD), and tetralogy of Fallot (TOF). Hypertrophic heart disease may be found up to 20o/o of patients
when present in a first-degree relative, and in up to 30%
of infants born to diabetic
mothers. The incidence of

CHD in infants of diabetic mothers is five times that of
the general population with transposition of the great
arteries (TGA) the prevalent lesion. Thus, TGA should be
considered in the cyanotic neonate born to a diabetic
mother. Patent ductus arteriosis is the most frequent cardiac anomaly in rubella syndrome babies, and is common
in premature infants and those born and living in high

altitudes.
When present in children, chest pain is rarely associated with CHD. The patient with aortic stenosis may have
atypical chest pain with exertion or at rest, and may also
have a history of syncope. Severe pulmonic stenosis,
mitral valve prolapse, and primary pulmonary hypertension all cause chest pain.
A history of squatting following exercise is characteristic in patients with tetralogy of Fallot. Flexing the hips
and knees increases systemic vascular resistance,

decreasing shunting and increasing flow through the pulmonary vasculature.
The age of onset of cardiac failure can provide a diagnostic clue regarding the underlying CHD. From birth to
I week old, hypoplastic left heart, transposition of the
great vessels, coarctation of the aorta, and patent ductus
artenosus are most common. Coarctation of the aorta and
transportation of the great vessels are predominant when
cardiac failure occurs between I week and 2 months of
age. Beyond 2 months, ventricular septal defect is the
most common lesion producing cardiac failure.
Specific CHD predominates in certain clinically recognizable syndromes (Table 240). Forty to 50o/o of
patients with Down syndrome also have a CHD, most
commonly ventricular septal defect or atrioventricular
septal defect (endocardial cushion defect). About 30% of
patients with Turner's syndrome have CHD, with coarctation of the aorta most common. Thirty to 40%o of infants
with fetal alcohol syndrome have CHD, most frequently
ventricular or atrial septal defects or tetralogy of Fallot.
Fetal hydantoin syndrome is associated with an increased
risk of CHD, especially ventricular and atrial
septal
732 /
Eur,ncrNcv MporcrNn: Trtn Conn Cunnrcur-unr
defects, coarctation of the aorta, and patent ductus arteriosus. A child with chronic hoarseness and a heart murmur may have cardiovocal syndrome. These patients have
large left-to-right shunts with pulmonary hypertension
producing a large pulmonary artery that compresses the
left recurrent laryngeal nerve against the aorta and trachea. Atrial and ventricular septal defects and patent ductus arteriosus are most commonly associated with cardiovocal syndrome.
TABLE
241.
Cardiovascular examination
Feature
Deceased femoral pulse
Prominent cardiac impulse
Widely split S
Continuous murmur
Associated Airway
Coarctation of aorta
PDA, VSD
ASD, PS, Ebstein's anomaly,
TAPVR, TOE RBBB
PDA, venous hum
PDA, patent ductus arteriosis; VSD, ventricular septal

defect; ASD, atrial septal defect; PS, pulmonary stenosis;
TAPVR, total anomalous pulmonary venous return, TOF,
tetralogy of Fallot; RBBB, right bundle branch block.
Physical Exuminstion
In addition to the general physical examination,
cific attention should be given to
spe-
abnormalities of
growth, evidence ofrespiratory distress, and the presence

of cyanosis. Obstructive lesions without cardiac failure,
such as aortic or pulmonary stenosis or coarctation of the
aorta, are associated with normal growth. Cyanotic
lesions generally impede both height and weight, while
weight is more adversely affected in lesions producing
cardiac failure. The patient's respiratory status should be
noted, including the rate, effort, and associated presence
of intercostal retractions or nasal flaring. Elevated pulmonary venous pressure is implied in the otherwise
happy infant with persistent shallow tachypnea.
The infant presenting with cyanosis deserves particular
attention. Peripheral cyanosis, in which the patient's arterial PaOz is normal, should be differentiated from central
cyanosis. Transcutaneous oximetry may be unreliable in
poor perfusion states such as shock. Infants with central
cyanosis are warm and well perfused with bluish conjunctivae and tongue. Both transcutaneous oxygen saturation and arterial PaOz are low. The management of the
patient is guided by differentiating between pulmonary
and cardiac causes of the cyanosis. The infant with pul-
monary cyanosis
will
have some degree of respiratory
will be abnormal. Crying, which improves ventilation, will often

lessen the cyanosis unless significant intrapulmonary
shunting is present. Infants with cardiac cyanosis will be
distress, even at rest, and the arterial pCOz
comfortable at rest but have increased cyanosis with agitation. The arterial pCOz is usually normal and there may
or may not be a murmur present. In response to administration of 100% oxygen, patients with pulmonary
cyanosis will show improvement in their oxygen saturation, PaOz, and clinical picture. Cyanotic heart disease
patients show little change in these parameters with oxygen administration due to shunting of blood away from
the pulmonary system.
C ardiovascular Exsmin ation
Radial and femoral pulses should be palpated and

compared for timing and strength. The hallmark of
coarctation of the aorta is strong radial pulses with weak
or absent femoral pulses (Table 241). Bounding pulses

can be a result ofincreased cardiac output (anemia, anx-
iety, increased catecholamine release), or from a lesion

associated with aortic runoff (patent ductus arteriosis,
aortic insufficiency, arteriovenous malformation). Arterial blood pressure should be measured in both the arms
and legs in any patient with suspected coarctation of the
aorta. Pressures in the legs are normally l0 mm Hg
higher than in the arms; coarctation reverses this relationship.
Examination of the chest should begin with inspection.
Suprasternal pulsations can occur with aortic stenosis,
aortic insufficiency, coarctation of the aorta, and patent
ductus arteriosis. A prominent cardiac impulse is seen in
patients with severe mitral regurgitation and large volume
loads such as patent ductus arteriosis and ventricular septal defects, especially in the presence of aortic regurgitation.
Auscultation of the heart should include the precordium, right axilla,and back, and should begin with
concentration on the various heart sounds and their relation to respiration. The second heart sound, caused by
closure of the aortic and pulmonic valves, more noticeably is split during inspiration due to delayed closure of
the pulmonic valve from increased right ventricular filling. Wide splitting is noted in pulmonary stenosis, atrial
septal defect, Ebstein's anomaly, total anomalous pulmonary venous return, tetralogy of Fallot, and right bundle branch block.
Except for aortic and pulmonic regurgitation, murmurs
occur during systole. Since the aortic and pulmonary
valves remain closed during isovolumic contraction, pansystolic murmurs represenf blood exiting the contracting
ventricle via either a ventricular systole defect or atrioventricular (mitral or tricuspid) vahular i4sufficiency. A
continuous muffnur extends from systole into diastole
and indicates continuous blood flow. When heard in the
second left interspace, a patent ductus arteriosis should
be considered. More common is the innocent continuous
murmur of a venous hum, which is most often in the right
upper sternal area. The venous hum murmur is louder in
the upright patient and disappears with compression of
the jugular venous system in the neck. In the absence of
Cerutovescul{R DISoRDERS
historical or physical evidence for underlying cardiac disease, a murrnur may be present without pathologic significance, such as the innocent murrnur of newborns, the
vibratory murmur, the innocent pulmonic ejection murmur, and the aforementioned venous hum. The innocent
murmur of newborns, also termed "peripheral pulmonary
artery stenosis of the newborn," is a short systolic ejection murmur present from birth to 3 months of age and it
is clearly transmitted to both axillae. Between the ages of
2 and 7 years, the vibratory murmur is heard. This systolic murmur has a twanging sound and lessens in the
upright position. The innocent pulmonic ejection murmur
is systolic, limited to the left upper sternal atea, and is a
result of turbulence during increased cardiac output.
Since murmurs of atrial septal defect and mild pulmonary
stenosis can sound identical to the innocent pulmonic
ejection murnur, the patient should be reexamined when
causes ofincreased cardiac output such as fever or apprehension are eliminated.
The age of onset of murmurs can guide the clinician in
diagnosis. Murmurs detectable at birth are usually due to
aortic or pulmonic stenosis. Patent ductus arteriosis murmurs may occur as early as 6 hours after birth. Murmurs
from ventricular septal defects are usually delayed for

several hours to a few weeks depending on the size ofthe
defect and the time at which the pulmonary vascular
resistance drops enough to allow shunting to occur. Atrial
septal defect murmurs may not be detectable for a year or
more after birth.
E xtruc ardi a c E xamin atio n
of
congenital syndromes should be

sought, as approximately 20o/o of patients with CHD will
have extracardiac anomalies. Auscultation of the lungs
should be done to search for rales and wheezes and evaluate the adequacy of ventilation. Most normal infants
have a palpable liver edge, but one more than 3 cm below
the costal margin is abnormal. Tachypnea, rather than
hepatomegaly, is a more reliable sign of congestive heart
The presence
failure. Puffiness of the eyelids, rather than edema of the
is a common manifestation of righrsided

cardiac failure in small children. Patients with persistently or permanently low arterial oxygen saturation
extremities,
(<90%) often have red fingers and toes as a precursor to

cyanosis and clubbing.
Ancillary Studies
Measurement of oxygen saturation is a fast, noninvasive way to monitor the patient and the response to oxygen treatment. The infant with a cardiac cause of cyanosis
will not show improvement in
oxygen saturation with

supplemental oxygen, but oximeter readings are unreliable in patients with poor skin perfusion. Arterial blood
133
with normal
pH and pCOz in the cyanotic infant with CHD.
The chest x-ray is invaluable in evaluating patients
with suspected CHD (Table 242). One should evaluate
the pulmonary vasculature, aortic arch, cardiac silhouette, and position of the gastric bubble and liver (abdomgases are more reliable and show a low PaOz
inal situs). In the cyanotic infant, fullness of the pulmonary vasculature is associated with transposition of
the great arteries, total anomalous pulmonary venous
return, and truncus arteriosis. In the acyanotic infant,
increased pulmonary vasculature will nearly always be
due to one ofthree lesions: atrial septal defect, ventricular septal defect, or patient ductus arteriosis. Decreased
pulmonary vasculature indicates obstruction to pulmonary flow. The lungs will appear overexposed, and
most patients will be cyanotic. Tetralogy of Fallot, pulmonary atresia, and tricuspid atresia are the most common CHDs associated with this finding. The presence of
irregularly branched pulmonary vessels suggests systemic collateral arteries and is most often associated with
severe pulmonic stenosis or atresia.
A right aortic arch is characterized radiographically by
transverse
aortic arch situated to the right side of the
a
The
tracheal air column will deviate to the left
trachea.
just below the clavicles, the superior vena cava will deviate to the right, and the descending aorta will usually be
on the same side as the aortic arch. A right aortic arch is
most commonly associated with tetralogy of Fallot (with
or without pulmonary atresia), truncus arteriosis, double
outlet right ventricle and single ventricle, as well
as
asplenia syndrome.
The cardiac silhouette may be normal or abnormal in

the patient with CHD. Cardiomegaly can be seen with
coarctation of the aorta, Ebstein's anomaly, patent ductus
TABLE
242.
Chest x-ray findings in CHD
Chest x-ray findings
Associated anomaly
Cyanotic: TOGA, TAPVR, TA

Acyanotic: ASD, VSD, PDA
vasculature
TOF, pulmonary atresia,
Decreased pulmonary
tricuspid atresia
vasculature
Systematic colateral arteries Severe pulmonic stenosis or
atresia
TOF, TA, double outlet right
Right aortic arch
ventricle, single ventricle
COA, Ebstein's anomaly,
Cardiomegaly
PDA, VSD, ASD, AS,
AVSD
TOF
"Boot shape"
TOGA
"Egg on side"
TAPVR
"Snowman"
lncreased pulmonary
TOGA, transposition of the great arteries; TAPVR, total

anomalous pulmonary venous return; TA, truncus arteriosis;
ASD, atrial septal defect; VSD, ventricular septal defect; PDA,
patent ductus arteriosis;TOF, tetralogy of Failot, COA, coarciation of the aorta; AS, aortic stenosis; AVSD, atrioventricular
septal defect (endocardial Cushing defect.)
134 t
EtrnRcrNcy MnorcrNn: THE Conn Cunrucur-ul,r
arteriosis, ventricular and atrial septal defects, aortic

stenosis, and atrioventricular septal defect. The classic
"boot-shaped" cardiac silhouette of tetralogy of Fallot is
due to an average size heart coupled with a concave pulmonary artery and decreased pulmonary markings. Transposition of the great arteries is characterizedby an,,egg
on side" appearance to the cardiac silhouette. This is due
to cardiomegaly with a narrow mediastinum, an aortic

arch convex to the right, and the right heart abnormally
convex in the frontal view. Total anomalous pulmonary
venous return without obstruction presents with a "snowman" or "figure of eight" cardiac silhouette. This is due to
anomalous drainage of the left pulmonary veins into a persistent left superior vena cava (vertical vein) together with
the irurominate vein and right superior vena cava.
Abnormal abdominal situs suggests complex cardiac
disease. The incidence of CHD is almost 100% in the
presence of visceral situs inversus and dextrocardia, and
75o/o to 85o/o in patients with normal visceral situs and
dextrocardia.
The ECG is almost always abnormal in the patient with
CHD. The ECG in the normal infant has right axis deviation and a dominant R wave in the right chest leads. The
T wave in Vr should be inverted after the first week of

life; persistence of a positive T wave is evidence for right
ventricular hypertrophy. Specific ECG findings depend
on the particular CHD.
Echocardiography provides noninvasive yet definitive

evaluation of the patient with CHD. Accurate evaluation
of the anatomy and physiology is provided, and most
patients can undergo initial operative intervention based
on echocardiographic results. MRI can be used to study
function as well as structure and is the imaging modality
of choice for confirming the presence of coarctation and
for characterization of the lesion. Cardiac catheterization
is generally used for balloon atrial septostomy, valvuloplasty, or angioplasty.
initially, followed by 25% of the digitalizing dose at g

hours and at 16 hours. Preterm neonates require a total
loading dose of 20 mcglkg, full-term neonates require 30
mcgkg, while 40 mcg/kg is used from I month to 2 years
of age and 3 5 mcg/kg (max 2.0 mg) for those 2 to I 0 years
old. Palliative and./or corrective surgery provides definitive management of CHD anomalies.
Most patients with mild CHD require no emergent
treatment and should be referred to the pediatrician or
pediatric cardiologist for continued care. patients with
CHD require antimicrobial prophylaxis for dental, urinary tract, and lower gastrointestinal tract procedures.
Disorders Due to Anatomic Anomalies (2.6.2)
Congenital heart disease is commonly divided into
cyanotic and acyanotic lesions. Cyanotic lesions can be
further subdivided into those with increased or decreased
pulmonary flow (Table 243). Acyanotic lesions can be
subdivided into those with an increased volume load and
those with an increased pressure load (Table 244).
Cyanotic Heart Disease
The common denominator with these lesions is admixing ofdeoxygenated venous blood with oxygenated arterial blood resulting in cyanosis. A pressure gradient exists
between the pulmonary circulation and the systemic circulation, creating a right to left shunt. Cyanosis increases
with increased right to left shunting. Thus, the less pulmonary flow, such as from increased pulmonary resistance or decreased systemic resistance, the greater degree
of cyanosis. Prostaglandin Er (alprostadil) treatment of

the cyanotic infant with CHD causes dilatation of the
ductus arteriosis and provides adequate pulmonary blood
flow until surgical intervention occurs.
D ec reas
E mergen cy D ep artm
nt I nterv entio n
on
ary F low
Tetralogy ofFallot
Supplemental oxygen should be administered to the

cyanotic infant with CHD. Endotracheal intubation may
be required for the critically ill. The patient should be
placed on a cardiac monitor and intravenous access
obtained. For the infant with cyanotic CHD, prostaglandin
Ey (alprostadil) should be infused at a rate of 0.05 to 0.1
mcglkglmin. Prostaglandin Er will maintain patency of
the ductus arteriosis; howeveq common side
ed Pulm
eflects
include apnea, hypotension, fever, and j itteriness. Cardiology consultation should be obtained and the patient admitted to the pediatric intensive care unit. Patients with congestive heart failure also require admission and cardiology
consultation. Furosemide can be administered for diuresis
at an initial dose of I mg/kg intravenously. Digitalization
may be required with half of the digitalizing dose given
Constituting 10Yo of all CHD, tetralogy of Fallot (TOF)

is the most common cyanotic CHD. Patients have the
TABLE
243.
Cyanotic CHD
Decreased pulmonary flow

Tetralogy of Fallot
Pulmonic stenosis
Pulmonic atresia
Tricuspid atresia
Ebstein's anomaly
I ncreased pulmonary flow
Transposition of the great arteries
Total anomalous pulmonary venous return
Truncus arteriosis
Hypoplastic left heart syndrome
Cenuovescur-AR DISoRDERS
TABLE 2-44. Acyanotic CHD
Pulmonary valve atresia may occur with or without an

intact ventricular septum. Cases with an intact septum
account for 25o/o of newborn cyanotic heart disease and
Atrial septal defect

Ventricular septal defect
Atrioventricular septal defect
Patent ductus arteriosis
Pressure load
Coarctation of the aorta
Aortic stenosis
Hypertrophic heart diseasea
Mitral valve prolapsea
severe symptoms occur with closure of the ductus arteriosis shortly after birth. The second heart sound is single
and loud and often there is no murmur. Patients with a
ventricular septal defect are described as having an

extreme form of tetralogy of Fallot with the entire right
aPressure may be normal.
tetrad of right ventricular outflow obstruction (pulmonic
of the aorta, ventricular
septal
defect, and hypertrophy ofthe right ventricle. The degree

of cyanosis and type of clinical dysfunction depends on
the severity ofthe right ventricular outflow tract obstruction. Patients may exhibit hypercyanotic or "tet" spells
characteized by abrupt cyanosis, dyspnea, and agitation
initiated by crying or straining. A harsh systolic ejection
munnur is heard over the pulmonic area along with a single second heart sound. Chest x-rays reveal a "bootshaped" cardiac silhouette, and the ECG reveals right
axis deviation and right ventricular hypertrophy. Echocardiography confirms the diagnosis.
Treatment of hypercyanotic spells involves placing the
patient in the knee-to-chest position in a calming environment. If needed, morphine sulfate (0.2 mg/kg/dose)
can be administered to decrease pulmonary blood flow
and relieve anxiety. Propranolol (0.1 mg/kg) intravenously can be used if other measures fail. Bolus infusion of intravenous fluids or administration of phenylephrine can raise systemic pressures and decrease
right-to-left shunting in refractory
135
Pulmonary Atresia
Volume load
stenosis), overriding
cases.
ventricular output ejected into the aorta. Continuous murmurs of a patent ductus arteriosis or bronchial collateral
flow are heard with a single loud second heart sound.
Cyanosis occurs shortly after birth. The ECG in each will
show right ventricular hypertrophy, and radiographs show
decreased pulmonary flow and a normal to enlarged cardiac silhouette. Prostaglandin E1 (alprostadil) infusion is
required until definitive shunt placement.
Tricuspid Atresia
This lesion may be associated with other anomalies

(e.g., ventricular septal defect). The entire systemic
venous return enters the left heart via a patient foramen
ovale or associated atnal septal defect. Cyanosis usually
presents within the first few weeks of life but patients
with a large ventricular septal defect may present with
symptoms of congestive cardiac failure. At least half of
the patients will have a harsh holosystolic murmur along
the left sternal border; the second heart sound will be single. Radiographs reveal a normal heart size with
decreased pulmonary flow. Left axis deviation and left
ventricular hypertrophy are seen on the ECG.
Prostaglandin E1 (alprostadil) infusion is required for the
severely cyanotic infant. Definitive surgical management
Definitive surgical management is to correct the ventricular septal defect and relieve right ventricular outflow
obstruction. The Blalock-Taussig shunt is most commonly used and involves anastomosing the right subclavian artery with the ipsilateral pulmonary artery.
begins with an aorto-pulmonary shunt (e.g., BlalockTaussig), followed by superior vena cava to pulmonary
artery anastomosis (bidirectional Glenn shunt) and a
Pulmonic Stenosis
Epslein's Anomaly
Pulmonic vahular stenosis is the most common form

of right ventricular obstruction. Symptoms depend on the
degree of stenosis, but cyanosis occurs when obstruction
is severe and right to left shunting occurs through a
patient foramen ovale or ventricular septal defect. Radiographs may be normal or show right atrial or ventricular
enlargement with decreased pulmonary vasculature.
Severe cases will show right axis deviation on the ECG.
Milder cases only require medical management with
valvuloplasty or surgical repair for more severe cases.
Antimicrobial prophylaxis for endocarditis is required for
all patients.
This rare condition consists of downward displacement

valve, resulting in tricuspid
regurgitation, decreased right atrial emptying, a poorly
functioning small right ventricle, and right-to-left shunting between the atria. Presentation in infancy implies
severe disease usually associated with pulmonic stenosis.
In many patients, symptoms are mild and fatigue is the
only complaint. The tricuspid regurgitation produces a
holosystolic murmur over the anterior left chest and a
gallop is common. Dysrhythmias such as ventricular
extrasystoles or supraventricular tachycardia are frequent. Large P waves, a right bundle branch pattern, and
caval-pulmonary isolation procedure when the patient is

between 1.5 and 3 years of age.
of an abnormal tricuspid
136 /
EnmncrNcy MrnrcrNr: Tun Conn CunrucuLUM
a prolonged P-R interval are seen on ECG. Radiographs

show decreased pulmonary flow, and right atrial and ven-
tricular hypertrophy produce an enlarged cardiac silhouette. Newborns with cyanosis require prostaglandin infusion until surgery, while control of supraventricular
dysrhythmias is important in the older patient. Those with
mild to moderate disease may need tricuspid valve repair

or replacement as young adults.
Increased Pulmonary Flow
Transposition of the Great Arteries
Transposition ofthe great arteries accounts for 5o/o to

of all CHD but is the most common cardiac lesion in
cyanotic neonates. It is more common in infants of diabetic mothers and in males (3:l). With the aorta arising
from the right ventricle and the pulmonary artery from
the left ventricle, two parallel circuits are formed. The
foramen ovale and ductus arteriosis allow mixture of oxygenated and deoxygenated blood, and about 50% will
also have a ventricular septal defect and present with congestive cardiac failure. Radiographs show an "egg-onside" cardiac silhouette with a narrow mediastinum and
increased pulmonary flow Patients require emergent
prostaglandin E1 (alprostadil) infusion followed by balloon atrial septostomy (Rashkind procedure). Patients
with an intact ventricular septum will have an arterial
switch (Jatene) procedure as a neonate while those with a
7oh
ventricular septal defect can undergo this procedure

within the first 2 to 4 months of life. Intraatrial baffle procedures (Mustard or Senning) have been done in the past
but patients commonly have brady- and tachydysrhythmias along with up to a 9o/o incidence of sudden death as
adolescents or young adults.
Totsl Anomalo u s Pulmonary Venous Return
In this rare lesion, there is no direct pulmonary venous

connection with the left atrium; hence, both systemic and
pulmonary venous blood flows to the right atrium. Right
atrial blood passes either to the right ventricle or via a

patent foramen ovale or atrial septal defect to the left
atrium. Three anatomic classifications exist based on the
location of the connection between the pulmonary venous
return and the systemic venous return: infracardiac,
supracardiac, and cardiac. The site of this connection,
along with amount of pulmonary venous obstruction,
results in three clinical patterns. Severe obstruction to
pulmonary venous return, most often seen with an infracardiac connection, results in neonatal cyanosis and pulmonary edema and requires emergent surgical repair.
Infants with a large left-to-right shunt and mild to mod-
erate obstruction to pulmonary venous return will

develop pulmonary artery hypertension and mild
cyanosis. In patients without pulmonary venous obstruction, pulmonary hypertension is absent and cyanosis is
mild to nonexistent. The ECG shows right ventricular
hypertrophy with tall, spiked P waves. In the patient with
pulmonary venous flow to the left innominate vein and a
persistent left superior vena cava, a "snowman" appearance is seen on chest x-ray due to the supracardiac
shadow. A "scimitar syndrome" has been described in
which the connection between the right pulmonary veins
and inferior vena cava produce a crescent-shaped vertical
shadow in the right lower lung fields on lateral chest radiographs. Patients presenting in pulmonary edema require
diuretics, digitalis, oxygenation, and possibly mechanical
ventilation with positive end-expiratory pressure prior to
definitive surgical repair.
Truncus Arteriosis
A single arterial trunk (truncus arteriosis) originates

from the heart to supply the systemic, pulmonary, and
coronary circulations in this anomaly, which constitutes
less than l% of CHD. Patients also have a ventricular septal defect resulting in admixture of blood. The degree of
cyanosis depends on the amount of pulmonary blood
flow. Over the first month of life, the pulmonary vasculature resistance drops and the infant develops congestive
heart failure with tachypnea, fatigue, and poor growth.
Patients have bounding pulses, a single loud second heart
sound, and a holosystolic murmur from the ventricular

septal defect. Increased flow through the mitral valve can
produce an apical diastolic rumbling murmur. Truncal
valve insufficiency produces a high-pitched, early diastolic decrescendo murmur in the upper right and mid-left
sternal border. Radiographs reveal enlargement of the
cardiac silhouette and pulmonary vasculature. A right
aortic arch is present in almost half of the patients. The
ECG may show right, left, or combined ventricular hypertrophy. The patient in congestive heart failure should be
managed with oxygen, diuretics, and digoxin. Definitive
treatment involves surgical repair of the ventricular septal defect and conduit placement between the pulmonary
arteries and right ventricle (Rastelli procedure), and, if
necessary, repair ofthe aortic arch.
Hypoplastic Left lIeart Syndrome
Usually diagnosed within the first day of life, this syndrome features underdevelopment of the left side of the
heart (left ventricle, mitral and aorlic valves) and

of the ascending aorta. Pulmonary venous
hypoplasia
blood passes to the right atrium via the foramen ovale or

atrial septal defect, and the right ventricle serves to main-
tain both pulmonary and systemic circulations.
Since
these patients are "ductus dependent" for systemic circu-
lation, ductal closure produces hypoperfusion with weak
Cannrov,A.scur-AR DISoRDERS
L37
develop cardiomegaly and increased pulmonary vascularity on chest x-ray. Right ventricular hypertrophy is seen
medium systolic ejection munnur at the left middle to

upper sternal border. Right ventricular hypertrophy and
an rsR' in the right precordial leads are seen on ECG.
on ECG. Surgical options include palliation (Norwood
Radiographs reveal increased pulmonary vascularity and
and Fontan procedures) and heart transplantation.
increasing right atrial and right ventricular size with
or absent peripheral pulses and shock. Patients rapidly
increasing shunt flow. The ascending aorta may be inconspicuous resulting in a triangular cardiac silhouette.
Patients presenting with congestive heart failure should
Acyanotic CHD
Acyanotic heart lesions can be subdivided into those
that produce a volume load or a pressure load on the
heart. Lesions producing a volume load are more com-
mon, with left-to-right shunt lesions predominating.

Lesions producing a pressure load are a result ofobstruction to ventricular outflow or narrowing of one of the
great vessels.
Volume Loud
With all these lesions, a communication exists between

the systemic and pulmonary circulation. Since the shunt
is left to right, fully oxygenated blood returns to the
lungs. Shunt dynamics are determined by resistance and
relative pressures of the pulmonary and systemic circulations as well as the size of the defect.
Atrial Septal Defects

Pqtent Forqmen Ovale
The foramen ovale normally permits right-to-left flow

in utero, and in most infants closes during the first year
of life. From 5o/o to 33oh of adults have a patent foramen
ovale (valvular competent patent foramen ovale). Since
there is no deficiency ofseptal tissue, it is not considered
a true atrial septal defect. Although usually of no hemodynamic significance, conditions resulting in high right
atrial pressures can produce righrto-left
shunting
through the patent foramen ovale.
Isolated atrial septal defects include those at the fossa

ovalis (also known as ostium secondum and is most common), those at the upper atrial septum (sinus venosus),
and those posterior and inferior to the fossa ovalis. Most
with atrial septal defects are
patients should be referred to the cardiologist for definitive evaluation. Surgical repair early in childhood is indi-
cated
for symptomatic patients and those with
shunt
ratios of greater than 2:1.

Ventricular
S eptal D
efect
Accounting for 25Yo of CHD, ventricular septal defect

is the most common cardiac malformation. Defects may
be found in various anatomic locations, but hemodynamics depend on the size of the shunt and the pulmonary
vascular resistance. Decreasing pulmonary vascular

resistance in the first few weeks after birth results in
increased left-to-right shunting in patients with large ventricular septal defects. These patients then present with
pulmonary edema at 6 to 8 weeks of age. Large uncorrected ventricular septal defects result in increasing pulmonary vascular resistance. When the systemic and pulmonary vascular resistance are almost equal, the shunt
becomes bidirectional and cyanosis develops (Eisenmonger complex).
Patients with small defects are asymptomatic but have
a loud harsh holosystolic murmur at the lower left sternal
border. A thdll may be present; the ECG and chest x-ray
are normal. Up to 50% have spontaneous closure of the
defect before 4 years of age. All require antimicrobial
prophylaxis for endocarditis.
Patients with large defects have poor growth, recurrent
pulmonary infections, feeding fatigue, and congestive
heart failure in early infancy. The precordium is hyperdy-
namic and the holosystolic murmur is less harsh.

Increased flow across the mitral valve produces a diastolic rumble. Patients in congestive failure also have an
Isolated Atrial Septal Defects
patients
be treated with diuretics and digoxin. Asymptomatic
asymptomatic
through childhood. With increasing age, the left-to-right

shunt may increase, resulting in development of congestive heart failure and a predisposition to atrial dysrhythmias. A characteristic feature is a widely split and fixed
second heart sound. When the right-to-left shunt ratio is
greater than2'.|, high flow across the tricuspid valve produces a mid-diastolic munnur. Increased flow from the
right ventricle to the pulmonary artery produces
53, rales, and hepatomegaly. Radiographs reveal a prominent left atrium, left ventricle, and pulmonary artery, and
can confirm the presence of congestive heart failure.

Biventricular hypertrophy is seen on ECG. Surgical
repair is indicated for symptomatic infants.
Atri oventric
u I ar S ept al D efe
ct
This group includes the ostium primum defect and the

endocardial cushion defect. The ostium primum defect is
in the lower portion of the atrial septum and usually is

accompanied by a defect in the mitral valve. A left-toright shunt occurs across the atrial defect and mitral
38 /
EnnRcrNcn MsorcrNr: THn Conn Cunrucururr,r
insufficiency is present. Most patients are asymptomatic

but more severe lesions are associated with easy fatigability and recurrent pulmonary infections. Auscultation
reveals a fixed, widely split second heart sound, a rumbling mid-diastolic murmur at the apex, and a pulmonary
systolic ejection murmur. Patients with minimal atrioventricular (AV) valve involvement have minimal symptoms.
Surgical repair is indicated for all defects.
logic closure. In patients that depend on a patent ductus,

such as those with pulmonic stenosis, tricuspid atresia, or
hypoplastic
left heart
syndrome, prostaglandin
(alprostadil) is administered at 0.01 to 0.05
E1
mcgk{min.
Pressure Load
The endocardial cushion defect, also known as the atrioventricular septal defect (AVSD), consists of contiguous
Coarctation of the Aorta
atrial and septal defects and markedly abnormal AV
Constituting 5o/o to 8o/o of CHD, coarctation of the

aorta is a constriction of the descending aorta. Ninetyeight percent occurjust distal to origin ofthe left subclavian artery atthe area ofthe ductus arteriosis (postductal). A bicuspid aortic valve is present in over 70Yo of
patients. Coarctation is the most common CHD associ-
valves. Valvular abnormalities vary; Down syndrome is

associated with the complete form in which there is a sin-
gle AV valve. Left-to-right shunting is both transarterial

and transventricular inAVSD. Symptoms usually develop
early in infancy with failure-to-thrive, recurrent pulmonary infections, and congestive heart failure. The
patients have a hyperdynamic precordium and palpable
thrill, a split 52, a harsh holosystolic murnur at the lower
left sternal border, and a pulmonary systolic ejection
murmur. Mitral insufficiency produces an apical diastolic
rumble.
The patient with a complete AVSD has increased pulmonary vascular markings with large pulmonary arteries
and enlarged cardiac silhouette on chest x-ray. The ECG
shows biatrial and biventricular hypertrophy, left axis
deviation, right ventricular conduction delay, and often

first-degree AV block. Surgical correction is definitive
but may iesult in pacemaker-requiring heart block or
other dysrhythmias, or subaortic stenosis.
Patent Ductus Arteriosis
The ductus arteriosis connects the left pulmonary

artery to the aorta, and normal functional closure occurs
shortly after birth. If the ductus remains patent, high pressure aortic blood flows to the pulmonary system as pulmonary vascular resistance falls. This flow is detrimental
to the normal infant but may be life sustaining in those
with CHD associated with decreased pulmonary flow.
Patients with a small patent ductus are asymptomatic
but may develop heart failure later in life. Those with
large defects exhibit failure to thrive and may present
with congestive heart failure. Infectious endarteritis is a
risk at any age no matter what the size of the ductus.
Examination reveals bounding arterial pulses, and char-
acteristic left upper sternal thrill and a continuous,

"machinery" murmur. Left ventricular or biventricular
hypertrophy is evident on ECG in the presence ofa large
patent ductus. A prominent pulmonary artery with
increased pulmonary vascular markings are seen radiographically.
Surgical closure ofthe patient ductus is a low risk and
preferable treatment
patients. Prostaglandin
inhibitors, such as indomethacin, may permit pharmaco-
in all
ated with Turner's syndrome.
When the narrowing occurs proximal to the ductus

arteriosis (preductal), differential cyanosis occurs as
deoxygenated right ventricular blood flows across the
ductus to perfuse the lower half of the body. Preductal
coarctation is frequently associated with other cardiac
defects and produces heart failure early in infancy. These
patients exhibit failure to thrive and may have a variety of
auscultory findings depending on the accompanying
heart defects. Radiographs reveal pulmonary vascular
congestion and an enlarged cardiac silhouette. The
patient should be stabilized for surgical repair with

furosemide, digitalis, and prostaglandin Er (alprostadil)
infusion.
Patients with postductal coarctation may have only
mild symptoms, such as lower extremity fatigue. Hypertension occurs in the vessels proximal to the coarctation.
The classic finding on physical examination is disparity
in pulses and blood pressures of the arms and legs.
Femoral pulses are delayed compared to radial pulses,
and lower extremity pulses are diminished or absent.

Brachial blood pressure is above the 95th percentile for
age and is higher than blood pressure noted on the legs (a
reversal of normal findings). Cardiac auscultation reveals
a short systolic mid-left sternal murrnur transmitted to
the left intrascapular area. A bicuspid aortic valve results
in a systolic ejection click or
suprasternal notch thrill.
The ECG can be normal or demonstrate left ventricular

hypertrophy. Radiographs show rib notching between the
fourth and eighth ribs in children over 5 years of age.
Indentation of the aorta at the narrowing, along with preand poststenotic dilatation, produces a "figure of three"
sign. MRI is the diagnostic modality of choice. Definitive
surgical therapy should be performed and transient

hypertension is seen in the postoperative period. Occasionally, postoperative mesenteric arteritis (postcoarctectomy syndrome) occurs. Abdominal pain, fever, hyper-
tension, and leukocytosis characteize the slmdrome.

Treatment includes bowel decompression and antihypertensive agents.
Cerurove.scur-A,R DTsoRDERS
Aortic Stenosis
Accounting for 5o/o of CHD, aortic stenosis is found
more often in males (3:l). Stenosis of the aortic valve
occurs in75%o of cases and a bicuspid valve is the most
common defect. A discrete subvalvular membrane is present in 20%o of patients and results in outflow obstruction.
Aortic stenosis may also be supravalvular or the obstruction may be due to asymmetric septal hypertrophy (see
Hypertrophic Heart Disease, below). Clinical symptoms
depend on the degree of outflow obstruction. Presentation in infancy is termed critical aortic stenosis and it is
accompanied by congestive heart failure and shock. More
is asymptomatic until the thir4

fourth, or fifth decade of life. Occasionally, the older
child has a history of easy fatigability, exertional chest
pain, or syncope. Sudden death has been reported with
commonly, the patient
aortlc stenosrs.
Physical findings vary with the degree of stenosis and
anatomic type of lesion. As stenosis progresses, pulses
decrease, a suprasternal thrill appears, and the aortic systolic murmur radiating to the neck becomes louder and
harsher. Radiographs show a prominent ascending aorta.
The ECG is normal but may show left ventricular hyper-
trophy with long-standing severe stenosis. Balloon

valvuloplasty is standard treatment for symptomatic
patients or those with a resting pressure gradient of at
prolapse of the mitral valve is so common, it has been

associated with numerous medical conditions. Patients
are usually asymptomatic but can have chest pain or palpitations. Auscultation reveals a midsystolic click that
becomes more audible with standing or Valsalva. A late
systolic murmur is heard when there is mitral regurgitation. Radiographs and ECGs are normal. Diagnosis is
confirmed by echocardiography. No specific treatment is
indicated. Patients with echocardiographically confirmed
prolapse and an audible murmur of mitral regurgitation
require antimicrobial prophylaxis
for dental,
gastroin-
testinal, and genitourinary surgical procedures.
SELECTED READING
Aghababian RY, ed. Emergency management
Boston: Butterworth-Heinemann, 1994.
of cardiovascular
disease.
Bailey LL, Gundry SR. Hypoplastic left heart syndrome. Pediatr Clin
North Am 1990;37(1): 137 150.
Bank ER. Magnetic resonance of congenital cardiac disease: an update.
Radiol Clin North Am 1993;31(3):553-571.
Behrman RD, Kleigman RM, Arvin AM, eds. Nelson s essentials of pediatlics. Philadelphia: Saunders, 1996.
Burton DA, Cabalka AK. Cardiac evaluation of infants. The first year of
life. Ped ian' C i n North Ant 1 994;4 1 (5):99 I -1 0 1 5.
Crowley Jl Oh KS, Newman B, Ledesma-Medina J. Telltale signs of congenital heart disease. Radiol Clin North Am 1993;3 1(3):573-582.
DiMaio AM, Singh J. The infant with cyanosis in the emergency room.
Pediatr Clin NorthAm 1992;39(5):987 1006.
Flyer DC, ed. Nadas' pediatric cardiology. Philadelphia: Hanley & Belfus,
I
1992.
least 60 mm Hg.
Hypertrophic Heart Disease
/ I39
(2.6.2. 1)
Also known as idiopathic hypertrophic
subaortic
stenosis (IHSS), this defect also includes those patients
with asymmetric septal hypertrophy. The cardiomyopathy
may or may not cause left ventricular outflow obstruc-
tion. Patients may be asymptomatic or have exertional

dyspnea, angina or weakness. Examination reveals a
brisk, often bifid pulse. A crescendo-decrescendo systolic murmur is present, and its intensity increases with
standing or Valsalva (increased preload) and decreases
with squatting (increased afterload). Radiographs and
ECGs are usually normal. Treatment, usually with betablockers, is aimed at decreasing the heart rate and
increasing preload. Dysrhythmias may be treated with
diisopyramide or amiodarone. Agents that increase contractility, such as digitalis or isoproterenol, should be
avoided.
Flynn PA, Engle MA, Ehlers KH. Cardiac issues in the pediatric emergency
room. Pediatr Clin North Am 1992;39(5):955-986.
Garson A, Bricker JT, McNamara DG, eds. The science and practice of
pediatric cardiology. Philadelphia: Lea & Febiger, 1990.
Gillum RF. epidemiology of congenital heart disease in the U.S. Am Heart
199 4 ; | 27 (4) :9 19-927
Giuliani ER, Gersh Bt McGoon MD, et al., eds. Mayo clinic practice of
cardiology. St. Louis: Mosby, 1996.
Hathaway WE, Hay WW, Groothius JR, Parsley JW, eds. Cun'ent pediatric
diagnosis and treatment. Englewood Cliffs, NJ: Prentice Hall, 1993.
Haworth SG, Bull C. Physiology of congenital heart disease. Arch Dis
C hi ld t993 ;68(5):7 07 -7 1 I.
Moss AJ. Clues in diagnosing congenital heart disease. West J lvIed 1992;
156(4):392-398.
Norwood WI Jr. Hypoplastic left heart syndrome. Ann Thorcc Surg
l99l;
52(3):688-695.
Perloff JK The clinical recognition of congenital heart disease. Philadelphia: Saunders, 1994.
Pinsky WW, Arciniegas E. Tetralogy of Fallot. Pediatr CIin North Ant
1990;37 (1):179-192.
ReisdorffEJ, Roberts MR, Wiegenstein JG, eds. Pediatric emergency medicine. Philadelphia: Saunders, 1993.
CARDIAC TRANSPLANTATION (2.7)

Complications
Mitral
Valve Prolapse (2. 6. 2. 2)
Prolapse of the mitral valve is a common condition in

which the valve leaflets bellow into the left atrium during
systole. Patients who are otherwise asymptomatic and

without clinical findings on examination should be considered as having a normal variant of the valve. Because
Over the past 20 years cardiac transplantation has

evolved from an experimental procedure to an accepted
treatment for end-stage heart failure. Approximately
1,600 transplants are performed each year in the United
States, and nearly 3,000 worldwide. One-, five-, and tenyear survival rates are approximately 90o/o, 70o/o, and
140 /
50o%,
Err,mncnNcy MnnrcrNe:
THr Conr CunnrculuM
respectively. As the number of transplant operations
performed each yeat increases, and survival rates

improve, emergency physicians must become increasingly skilled in the evaluation of the cardiac transplant
patient in the ED.
The most common reason for cardiac transplantation is
end-stage heart failure. More recently, however, the pro-
cedure has been performed in patients with refractory

angina, symptomatic cardiac arrhythmias, benign cardiac
tumors, coronary occlusive disease in previously transplanted patients, and for congenital heart defects in pediatric patients.
Physiologically the transplanted heart functions differently from the normal heart in several ways. The most
significant difference is that the transplanted heart

remains denervated, and therefore lacks efferent and
afferent fibers. The resting heart rate is usually higher
(e.g., 90 to 100 beats per minute) due to this lack of
parasympathetic control on the sinoatrial node. Similarly,
cardiac transplant patients depend on circulating cate-
cholamines to increase heart rate and contractility in

order to increase cardiac output in response to stress or
exercise; therefore, transplant patients are instructed to
shift gradually from the supine to a standing position, and
to perform warm-up exercises for 5 to 10 minutes prior to
strenuous physical activity. Cardiovascular drugs should
be used with caution in these patients since these medications may have an altered effect due to denervation.
See Table 245 for the effect of commonly used cardiac
drugs in transplant patients. In general cardiac function of
the transplanted heart is excellent with a nearly normal
left ventricular ej ection fraction.
Advances in immunosuppressive therapy account for
much of the improved survival rate in cardiac transplant
TABLE 2-45. Cardiovascular drugs after cardiac

transplantation
Drug
Effect in recipient
Digitalis
Normal increase in
contractility, minimal
AV nodal effect
None
lncreased contractility
and chronotropy
I ncreased contractility
and chronotropy
Normal increase in
inotrophy and
chronotropy
No vagalytic effect
NormalAV block
No reflex tachycardia
No reflex tachycardia
lncreased antagonist
effect during
exercise
Atropine
Epinephrine
Norepinephrine
lsoproterenol
Quinidine
Verapamil
Nifedipine
Hydralazine
Beta-blockers
Mechanism
Denervation
Denervation
Denervation
Hypersensitivity
Denervation
Hypersensitivity
No neuronal
uptake
Denervation
Direct effect
Denervation
Denervation
Denervation
patients. Most immunosuppressive protocols are divided
into two periods. The first period is the induction phase

and involves the administration of high doses of immunosuppressive drugs for the first few weeks following
surgery to prevent rejection. The second period is the
maintenance phase, during which immunosuppressive
medications are given at lower doses. The most common
regimen currently employed for maintenance consists of
triple drug therapy: cyclosporine, azathiopine, and lowdose prednisone. Other immunosuppressive medications
used, or under study, include antithymocyte globulin,
OKT3, FK 506, rapamycin, RS-61443, and mizoribine.
Successful long-term immunosuppressive therapy

requires depressing the patient's immune response
enough to prevent rejection, but not so much as to invite
infection. Similarly, there are a number of drugs that
interact with cyclosporine, resulting in increased or
decreased levels, or increased nephrotoxicity. Drugs that
increase cyclosporine levels include diltiazem, verapamil, ketoconazole, fluconazole, bromocriptine, methylprednisone, metoclopramide, and erythromycin. Phenobarbital, phenytoin, carbamazepine, and rifampin cause a
decrease in cyclosporine levels. Emergency physicians
should alter the doses or add or withdraw immunosup-
pressive medications only after discussion
with
the
patient's transplant physician.
The leading causes of morbidity and death following

cardiac transplantation are rejection and infection. Other
serious complications include allograft coronary artery
disease, malignancy, and complications associated with
the use of immunosuppressive medications.
Rejection
Allograft rejection remains one of the leading causes

of morbidify and mortality in transplant recipients. Acute
cell-mediated rejection is responsible for the vast majority of such episodes. Rejection may occur at any time, but
90%o occur within the first 6 months; it is relatively
uncommon after I year without significant alterations in
immunosuppression. The most important risk factors for
rejection include female recipient (especially multiparous), human leukocyte antigen (HLA) mismatch, and
female and younger donor hearts.
The majority of patients with acute rejection
are
asymptomatic. Occasionally patients may present with

complaints of fatigue, malaise, or palpitations. Less than
l}Yo of patients with rejection present with hemodynamic compromise. Arrhythmias have been shown to be
associated with rejection, especially of atrial origin; in
general, though, there is poor correlation between patient
symptoms and rejection.
The gold standard for the diagnosis of acute rejection
in adults is endomyocardial biopsy. Echocardiography
may prove useful in adult patients with rejection; dias-
CenotovRsculen Dlsonor,ns
tolic dysfunction appears to be an early and sensitive

finding. Echocardiography is already the primary tool to
diagnose rejection in the neonate and pediatric cardiac
transplant patient due to its less invasive nature. All
patients suspected of acute rejection require admission,
continuous cardiac monitoring, and arrangement for
definitive diagnosis. Standard therapy for acute rejection
involves high-dose corticosteroids, usually methylprednisolone I g IV per day for 3 days. Other treatment measures include the use of cyclosporine and FK 506. The
diagnosis of rejection should be confirmed by biopsy (or
echocardiography in the case of pediatric patients) prior
to initiating treatment; the only exception is the hemodynamically unstable patient. In this situation, vasopressor
agents and empiric corticosteroid therapy may be initiated following discussion with the transplant physician.
TABLE
early or late. The majority of life-threatening infections

occur in the first 3 months following surgery (e.g., early),
when immunosuppression is maximal. During this period
most infections are nosocomial, often catheter-related,
and usually involve Staphl,lococcas species and gramnegative organisms. Late infections (>3 months posttransplant) are less common, and occur in approximately
20Yo of patients per year of follow-up. The lung remains
the most common site of infection in cardiac transplant
patients, followed by blood, urine, the gastrointestinal
tract, and the sternal wound. See Table 246 for a list of
early and late infectious complications.
All cardiac transplant patients with signs or symptoms
of infection require a thorough evaluation. Fever may not
always be present in these patients; similarly, fever does
not always indicate infection (e.g., a sign of rejection).
Bloo4 urine, and sputum should be sent for analysis and
culture. All patients require a chest x-ray. Prophylactic
antibiotics should be initiated in the ED; the specific drug
regimen to be used is best determined in consultation
with the patient's physician.

The total artificial heart has been removed from clinical use except in a few, highly specialized centers. Currently their only role is to serve as a short-time bridge to
cardiac transplantation. Complications such as thromboembolism and infection limit their use as permanent
replacements.
Allograft Coronary Artery Disease

Allograft coronary artery disease, also known as graft
atherosclerosis, transplant coronary artery disease, or
chronic rejection, is the leading cause of death in these
L47
Early and late infectious complications of

heart transplantation
Early (<3 months after transplantation)

Pneumonia
Nosocomial bacteria
Cytomegalovirus (CMV)
Legionella species
Aspergillus species
Streptococcus pneumoniae
Toxoplasma gondii
Rare pathoge ns: Mycobacte ri u m tube rcu loslq herpes
simplex virus (HSV), Candida, Histoplasma, and
Coccidioides
Sternal-wound infections and mediastinitis
Staphylococci
Enteric gram-negative bacilli
Mycoplasma hominis
Mycobacteria
Candida species
CNS infections
T. gondii
Aspergillus or Candida species (brain abscess)
Infection
The use of immunosuppressive medications to prevent
allograft rejection results in an increased risk ofinfection.
Infectious complications are usually categorized as either
246.
Phycomycetes (rhinocerebral phycompycosis)

ucocutaneous infections
HSV
Candida species
Abdominal infections
CMV and HSV (esophagitis and gastroenteritis)
Cholecystitis
Urinary tract infections
Catheter-associated bacterem ia
Late
Pneumonia
Pneumocystis carinii
Nocardia asteroides
CMV
Community-acqui red bacteria
CNS infections
Listeria monocylogenes (meningoencephalitis)
Cryptococcus neoformans (meningitis)
N. asteroides (brain abscess)
M
T.
gondii
JC virus (progressive multifocal encephalopathy)

Skin
Varicella-zoster virus (reactivated)
patients after 1 year posttransplantation. It is detectable

angiographically in 30Vo to 50% of recipients by 5 years
after surgery. Unlike normal atherosclerosis, this disease
is concentric and tubular, and is a diffrrse process that
effects both the proximal and distal coronary vessels.
Risk factors for allograft coronary artery disease include
hyperlipidemia, obesiry donor age, and donor ischemic
trme.
Because the heart is denervated cardiac transplant

patients do not experience angina. Rather, they may present with new-onset or worsening shortness of breath,
chest fullness, or signs and symptoms of congestive heart
failure. Graft failure, AMI, and sudden death, alone or in
combination, are the most common presenting symptoms
of allograft coronary artery disease.
142 /
ElanRcrNcy
Mnolcnr: Tun Coru
Cunruculurr,r
The patient should be placed on supplemental oxygen,

and continuous cardiac monitoring and IV access should
be established. An ECG should be performed and blood
drawn for cardiac enzymes. Echocardiography may
reveal segmental wall motion abnormalities in the presence of ischemia. The only definitive method to make the
diagnosis is coronary angiography.
Because of the disease's diffuse nature, PTCA and
CABG are not usually effective in the treatment of allograft coronary artery disease. Retransplantation is the
only option for the majority of such patients.
SELECTED READING
Hosenpud JD, Morton MJ. Physiology and hemodynamic assessment of the
transplanted heart. In: Hosenpud JD, Cobanoglu A, Norman DJ, Starr A,
eds. Cardiac transplantation. New York: Springer-Verlag,
1991;
69-l 89.
Miller LrWl Long-term complications of cardiac transplantation. prog Cardiov as c Dis 1 99 1 ;33 :229182.
Oaks TE, Wallwork J. Cardiac transplantation: a review. Br J Biomed Sci
1
1993;50:200-21
l.
Petri WA. Infections in heart transplant recipients. CIin Infect Dis 1 994; 1 g:
141-148.
HYPERTENSTON (2.8)
Malignancy
Hypertension is one of the most common illnesses in
The development of malignant neoplasms is a major
side effect of immunosuppressive therapy. The current

estimate of the risk of malignancy after cardiac transplantation is l% to 2o/o per year. The frequency depends
primarily on the specific immunosuppressive drugs utilized. Cutaneous malignancies (usually squamous cell)
are the most common tumors associated with the use of
azathioprine. In contrast the most common tumor linked
to cyclosporine therapy is a unique form of lymphoma,
referred to as posttransplant lymphoproliferative disease.
The incidence of this tumor peaks 3 to 6 months after
transplantation. The clinical presentation of posttransplant lymphoproliferative disease ranges from a mild
lymphadenopathy and fever to widely disseminated disease.
Complications of Immunosuppressive Medications

A number of side effects are associated with the use of
immunosuppressive medications. The two most common, and important, are hypertension and nephrotoxic-
ity. Hypertension occurs in 50% to 100% of cardiac

transplant patients on cyclosporine. It tends to develop
within 4 to 6 weeks after surgery and usually requires
multiple drug therapy to control. Calcium channel blockers and angiotensin-converting enzyme (ACE) inhibitors
are the drugs most frequently used to treat this complication.
Nephrotoxicity
is
another common side effect of

chronic cyclosporine use. The majority of the decline in
renal function occurs within the first 6 months following
surgery. Nephrotoxicity may present as transient acute
renal failure in the first few months following transplantation. After I year renal function tends to remain stable.
The emergency physician must be aware of a number of
medications that exhibit a nephrotoxic synergistic effect
with cyclosporine, including cimetidine, ranitidine,
diclofenac, aminoglycosides, and trimethoprim with sulfamethoxasole.
the world. Approximately 60 million people in the United

States have been found to have a blood pressure greater
than 140/90 or have been told by a physician that they

have hypertension. It is, therefore, a frequent condition
encountered in the ED. Men and blacks have the highest
prevalence of hypertension. Demographic differences
apply to both primary and complicated hypertension.
Severe disease is generally more common in the middleaged with a peak incidence at 40 to 50 years. Improvements in early recognition and treatment of hypertensive
patients over the last two decades have lowered the incidence of complications, but severe and complicated disease still occurs, especially in underserved or noncompliant populations. Since rapid identification and treatment
of hypertension-associated illness may make a critical
difference in patient outcome, the emergency physician
should be thoroughly familiar with all aspects of this condition.
Measurement of the Blood Pressure

The blood pressure should be checked in a standardized manner, with several readings taken over time,
before a definitive diagnosis is made. Blood pressure
may be transiently elevated for a number of reasons in the
ED. In most cases, blood pressure will fall to near normal
if rechecked after pain and anxiety are controlled. The

patient should be relaxed while the pressure is checked.
The arm should be at the level ofthe heart and held by the
examiner. Keeping the arm lowered at the patient's side or
having the patient exert to hold the arm straight out may
increase the pressure by as much as 10%. The equipment
and examiner hands should be warm to avoid spurious
elevations. Ifthe cardiac rhythm is irregular, the average
ofseveral readings is considered the actual pressure. The
higher recording of either arm should be considered the
more accurate reading. While the systolic pressure is usu-
ally easily determined, occasionally Korotkoff's sounds

do not become inaudible with release of cuff pressure,
leading to confusion in diastolic reading. The first muf-
CennrovRscur-AR DTsoRDERS
fling of Korotkoff's sounds is then considered to be the

diastolic pressure.
Definitions
Hypertension is classified by the Fifth Report of the
Joint National Committee on Detection, Evaluation, and
Treatment of High Blood Pressure (JNC-V) according to
the risk of developing cardiovascular and cerebrovascular
complications (Table 247). The diagnosis of hypertension requires a reading greater than 140190 on two occasions at separate sessions. Hypertensive crisis is defined
as severe hypertension (usually greater than 120 diastolic). A hypertensive emergency exists when there is
severe hypertension associated with acute end-organ
damage to the cardiovascular system, central nervous
system, kidneys, or hematologic system. These patients
are appropriately treated with intravenous medications
and admitted to intensive care settings. A hypertensive
urgency exists when there is severe hlpertension, without
evidence of end-organ damage. These patients can have
the hypertension controlled more gradually with oral
medications over 24 to 48 hours.
Hemodynamics and Pathophysiology
Blood pressure reflects a complex
relationship
between cardiac output and systemic vascular resistance.

Cardiac output is defined as heart rate times stroke volume-itself dependent on preload, afterload, and the
inotropic state of the myocardium. Systemic vascular
resistance is determined by neurohumoral control mech-
anisms and renal vascular resistance. Peak pressure

occurs during the systolic part ofthe cardiac cycle during
which the pulsation of flow from the ventricle to the aorta
exerts its maximal effect on the arterial wall. The diastolic pressure occurs when there is runoff of blood from
TABLE 2-47. Classification of blood pressure in adults

aged 18 and older"
Category
Systolic
Diastolic
(mm Hg)
(mm Hg)
<1 30
Normal
1 30-1 39
High normal
Hypertension
1 40-1 59
Stage 1 (mild)
1 60-1 79
Stage 2 (moderate)
1 80-209
Stage 3 (severe)
>210
Stage 4 (very severe)
aPatients not acutely ill or taking antihypertensive
tion.
<85
85-89
90-99
1
00-1 09
110-119
>120
medica-
Modilied from the Fifth Report of the Joint National Committee on Detection, Evaluation, and Treatment of High Blood
Pressure (JNC-V), 1 993.
143
the arteries to lower pressure areas in the circulation

while the heart is in its refilling phase. The circulation
adjusts to forces that ultimately affect the pressure. These
include fluid shifts affecting intravascular volume, sympathetic tone, vascular resistance, and hormonal influences. The latter result from the actions of the reninangiotensin-aldosterone system, adrenal release of
epinephrine and norepinephrine, and pituitary release of
argenlne vasopressln.
There are multiple hemostatic control mechanisms to
prevent injury to the vascular endothelium by higher than
normal pressures. With mild to moderate increases, an
autoregulatory process is activated in which tissue perfu-
sion is maintained by arteriolar vasoconstriction. With

increasing pressures, this system of autoregulation fails
with resulting vascular injury. Fibrinoid material leaks
into the vascular wall and eventually narrows the lumen
with subsequent tissue ischemia.

There is no absolute pressure at which injury occurs.
Individuals with long-standing hypertension may not
experience vascular damage even with very high pressures (>130 diastolic) for short periods of time, if the
pressure rise is gradual. However, in a previously normotensive individual, much lower pressures may precipitate complications. Patients at risk for neurologic, renal,
and cardiac complications are children with acute
glomerulonephritis, young women with preeclampsia,
and patients who discontinue their normal regimen of
antihypertensive medication. Diastolic pressures as low
as 100 mm Hg have been known to precipitate complications in these circumstances.
Many interrelationships determine the ultimate tissue
damage that occurs to vascular beds as a result ofhypertension. To protect critical vascular beds, the arterioles
respond to changes in blood pressure so that changes in
flow are minimized. Autoregulation, present in
most
organs, is most critically functional in the central nervous
system. The variable response of the vasculature to
changes in blood pressure permits constant blood flow
despite wide fluctuations in mean arterial pressure from
60 to 150 mm Hg. However, hypoperfusion, of most concern in the central nervous system, occurs when pressures
fall outside these ranges.
With chronic vascular disease or hypertension, the normal autoregulatory responses are impaired. Normal blood
flow is maintained only in a much narrower range of

mean arterial pressure (MAP) compared with those without vasculopathy. The clinical manifestations of hypoperfusion or hyperperfusion can occur with more modest
fluctuations in pressure. Cerebral hypoperfusion may
occur at mean arterial pressures as high as 110 in those
individuals with chronic systemic hypertension whose
vascular beds no longer function with normal autoregula-
tion. In hypertensive patients, the limits are reset at a

higher level. The physiology of autoregulation accounts
744 t
EurRcnNcv MBucrNn: THn Conr CuRrucurunr
for the nonlinear relationship between blood pressure and
clinical symptomatology. As a general guideline, the

lower limit of cerebral autoregulation is about 25o/obelow
MAP. Therefore, it is usually safe to lower the pressure to
this degree, but not more, in patients requiring rapid pressure reduction.
The desired degree and speed ofblood pressure change
depends on a careful analysis of the risks of hypoperfusion and the potential dangers of persistently elevated
pressures. In some cases immediate reduction is neces-
TABLE 2-49. Drugs to exacerbate hypertension

MAO inhibitors
Sympathomimetics
Cocaine
Anti histami nes/decon gestants
Anticholinergics
Tricyclic antidepressants
Oral contraceptives
Nonsteroid antiinflammatory drugs
Ergotamines
Steriods
sary.
History
The duration of hypertension, compliance with treatment, and past history of hypertensive emergency or
urgency are elicited. Symptoms that may indicate a true
emergency should specifically be sought (Table 248).
Risk factors for cardiovascular disease are important in
deciding the need for intervention. Current or recent use
of medications known to cause hypertension is sought as
well as discontinuance of antihypertensive drugs (Table
249). Concurrent chronic illness may be a predisposition for hypertension. A history of seizures, stroke, focal
neurologic deficits, and altered mentation indicate central
nervous system dysfunction.
Examination
Markedly elevated readings should be checked with
attention to proper technique. Ifstill elevated, differences
in upper extremity pressures should be sought. An examination of the CNS, renal, cardiovascular, and pulmonary
systems, searching for signs of end-organ damage should
be performed. Auscultation of the upper abdomen and

flanks may reveal the bruits of renal artery stenosis. Signs
ofright or left ventricular failure should be sought. The
presence of a sympathomimetic toxidrome may indicate
medication as the cause of the hypertension.
Laboratory Testing
complete blood count, electrolytes, renal function
if
tests, and urinalysis should be performed on all patients

hypertensive emergency is suspected. Proteinuria and
hematuria may indicate renal damage. An ECG should be
obtained to determine the presence of myocardial strain

TABLE
248.
or ischemia. A chest x-ray to evaluate the mediastinum,

heart size, or signs of cardiac failure should be performed. A CT scan of the head is indicated if signs of
CNS dysfunction are present. Toxic screens may confirm
drugs as the cause of the hypertension and may be helpful in those cases suspicious for this etiology.
Acute Hypertensive Crisis (2.8.1) (See 12.3.6)

Conditions Requiring Immediale Lowering of Blood
Pressure (Ilypertensive E mergencies)
The presence of end-organ damage with elevated

blood pressure is a hypertensive emergency. The goal is
to reduce blood pressure within I hour. Diastolic pressures in these cases generally exceed 120 mm Hg. There
are, however, circumstances where end-organ damage
can occur with mild to moderate elevations (children,
pregnant women, sudden elevations in blood pressure in
previously normotensive patients). Less than lYo of all
hypertensive patients ever experience a hypertensive crisis. Given the large number of patients with hypertension,
these individuals represent a significant segment of the
population.
Hyp ert ens iv e Enc
ep
hal op athy
This condition results from the failure of protective

central nervous system autoregulatory mechanisms. The
collapse of normal compensatory systems leads to ischemia and encephalopathy. Advanced age, chronic hypertension, and cerebral vascular disease predispose to this
condition. The brain is flooded with transudate, leading
to cerebral edema. Since carbon dioxide regulates cerebral blood flow, hypercapnea exacerbates the clinical syndrome.
History in the hypertensive patient
Duration of hypertension
Medications and compliance
Symptoms of cardiac ischemia or failure
Symptoms of CNS dysfunction
Recreational drug use
Postmortem examinations reveal petechial hemorrhages, microinfarcts, edema, and mural thrombi of the
brain. Damage to the vessel intima leads to fibrinoid

necrosis. Loss of vessel integrity may be demonstrated by
fundoscopy. Segmental spasm of arterioles, loss of flow,
and retinal hemorrhages and exudates can occur.
Cerurovescur-AR DTsoRDERS
145
Hypertensive encephalopathy usually begins insidiously and progresses over several days. Nausea, vomit-
Very high blood pressure (>120 diastolic) can be lowered by judicious use of agents that optimally have very
ing, aphasia, transient hemiparesis, nystagmus, confu-
few CNS effects and are short acting. Blood pressure

should be reduced gingerly (approximately 20o/o of MAP)
and if there is worsening of neurologic deficit, medication should be immediately discontinued. Sodium nitro-
sion, visual disturbance, seizures, and coma may occur.

Children may present with abdominal symptoms. Vomiting, occasionally projectile, may be the presenting complaint.
The absolute level of blood pressure alone does not
distinguish pathologic processes involved. Hypertensive
encephalopathy is a diagnosis of exclusion. Stroke syndromes (subarachnoid hemorrhage, intracerebral hemorrhage, cerebrovascular thrombosis, or embolism) must be
considered and evaluated by CT or MRI, prior to instituting treatment.
The treatment goal of hypertensive encephalopathy is
to decrease the pressure, allowing inherent autoregulatory mechanisms to function. Precipitous reduction
below the limits of autoregulation may provoke worsening ischemia, stroke, blindness, paraplegia, and myocardial infarction. A25o/o reduction in the mean arterial pressure is generally tolerated without untoward effects.
The ideal drug for this condition maintains cerebral
blood flow, has rapid onset of action and short half-life,
and does not adversely affect the heart or other organs.
Nitroprusside
is the drug of choice for
hypertensive
encephalopathy. The drug can be started in the ED with

automated blood pressure recording every few minutes.
Ultimately, admission to intensive care and insertion of
arterial catheters for continuous blood pressure readings
should occur. Alternatives are labetalol or nicardipine

administered intravenously. Most patients with hypertensive crises are volume depleted and do not require diuretics, unless volume overload is specifically determined to
be present. Reflex volume retention may occur after sev-
eral days
of
nondiuretic antihypertensive
therapy.
Diuretic therapy can then be administered.
prusside or labetalol are the most appropriate. The drugs

must be administered intravenously and require an intensive care setting with arterial blood pressure monitoring.
Subarachnoid and Intracraniul
leed
Bleeding in and around the brain is often associated

with hypertension. This may be a response to increased
intracranial pressure, the result ofirritant effects ofblood
in the CNS, or the result of chronic hypertension. While
it is true that hypertension is associated with an increased
early mortaliry there is no evidence at the present time
that immediate blood pressure reduction improves neurologic outcome or mortality, and the approach to these
patients is controversial. High blood pressure may be necessary to maintain brain viability and avoid ischemia and
vasospasm.
While controversial, some recommend treatment of

hypertension associated with intracerebral bleeding when
systolic pressures exceed 170 in the absence of hydrocephalus or vasospasm. The goal is to reduce the pressure
modestly to the 140
to
160 systolic range with intra-
venous controllable agents. The MAP should be lowered
by no more than 20o/o. The patient should be followed

closely and the pressure permitted to return to elevated
levels if there is deterioration in the clinical status following antihypertensive therapy. The addition of
nimodipine may protect against vasospasm and ischemia.
It has the hemodynamic responses of other calcium channel blockers and may ameliorate the hypertension associated with this disease.
Stroke
Acute Aortic Dissection

Hypertension is the major risk factor for stroke. Longterm control of hypertension reduces the incidence and
severity of cerebral vascular disease. Treatment of hypertension in the poststroke period is, however, controver-
sial. Blood pressure is frequently elevated in acute

stroke. While hypertensive patients have a higher mortality than normotensives, there is little evidence that
acute treatment affects the outcome. The pressure normally declines without specific therapy over several days
after a stroke.
Ischemic stroke produces a zone of severe tissue
hypoperfusion immediately surrounding the blocked vessel. Tissues distant from the central area are subjected to
relative ischemia with potential viability. High cerebral
perfusion pressures are necessary for blood flow into the
ischemic zones.
About 2,000 new cases ofaortic dissection occur annually in the United States; 25% of victims die within the
first 24 hours and 90%o die within a year of presentation.
Hypertension is responsible for more than 50% of the
cases, but Marfan syndrome, Ehlers-Danlos syndrome,
bicuspid aortic valve, coarctation, luetic aortitis, and pregnancy are other causative factors. A sudden tear of aortic
intima with extravasation of blood into the media of the
aorta occurs. Anatomic and hemodynamic factors subject
the proximal aorta to maximal flexion and shearing stress.
With each systole, blood dissects for varying distances in
either direction. Deficits related to loss of proximal aortic
integrity may cause acute myocardial infarction, aortic
valve incompetence and hemopericardium. Interruption

of flow to a branch of the aorta may lead to acute stroke,
746 /
EunncnNcy MrorcrNn:
paraplegia (involvement
of
Tur Conr CunnrculuM
anterior spinal
vessels),
intestinal infarction, or extremity ischemia.

Aortic dissection presents with severe pain that is usually maximal at onset. It is often described as tearing or
ripping. The pain may be present in the anterior chest or
in the interscapular area and is controlled with difficulty.
Depending on the degree of distal aortic involvement,
back pain, abdominal pain, extremity pain, or acute neurologic deficits occur. There may be a significant difference in blood pressure between the upper extremities,
indicating partial compression of a subclavian artery. If
dissection is suspected, diagnosis should be made rapidly
with contrast enhanced Cl transesophageal echo (TEE),
MRI, or aortography.
Blood pressure should be lowered immediately to limit
the extent of dissection. Systolic blood pressure should be
to less than ll0 mm Hg with nitroprusside.

Additionally, the shearing stress (dP/dT.u*) should be
minimized by the administration of beta-blocking agents
such as propranolol, labetalol, or esmolol. Dissection of
proximal and complicated distal dissections require surgical repair while the uncomplicated distal tear can be
reduced
treated medically.
Myocurdial Ischemiu andAcute Pulmonary Edema

Coronary artery disease, increased oxygen demands,
and depressed ejection fractions occur with hypertension.
Hemodynamic demands resulting from severe hypertension can lead to unstable angina, myocardial infarction, or
pulmonary edema.
Therapeutic interventions should reduce cardiac work

and oxygen demand. Preservation of myocardial tissue by
enhancing coronary perfusion is also critical. Decreasing

heart rate, afterload, preload, and wall tension may ameliorate the condition. Nitroglycerin and loop diuretics are
the first-line drugs. Drugs inducing tachycardia should be
avoided.
Hypertension Associated with Spinal Cord Syndromes

Patients with a variety of serious spinal cord diseases
may experience a syndrome of sympathetic overactivify
characlerized by headache, nausea, severe hypertension,
reflex bradycardia, and diaphoresis. Episodes may be
triggered by stimulation of nervous pathways below the
cord lesion. The initial approach is to remove any stimuli
that might be causing the reflex autonomic activity. If that
is not possible or effective, labetalol, sodium nitroprusside, or phentolamine can be used in much the same way
one would approach any patient with sympathomimetic
cholamines. While these are uncommon, they are important to recognize since their treatment is significantly different from other secondary etiologies. Most may be suspected or recognized by a careful, focused history and
examination. A history of medication use and interaction,
or signs of sympathomimetic overactivity are usually present.
Pheochromocytoma
Pheochromocytoma is a rare cause of hypertension.

While it may present with sustained hypertension, it is
classically suspected on the basis of paroxysms of
headache, diaphoresis, palpitations, anxiety, nausea,
vomiting, abdominal pain, and high blood pressure.
Patients with this disorder can be treated with an alphablocker (phentolamine) or labetalol. Nitroprusside is useful in refractory cases. Beta-blockers given without concomitant alpha-blockade may significantly worsen the
hypertension.
Me di c ation W thdrawal Syndro m e s
The sudden cessation ofcentrally acting antihyperten-
sive medications is a common cause of acute severe

hypertension. Clonidine and beta-blockers are the drugs
most commonly implicated. The clinical manifestations
simulate those of pheochromocytoma. If the patient is
asymptomatic and does not meet criteria for a hypertensive emergency, simply restarting the original medication
usually brings about a smooth reduction in blood pressure. Patient education is of utmost importance in preventrng recurrences.
Recreational Drugs
Street drugs with sympathomimetic properties can precipitate severe hypertension. This condition should be
suspected in patients for whom no other explanation is
apparent. Drugs such as amphetamines, PCP, phenyl-
propanolamine, LSD, and diet pills may lead to this syndrome, but the current favorite is cocaine, which produces a wide range of symptoms. Either phentolamine or
labetalol can be used for the treatment of hypertension in
these patients.
Drug Interactions
Patients on MAO inhibitors may experience sudden
overactrvlty.
and severe hypertension when they ingest medications or

foods with tyramine or sympathomimetic properties
Syndromes of Catecholamine Excess
(Table 2-50). These patients may experience sudden

severe hypertension with headache, diaphoresis, and
chest pain. Treatment is usually successful with phento-
There are a number of secondary causes ofhypertension related to the presence of elevated circulating cate-
lamine, labetalol, or nitroprusside.
Cenorovescur-{R DTsoRDERS
TABLE 2-5O. MAO inhibitor interactions
Trycyclic antidepressants
Sympathomimetics
Caffeine
Dextromethorphan
Meperidine
Antihistamines
Foods containing tyramine
Cheese
Wine
Beer
Chocolate
Pickled herring
Yeast
Chicken liver
Broad bean pods
Sauerkraut
Yogurt
Salami
747
been used with success. It is important to note that some

medications commonly used in nonpregnant patients with
hypertensive emergencies are contraindicated during

pregnancy. Nitroprusside may cause fetal cyanide poisoning and ACE inhibitors are not considered safe in pregnancy. Because of the high incidence of maternal-fetal
complications during the treatment of hypertension in

pregnancy, these patients should ideally be monitored in
a setting capable of carefully attending to both mother
and fetus.
Preeclamptic patients require medications to prevent
seizures. Prophylactic medications should be used in

those with diastolic pressures greater than 100 or with
other signs of advanced disease. Magnesium is the preferred drug for this purpose, although dilantin may also
be used.
Conditions Requiring Lowering of Blood Pressure over

24 Hours
Preeclampsia and Eclampsia

Normal pregnancy is characterized by a gradual fall of
the blood pressure to a nadir that occurs at about 20
weeks' gestation. Thereafter, the pressure gradually rises
to normal levels at term. As a result of complex neurohumoral changes during normal pregnancy, the upper limits
of diastolic pressures are 75 mm Hg in the second and 85
mm Hg in the third trimester. Note that this is lower than
the definition of hypertension in nonpregnant adults. An
increase of greater than either 30 mm Hg in the systolic
or 15 mm Hg in the diastolic pressure over baseline values defines hypertension during pregnancy. After 20
weeks' gestation, any patient with blood pressures greater
than 140/90 or who experiences a rise in the blood pressure as stated above are preeclamptic.
Other findings in the preeclamptic patient include proteinuria (+l or greater on dipstick) and edema. These cri-
teria may not occur simultaneously, and all three classic

abnormalities may not be present at the time of diagnosis
in all patients. The blood pressure criteria therefore are
most important.
High blood pressure, occurring in 8oh to l0% of all
pregnancies, may lead to potentially serious complications. If left untreated, fetal demise as well as serious
maternal illness may occur. These individuals are predisposed to abruptio placentae, disseminated intravascular
coagulation, intracranial hemorrhage, and acute liver and
renal failure.
A pregnant patient not previously known to be hypertensive with a blood pressure greater than 105 diastolic
should be considered to have a hypertensive emergency.
Drug treatment should be started with a goal of reducing
the diastolic to 90 to 100 mm Hg, a level at which placental hypoperfusion is unlikely to occur.
Magnesium and hydralazine are the most commonly
used medications for this indication, although others have
Renal Insfficiency
Acute deterioration in renal function may be either a

of severe hypertension. Almost any renal
pathologic process can cause the syndrome. Therapy
should be directed at reducing systemic vascular resistance without compromising renal blood flow. Nitroprusside is effective but cyanide toxicity is more likely in the
cause or result
setting of renal dysfunction. Labetalol and calcium channel blockers are also effective agents. Pure beta-blockers
may decrease renal plasma flow and should be avoided.
Malignant Hyp ertension
When severe hypertension results in the failure of

autoregulation, vascular changes ensue. Loss of arteriolar
and capillary wall integrity leads to exudation of plasma
constituents and obliteration of the vessel lumen. Ultimately, widespread fibrinoid necrosis results. Retinal
hemorrhages, exudates, and papilledema are the primary
clinical manifestations. The fundoscopic changes are usually present bilaterally. The prognosis is unrelated to the
type ofretinal change present. Changes in other vascular
beds lead to hematuria, proteinuria, and, ultimately, renal
failure. As renal function deteriorates, there is activation
of hormonal mechanisms, leading to worsening hypertenslon.
Patients with malignant hypertension present most

commonly with headache (85o/o), which is at its worst in
the morning and is usually occipital or frontal. Blurred
vision, loss of acuity, and blindness may also occur. Confusion, focal neurologic deficits, excessive sleepiness,
depressed mentation, convulsions, and, ultimately, coma
may ensue.
Untreated malignant hypertension has a dismal prog-
nosis. Mortality rates approaching 90% within 1 year
148 / EnrncrNcv MnorcrNo: Tnn Conn Cunnrculurvl

have been reported. Uremia, stroke, congestive heart
fail-
ure, and myocardial infarction account for most deaths.

The degree of renal involvement is a good prognostic
indicator. Although hypertension affects every organ system, signs and symptoms of failure of one organ system
may predominate. The clinical slmdromes often coexist to
varying degrees.
When the evidence of end-organ damage is limited to
fundoscopic changes, the blood pressure can be lowered
more gradually than is necessary in true emergencies.

The goal is to get the blood pressure into a more acceptable range (160 to 180/100 to 10) over 24 hours. This can
often safely be accomplished with oral medications. This
approach may be used even in patients with papilledema.
Once there is improvement of the blood pressure in the
ED, the stable, compliant patient can be discharged on
oral medications as long as follow-up in the next 24 hours
can be assured.
TABLE 2-51. Treatment of hypertension not requiring hospitalization
Adrenergic antagonists
Atenolol, # 25-100 mg/day
Preferential p-antagonist; diminishes heart rate and output and renin levels; recommended agent
for initiation of oral therapy for mild uncomplicated hypertension; full effect may not be manifest
for a week; relatively contraindicated in diabetes, heart block, CHF, asthma, and COPD
Labetalol, # 100-400 mg/bid
or-Fr-, and p2-antagonist; well absorbed with high pass metabolism through liver dosage
adjustment in liver or kidney disease not required; may cause hypotension, fatigue, impotence;
relative contraindications are diabetes, heart block, CHF, asthma, and COPD
Metoprolol, # 50-450 mg/day
Preferential pr-antagonist; maximal effect in 1 week; shorter half-life than labetalol and crosses
blood-brain barrier; relatively contraindicated in diabetes, heart block, CHF, asthma, and COPD
Calcium antagonists
Nifedipine, # oral or sublingual:10-20 mg

Sustained release preparation available
Blocks calcium entry and dilates arterioles with afterload reduction; reduces angina; may cause
severe hypotension and precipitate myocardial and stroke; avoid in aortic stenosis
Verapamil, # 80-120 mgitid
Sustained release preparations available
Blocks calcium entry and dilates arterioles with afterload reduction; reduces angina and
ventricular response in atrial fibrillation; drug of choice for PSVT; interacts with beta-blockers
and digitalis with worsening bradycardia and AV blocks; contraindicated in Wolff-ParkinsonWhite syndrome
Diltiazem, # cardizem CD:180-240 mg/day
Actions similar to verapamil; contraindicated in CHF, AV conduction problems, hypotension, and
liver injury
Angiotensin-converting enzyme antagonists

Captopril, # 25-50 mg/bid or tid
Suppresses ACE in lung, thus reducing angiotensin ll synthesis; also suppresses aldosterone;
drug of choice hypertension with high renin levels, diabetic nephrophathy, and CHF; may cause
neutropenia, severe angioedema, and initial hypotension; prolonged action in renal disease
Lisinopril, # 10-40 mg/day
Mechanism of action similar to other ACE inhibitors; dosage should be reduced in renal failure;
once a day dosing adds to convenience and compliance
Diuretics
Furosemide, 10-80 mg/day
Loop diuretic, inhibiting chloride reabsorption in ascending loop; high potassium losses; drug of
choice in hypertensive emergency, low GFR states, pulmonary edema, and to mobilize large
volumes; may cause severe electrolyte depletion-potassium, sodium, and increase calcium,
uric acid, and blood sugar levels; increases effects of ototoxic and nephrotoxic drugs
Hydroch loroth iazide, 1 2.5-50
m giday
Thiazide diuretic, inhibits reabsorption of sodium and chloride in ascending loop and proximal
distal tubule; ideal starting agent for mild hypertension; causes hypokalemia, hyponatremia,
hyperglycemia, hyperuricemia, hypercalcemia, oliguria; decreases placental flowcontraindicated in pregnancy
Spironolactone, 50-1 00 mg/day
Aldosterone antagonist causing sodium excretion, sparing potassium; used with thiazide diuretics
in cirrhosis and nephrotic syndrome; may cause hyperkalemia and endocrine problems like
acne, hirsutism, and gynecomastia; avoid in diabetics
Cennrovescut-AR DTsoRDERS
Severe Hypertension in the Asymptomatic Patient
Some patients have markedly elevated blood pressure
with no evidence of end-organ damage. The approach to

these patients in the ED is controversial. A significant
number of patients experience a reduction in blood pressure with simple, nonpharmacologic measures such as
149
patients. Optimally the patient should be started on normal maintenance antihypertensive therapy and follow-up
arranged in accordance with the recommendations of the
JNC-V (Table 2-52). The elderly, and patients with cardiac disease or volume depletion, should be started on
lower than usual doses to prevent side effects oforthostasis and tissue hypoperfusion.
rest and control ofpain and anxiety. Furthermore, severe
complications (myocardial infarction, stroke) may occur

with overly aggressive therapy.
The major effort in the ED is to rule out end-organ
injury. A careful history and physical examination are
augmented by ancillary tests such as an ECG, renal func-
tion tests, urinalysis, and a chest x-ray. Ifno evidence of

end-organ damage is found, the patient can be started on
oral antihypertensive therapy and follow-up arranged.
Treatment can be initiated from any of the major
classes
of antihypertensive medications available (Table
2-51). Reducing blood pressure to normal levels rapidly

has not been shown to decrease complications in these
Medications Used in the Treutment of Hypertensive

Emergency
There are now a wide variety of agents available for the
treatment of all stages and degrees hypertension. When
serious elevations ofblood pressure occur in association
with end-organ damage or one of the several conditions
mandating immediate control, intravenous drugs with
short half-lives and predictable responses should be used.
While several oral preparations are available that can
lower blood pressure rapidly, the therapeutic response is
TABLE 2-52. Drugs recommended in the treatment of hypertensive emergencies

Drug
Dose arld route
Nitroprusside
lnf
Esmolol
lnfusion:200-500 pg/kg/min
x 4 min, then 50-300 pg/kg/min
Onset
Duration
usion: 0.25-1 0.0 pg/mg/min

<1 min
2-5 min
Direct vasodilator; drug of choice for all conditions except eclampsia; titratable; needs arterial line;
may cause cyanide toxicity
'l min
Nitroglycerin
lnfusion:5-100 pg/min
2-1 0 min
Direct vasodilator; drug of choice for coronary ischemia and pulmonary edema; titratable; decreases
myocardial work; causes headache
Labetalol
lnfusion: 0.5-2.0 mg/min
2-10 min
2-G hr
Bolus:20-80 mg/10-15 min to
maximum of 300 mg
crr-, Fr-, Bz-antagonist; drug of choice for aortic dissection; ? ellicacy in patients on p-antagonists;
reduces blood pressure without reflex tachycardia (Br-antagonism); well absorbed, highiirst pass
liver metabolism; dose reduction unnecessary in hepatic/renal failure; relatively contraindicated in
diabetes, heart block, CHF, asthma, and COPD
1-2
min
10-30 min
Br-antagonist; tritratable and short acting; hypotension; contraindicated in heart block, bronchospasm,
diabetes, and hypertension due to cocaine
Phentolamine Bolus:5-15 mg lV push

1-2 min
30-90 min
or-, cr2-antagonist; drug of choice for catecholamine crises (pheochromocytoma, MAO crisis);
Raynaud's disease; severe orthostasis; arrhythmogenic; sexual dysfunction
lnfusion: 5 mg/hr, increasing by
5-10 min
1-4hr
2.5 mg every 5 min to
15 mg/hr max
Calcium antagonist; contraindicated in CHF and aortic stenosis; increases ICP; causes tachycardia,
headache, and hypotension
Enalaprilat
Bolus:1.25-5.0 mg q6hr
10-15 min
4-6 hr
ACE inhibitor; rapid fall in blood pressure in hyperreninemia; angioedema; cough; hypotension;
marrow depression; contraindicated in pregnancy; may result in severe hypotension if relative
hypovolemia exists
Magnesium sulfate Bolus:4-O g of 10% soln.
2-5 min
5-1 0 min
lnfusion:1 g/hr
Drug of choice for eclampsia; may precipitate respiratory depression; renal failure
Hydralazine
10-20 mg lV or lM
10-15 min
1-6 hr
Preeclampsia only indication; may precipitate angina and tachycardia and elevate ICP and dP/dT
Nicardipine
These drugs are administered intravenously and require monitoring with arterial cannulation in intensive care, although treatment can be initiated in ED.
50 /
EurncrNcv MrorcrNr: THn Conn CunrucuLUM
not controlled, and serious hypotension and critical organ

hypoperfusion may occur (Table 2-52).
Other side effects are tachycardia, nausea, vomiting, diarrhea, and headache.
Sodium Nitroprusside
Prehospital Care of the Patient with Severe

Hypertension
Sodium nitroprusside (SNP) is a potent direct-acting

vasodilator, reducing preload by venodilatation of capacitance vessels and afterload by general arteriolar dilatation. Its effects are manifest within a minute of commencement of infusion, and blood pressure response can
be closely titrated, which makes it an ideal drug for acute
hypertensive emergencies. SNP decreases vascular resistance and pulmonary wedge pressure, has no appreciable
effect ofcardiac output or rate, and increases cerebral and
renal blood flow
Nitroprusside is metabolized to cyanide and then to
thiocyanate in the liver. Thiocyanate is excreted in urine.
Liver disease will therefore result in cyanide toxicity
while those with impaired kidney function may demon-
strate significant thiocyanate levels and toxicity. Prolonged administration of SNP, generally beyond 72 hours,
in high doses increases risk of toxiciry and levels of
cyanide and thiocyanate should be monitored. Treatment
should by augmented with antihypertensive agents from
other classes of drugs (SNP reacts adversely with cloni-
dine and methyldopa). Intravascular volume should be

maintained. Common side effects are hypotension, nausea, vomiting, apprehension, delirium, convulsions, muscle spasms, and tinnitus.
Prehospital patients are often anxious and in pain. Relative hypertension is therefore common in this group. In
general, the same principles of care should apply to this
patient population as it does to those in the ED except
that much fewer diagnostic and therapeutic alternatives
are available. The patient who is hypertensive but asymptomatic can be transported without specific treatment of
the hypertension per se. Reassurance and pain reliefoften
ameliorate exaggerated responses from those conditions.
Patients with potential cardiac ischemia should be

assessed for reversible pathophysiologic responses,
which can influence the ischemia, including hypertension. Most prehospital ALS units have sublingual nitroglycerin available. This drug has vaso- and venodilating
properties that generally lower blood pressure while
reducing ischemia, and should be administered liberally.
Patients with cerebral ischemia comprise a diverse
group whose treatment is controversial in any setting. It is
often impossible to identif' the lesion causing neurologic
symptoms without CT or MRI. In the fiel4 it is best to
avoid aggressive lowering of the blood pressure as such
treatment could adversely impact cerebral perfusion.
Chronic Hypertension (2.8.2)

Labetalol
Labetalol is an
ot-, Fr-, and Bz-antagonist. It
reduces
not only blood pressure but also the shearing forces on

the proximal aorta. It is useful in the treatment of aortic
dissection and hypertension due to high catecholamine
levels (pheochromocytoma, antihypertensive drug withdrawal). It can be administered as a continuous infusion
or in intravenous boluses or orally. It may precipitate
severe hypotension, so close monitoring of the patient is
essential. It is relatively contraindicated in patients with
reactive airway disease, CHE, diabetes, or atrioventricular
block.
Phentolamine
Phentolamine is a pure c-antagonist, with most affinity for cr,1-receptors, and is the drug of choice for hypercatecholamine states. It is also the drug of choice for
crises due to pheochromocytoma, MAO inhibitor reactions, clonidine withdrawal, cocaine and amphetamine
intoxication, and overdose of epinephrine preparations. It
can be administered as a bolus or as an infusion and may
cause profound hypotension in volume-depleted patients.
Hypertension is one of the most common diseases in

adults throughout the world. Many studies over an
extended period of time have conclusively demonstrated
the importance of even mild blood pressure elevation as a
risk factor for vascular disease. Treatment is now widely
available and effective in ameliorating some of the serious effects of chronic hypertension. It is therefore important for all health care providers to identifu patients at
risk.
DeJinitions
The Joint National Committee on The Detection, Evaluation, and Treatment of Hypertension (JNC-V) recently
analyzed the literature regarding hypertension and its

treatment. The committee defined categories of illness
based on the level of elevation of blood pressure and
underlying cardiovascular risks (Table 247). There are
four stages of illness: mil4 moderate, severe, and very
severe. At nonurgent levels, two readings, taken at separate visits, are required for definitive diagnosis. Recommendations for treatment are based on the stage of disease and associated conditions. Control ofblood pressure
Ce-norovescut-{R DTsoRDERS
does not totally eliminate cardiovascular and cerebrovas-
/ l5l
Screening for Hypertension in the ED
cular risk. Therefore, modification of other risk factors

should accompany the efforts made to reduce blood pressure.
Essential (2.8.2.1)
Most patients with hypertension do not suffer from

secondary causes. Blood pressure elevation, not related to
discoverable renal, adrenal, hormonal, vascular disease,
or transient conditions such as anxiety or pain, is called
essential. Even though the illness is unrelated to these
pathologic processes, it may cause serious vascular disease if left untreated over time.
Many disparate factors are associated with essential
hypertension-race, age, sex, obesity, diet, and tobacco
use. There are a number of pathophysiologic subsets.
Some patients have heightened arteriolar tone. Mild

hypovolemia may ensue with compensatory tachycardia. Calcium channel antagonists are particularly useful
in these patients. In some patients there is a high degree
of resting sympathetic tone resulting in a hyperdynamic
circulation with tachycardia and increased cardiac output. These patients are sensitive to drugs that blunt
adrenergic response. A third group of patients may be
hypertensive based on renovascular mechanisms. Multiple factors cause the kidney to produce higher quantities
of renin. High levels of angiotensin II result in hypertension; this subset of patients responds best to ACE
antagonrsts.
Secondary (2.8,2.2)
For a small percentage of patients, hypertension has an
underlying renal, cardiac, metabolic, or hormonal basis.
Identification is important since it may allow for tailored
therapy, such as surgery or specific medications. One
should be suspicious of secondary causes in patients who
are young at onset, or who have severe or refractory disease. Sudden deterioration in patients whose pressure has
been previously well controlled, and the presence of
The evaluation, monitoring, and treatment of hyperten-
sion is now the most common reason for a visit to a

physician in the United States. Since a large percentage
of the adult population has unrecognized hypertension,
the ED is an important locus for detection, patient educa-
tion, and, when appropriate, initiation of treatment for

this condition.
The Hypertensive Patient Who Presents for
Evaluation of an Unrelated Condition
Many patients are found to have elevated blood pressures in ED triage. The same principles apply to patients
who are known to be hypertensive as to those who are
unaware of the problem. If the patient is asymptomatic

and the reading is not so high as to require a search for
end-organ damage (2101120 or greater), then the patient
should be treated for anxiety and pain and be permitted to
rest if possible. Some readings will be higher in the ED
setting and may not accurately reflect true ambulatory
levels. Patients should be encouraged to continue their
antihypertensive medications and to take them regularly.
Elevated blood pressure in those individuals not known
previously to be hypertensive should be rechecked in the
ED before discharge. If still elevated, but unassociated
with a condition requiring immediate treatment, followup should be arranged according to the recommendations
of the JNC-V (Table 2-53).In those with very high pressures, it may be appropriate to begin antihypertensive
medication. The patient should have a brief history, physical examination, and routine labs (BLIN, creatinine,
lytes, U/A, ECG, and CXR) prior to the initiation of treatment in the ED. Oral medications suggested for the treatment of these individuals are found in Table 2-51. A call
to the primary care physician who will ultimately care for
the patient may be appropriate so that preferences and
formulary requirements for long-term medication can be
discussed.
abdominal and flank bruits also indicate secondary

causes. Consideration should be given to the possibility
of pheochromocytoma, renal failure or renal vascular disease, volume overload, adrenal adenoma, coarctation of
the aorta, medications (sympathomimetics, caffeine,
cocaine, amphetamines), and medication withdrawal in

those individuals with a history or physical examination
suspicious for these conditions. These patients should be
encouraged to discontinue any medications or drugs that
might contribute to the hypertension. They should be

carefully educated about the need for follow-up evaluation for secondary causes of their illness, and that followup should be secured.
TABLE 2-53. JNC-V recommendations for approach to

patients with hypertension on initial screening
Systolic
Diastolic
30
130-'139
1 40-1 59
1 60-1 79
85-89
90-99
<1
80-209
>210
<85
00-1 09
110-119
>120
Recommendations
Recheck
Recheck
Recheck
Evaluate
Evaluate
Evaluate
in 2 years
in 1 year
in 2 months
or refer within 1 month
or refer within 1 week
or refer immediately
Modified from the Filth Report of the Joint National Committee on Detection, Evaluation, and Treatment of High Blood
Pressure (JNC-V), 1 993.
152 /
ElmncrNcy MsnrcrNn: Tun Coru CunrucuLUM
IABLE 2-54. Classification of hypertension at the 90th

percentile for children by age (significant/severe
hypertension)
Age
Calhoun DA, Oparil S. Treatment of hypertensive crisis. N Engl J Med
Systolic bp
0-7 days
>96/1 06
8-30 days
>1041110
>1121118
>1161124
<2 years
3-5 years
6-9 years
Diastolic bp
16-18 years
>74182
>1261134
>76/84
>78/86
>82/90
>136/144
>142/150
>86192
>92198
Modified from Miller K. Pharmacological management of

hypertension in paediatric patients. Drugs 1994;48(6).
Hypertension in Children
The incidence ifhypertension in children ranges from
lohto l0% depending on the population studied. Significantly elevated blood pressure should be identified in
children and correlated with the 90th percentile levels for
each age group (Table 2-54). Except
in
adolescents,
where essential hypertension prevails, children are much

more likely to have a secondary cause. While most adolescents are asymptomatic, children may present with
nonspecific symptoms such as poor feeding, growth failure, and behavioral abnormalities. Rapid rise in blood
pressure as seen in acute glomerulonephritis may lead to
initial manifestations. In general,

the same medications can be used to treat hypertensive
emergencies in children as are used in adults. Nitroprusside, labetalol, phentolamine, and nicardipine are the
nausea and vomiting as
firstline intravenous medications recommended.

Complications of Antihypertensive Therapy
Antihypertensives are among the most widely prescribed medications in the world. They may lead to a
variety of serious side effects that may precipitate ED
visits. Diuretics can cause electrolyte
disturbances
(hypokalemia, hyponatremia, metabolic alkalosis) and

volume depletion. Patients can have profound symptomatology from these side effects. Beta-blockers and calcium channel blockers can cause hypotension or bradydysrhythmias. Alpha-blockers and ACE inhibitors can
lead
to
hypotension, especially
with
concomitant
diuretic use. ACE inhibitors are associated with renal

failure, angioedema, and hypotension. Sudden withdrawal of beta-blockers, clonidine, or calcium channel
blockers can cause severe hypertension, dysrhythmias,
cardiac ischemia, or myocardial infarction. Patients on
these medications who develop symptoms should be
assessed for these side effects and then treated based on
the symptomatology and laboratory

found.
1990;323:17.
abnormalities
with
An idea whose time has come-and gone [editor-
Fagan TC. Acute reduction of blood pressure in asymptomatic patients
severe hypertension.
>122/130
10-12 years
13-15 years
SELECTED READING
iall. Arch Intern Med 1989;149:2169-2170.

Gifford RW Jr. Management of hypertensive crises. JAMA 1991;266:6.
Houston MC. Pathophysiology, clinical aspects, and treatment ofhypertensive crises. Prog Cardiovasc Dis 1989;32:2:99-148.
Joint National Committee on Detection, Evaluation, and Treatment of High
Blood Pressure. The Fifth Report of the Joint National Committee (JNCY). Arch Intern Med 1993;153:152t 183.
Kaplan NM. Management of hypertensive emergencies. Lancet 1994;
344(
l2):1335-l 338.
Kaplan NM, Rose BD. Hypertension. UpToDate in Medicine, computer

laser optical disk, Vol. 3. Wellesley, MA:BDR-UpToDate, 1995.
Littenberg B. A practice guideline revisited: screening for hypertension.
Ann Intern Med 1995;122(12):937 -939.
Powers WJ. Acute hypertension after stroke. The scientific basis for treatment decisions Nern'ology 1993 :43:461467 .
Probst BD. Hypertensive disorders of pregnancy. Emerg Med Clin North
Am 1994;12:l:73-89.
Weber MA. Controversies in the diagnosis and treatment of hypertension: a
personal review of JNC Y. Am J Cardiol 1993;72:3H-9H.
PRIMARY TUMORS OF THE HEART (2.9)

Benign Tumors
Myxomas
Primary tumors of the heart are rare, comprising only
0.001% to 0.5oh of routine postmortem examinations.
Four-fifths of these tumors are benign. Myxomas, the
most common of these tumors, are generally found in 30to 60-year-olds and are primarily atrial in location. Ventricular myxomas are more common in children than
adults.
Pathologic examination of myxomas show them to be

gelatinous masses that are pedunculated, polypoi( and
friable. They are made up of a mucopolysaccharide myxoid matrix with endothelial mesenchymal cells. Clinically, three-fourths are in the left atrium, usually originating from the area of the fossa ovalis. About 20oh are
found in the right atrium and the remainder in the right or
left ventricle.
Clinical manifestations in 90% of cases of myxomas
generally include systemic illness including fatigue,
fever, weight loss, arthralgias, rashes, clubbing of the fingers, and Raynaud's phenomenon. Laboratory findings
may show a vaiety of abnormalities including anemia
(generally hemolyic), leukocytosis, thrombocytopenia,
an elevated sedimentation rate, and an elevated IgG. The
other major clinical manifestations result from tumor
embolization, which occurs'in 30oh to 60Yo of patients.
These emboli most commonly flow to the brain (50% of
cases when embolization occurs), kidneys, and extremities. Large tumor emboli may become lodged at the aortic bifurcation. In patients who have normal sinus rhythm,
embolization from a left atrial myxoma should be con-
Cerol.ovesculAR DTsoRDERS
sidered ifbacterial endocarditis has been ruled out. Retinal artery embolization may occasionally occur and generally is associated with ipsilateral middle cerebral artery
embolization and neurologic abnormalities. These retinal
artery emboli may cause transient or permanent loss of
vision. Coronary artery emboli are rare, but a myocardial
infarction may be the first evidence of a left atrial myxoma.
Left atrial myxomas may also have symptoms associated with their intercavitary location and the mobility of
the tumor, which may temporarily create blockage of the
mitral valve or pulmonary vein orifices. This produces
symptoms of pulmonary venous hypertension and righr
sided heart failure that may include dyspnea on exertion,
paroxysmal nocturnal dyspnea, orthopnea, pulmonary
edema, cough, hemoptysis, palpitations, fatigue, peripheral edema, and chest pain. If there is a transient, complete obstruction, syncope may occur.
The physical examination of patients with left atrial
myxomas may reveal a loud first heart sound that is frequently split. There may also be what sounds like an early
third heart sound. This sound comes between the normal
locations ofthe second and third heart sounds and represents the "tumor plop" as the tumor shifts. In addition to
these auscultatory findings, there may intermittently be
the sounds of mitral stenosis (unlike that of rheumatic
mitral stenosis, which generally is constant).
The electrocardiograms in cases of left atrial myxoma
generally reveal normal sinus rhythm with or without
signs of left atrial hypertrophy. The chest radiographs
may show left atrial enlargement and findings of pulmonary venous congestion. The diagnostic test of choice
is the two-dimensional echocardiograph, which shows all
four chambers. Since myxomas may be multiple and may
involve more than one chamber, the four-chamber
echocardiograph is preferred to the M-mode echocardiograph. In addition, the two-dimensional echocardiograph
helps to distinguish a left atrial myxoma from an intraatnal thrombus (which is more common). CT of the heart
and MRIs have been also been used for diagnosing myxomas. There may be a danger in using arteriography for
diagnosis since the catheter may dislodge parts of the
myxoma, which may embolize.
The differential diagnosis of a left atrial myxoma
includes mitral valvular disease and" when there is a predominance of systemic symptoms, infective endocarditis
and vasculitis.
Right atrial myxomas make up about 20%o of cardiac
myxomas. These tumors are frequently associated with
constitutional symptoms and symptoms of low cardiac
output. Commonly, they are associated with tricuspid
valve obstruction. The clinical manifestations of these
tumors includes jugular venous engorgement with prominent "a" waves. Right-sided heart failure may occur when
the tumor has been present for some time. Symptoms in
patients with this condition may include fatigue, dyspnea
/ I53
on exertion, clubbing, and cyanosis. Importantly, these

symptoms may be episodic and may change with body
position. The associated findings are peripheral edema,
hepatomegaly, and ascites. When pulmonary emboli
occur, they may be small and there may be relatively few
symptoms. Embolization from right atrial myxomas is
less common than from left atrial myxomas. A calcified
right atrial myxoma may directly damage the tricuspid
valve producing signs and symptoms of severe tricuspid
regurgitation.
The electrocardiographic findings of right atrial myxomas generally reveals normal sinus rhythm, but there may
also be atrial fibrillation, right bundle branch block, right
atrial hypertrophy, low voltage, or right axis deviation. As
with myxomas of the left atrium, right atrial myxomas are
diagnosed using two-dimensional echocardiography.
The differential diagnosis of right atrial myxomas

includes rheumatic tricuspid valve disease, but isolated
rheumatic tricuspid valve disease is rare, and there is usually evidence of mitral valve or aortic valve involvement.
Atrial fibrillation is more common with rheumatic tricuspid valve disease, while normal sinus rhythm is more
common with a right atrial myxoma.
Left ventricular myxomas make up about 5oh of myxomas and are associated with syncope and chest pain.
There may be evidence of aortic stenosis due to outflow
tract obstruction. Echocardiography is diagnostic.
Right ventricular myxomas are rare and may produce
evidence of either pulmonary valve or tricuspid valve
obstruction. Symptoms may be constitutional or may
involve syncope, dyspnea on exertion, or symptoms compatible with pulmonary embolization.
The treatment for myxomas (no matter from which
chamber they may arise) is surgery, which generally is
curative.
Other Benign Tumors
Other benign cardiac tumors are very rare. These

include rhabdomyomas and fibromas. The rarest of these
tumors include mesotheliomas, papillary fibroelastomas,
lipomas, hemangiomas, and congenital benign thyroid
rests.
Rhabdomyomas are rarely seen in adults, almost

always occurring in children. Thirty percent of these are
associated with tuberous sclerosis and,90o/o are multiple.
Fibromas may occur at any age and tend to be solitary.
These tumors are found on the valves and the associated
symptoms depend on which valve is involved. These

tumors are associated with dysrhythmias and sudden
death. Treatment is surgical.
Malignan-r{umors
Almost all malignant cardiac tumors are sarcomas,

generally hemangiosarcomas. Affected individuals are
754 /
ElrencrNcy MporcrNn: TnE Conr CunnrculuM
usually male adults from the ages of 30 to 60. These sarcomas infiltrate the heart and pericardium and metastasize. Three-fourths of patients have distant metastases at
the time of diagnosis of a cardiac sarcoma. Symptoms
vary with the part of the heart involved. Primarily myocardial tumors produce congestive heart failure. If the
tumor grows primarily in the chambers, symptoms of
obstruction predominate. If the pericardium is involved
pericardial effusion and associated symptoms develop.
The presumptive diagnosis is made with echocardiography. Following biopsy for confirmation, treatment is palliative.
Other malignant cardiac tumors include
rhab-
domyosarcomas, fibrosarcomas, liposarcomas, primary

malignant lymphomas, and fibrous histiocytomas.
Pericardial
Primary pericardial tumors include pericardial cysts,
teratomas, and mesotheliomas. Pericardial cysts are the
most frequent benign tumor of the pericardium. These

cysts are usually an unexpected finding on routine chest
radiographs even though they may cause chest pain,
tachycardia, dyspnea, and a cough. The most common
location on a chest radiograph is the right costophrenic
angle. Teratomas occur in infants and children, particularly in females. Treatment is surgical and they are rarely
found to be malignant. Mesotheliomas are malignant and
yield a poor prognosis. The mesotheliomas may present
with signs of constriction of the pericardium and vena
caval obstruction.
SELECTED READING
Arciniegas E, Hakimi M, Farooki ZQ, et al. Primary cardiac tumors in children. J Thoracic Cardiovasc Surg 1980;79:.591.
Cheitlin MD, McAllister HA Jr, de Castro CM. Myocardial infarction without atherosclero sis. JA MA 197 5 ;23 1 :95 1-9 59.
Feigin DS, Fenoglio JJ, McAllister HA, Madewell JR. Pericardial cysts: a
radiographic-pathologic correlation and review. Rodiology 1977 ;125:
15-20.
Frenay JJ, Bonte J, Franken P, et al. Left atrial myxoma with left retinal
emboli, right hemiparesis, and myocardial infarction: neurologic and
echocardiographic diagnosis: surgical treatment. Acta Neurol Belg l98l:'
81:215.
Glasser SB Bedynek JL, Hall RJ, et al. Left atrial myxoma: report
ofa
case
involving hemodynamic, surgical histologic and histochemical characteristics. Am J Med 1869:50:113.

GoldschlagerA, Popper R, GoldschlagerN, et al. Right atrial myxoma with
right-to-left shunt and polycythemia presenting as congenital heart disease. Am J Cardiol 1972;30:82.
Hall Rl Cooley DA, McAllister H, Frazier OH. Neoplastic heart disease.
In: Schlant RC, Alexander RW eds The heart, arteries and veins (8th
ed). New York: McGraw-Hill, 1,994;2007 J009.
Harvey WP. Clinical aspects of cardiac tumors. Am J Cardiol 1968;21:328.
McAllister HA Jr. Primary tumors and cysts of the heart and pericardium.
In: Harvey WP, ed. Current problems in cardioLogy. Chicago: Year Book
Medical, 1979.
McAllister HA, Fenoglio JJ. Tumors of the cardiovascular system In:

McAllister HA, ed. Atlas of tumor pathology, fascicle I 5, second series.
Washington, DC: Armed Forces Institute of Pathology, 1978.
Meller
Teichholz LE, Pichard AD, et al. Left ventricular myxoma:
echocardiographic diagnosis and review of the literature. An J Med

1977:'63:816.
O'Neil MB, Grehl TM, Hurley EH. Cardiac myxomas: a clinical diagnostic challenge. Am J Sttrg1'979 138:68-76.
Peters MN, Hall RJ, Cooley DA, et a[. The clinical syndrome of atrial myxomas. JAMA 197 4;230:695.
Roudaul R, Pouget B, Videan P, et al. Right atrial myxoma in an asymptomatic child: echocardiographic diagnosis. Eur Heart J 1980;1:453.
Schlattenberg T. Echocardiographic diagnosis ofleft atrial myxoma. Malo
Clin Proc 1968;43:62O.

Silverman NA. Primary cardiac tumors. Ann Surg 1980; 191 : 127.
Snyder NS, Smith DC, Lau FY, et al. Diagnostic feature of right ventricular myxoma. Am Heart J 1976;91:240.
Sutton D, Al-Kutoubi MA, Lipkin DP. Left atrial myxoma diagnosed by
computerized tomography. Br J Radiol 1982;55:80.
Sutton MGS, Mercier LA, Giuliani ER, et al. Atrial myxomas: a review of
clinical experience in 40 patients. Mayo Clin Ptoc'1980;55:371.
Talley RC, Baldwin Bl Symbos PN, et al. Right atrial myxoma: unusual
presentation with cyanosis and clubbing. Am J Med 1970;48:256
Williams DB, Danielson GK, McGoon DC, et al. Cardiac fibroma: longterm survival after excision. J Thorac Cardiovasc Surg 1982;84:230.
Yitling FP, Schlant RC, Hertzler GL, Krzyaniak R. Peri-cardial mesothelioma. Chest I 982;8 I :520-523
MYOCARDIAL MANIFESTATIONS OF
SYSTEMTC DISEASES (2.10)
A number of systemic diseases can adversely affect the
cardiovascular system, and the consequences can be devastating. hnmunologically mediated diseases can damage
the heart and vessels. Infections can attack the heart
directly or secondarily damage the cardiovascular system
through the immune response to the infection. Metabolic
derangements affect tissues throughout the body, including the heart and blood vessels. Toxins can have a variety
of cardiovascular consequences. A discussion of systemic
illnesses that can damage the heart and vessels could fill
a volume, and this discussion will be limited to the more
common disorders with emphasis on those that can have
an emergency presentatlon.
Infections (2.10.1)
Early (Endocarditis Secondary to Sepsis) (2.10,1,1)

Infective endocarditis can present in a plethora of
ways. Most commonly bacterial, it can be caused by fun-
gal, rickettsial, collagen-vascular, sterile, or thrombotic

factors. It primarily afflicts anatomically defective structures (e.g., prolapsed mitral valves, prosthetic valves,
congenital heart disorders, valves previously damaged by
diseases including rheumatic valvular disease and previous endocarditis) and intravenous drug abusers.
Traditionally, infective endocarditis has been categoized as acute or subacute and right- or left-sided. Acute,
fulminant syndromes tend to be caused by more virulent
organisms that are prone to metastasize to other foci.
Subacute syndromes tend to be more indolent and infrequently metastasize to other locations. Right-sided endocarditis involves the tricuspid or pulmonic valves and is
Cenorovescur-{R DTsoRDERS
IV drug abusers. Left-sided

endocarditis involves the aortic and mitral valves.
more colnmonly seen with
Other ways of classiffing infective endocarditis are

native valve endocarditis, early or late prosthetic valve
endocarditis, or endocarditis ofIV drug abuse. Staphylococci and streptococci are the common infective agents in
native valve endocarditis. Fungi, staphylococci, and

gram-negative bacilli are the most common culprits seen
in early (<2 months postsurgery) prosthetic valve endocarditis. Beyond 2 months after prosthetic valve surgery,
the organisms responsible for late prosthetic valve endocarditis are staphylococci and streptococci. When infective endocarditis is seen in IV drug abusers, it is usually
due
to
staphylococci, streptococci,
or
gram-negative
bacilli.
Diagnosis is suspected based on avaiety of presenting
symptoms. Complaints can be as general as increased
fatigability, malaise, fever, night sweats, and anorexia to
severe rigors and an acute onset ofheart failure. On physical examination, the classic findings may include any or
all of the following: splenomegaly, Janeway lesions,
Osler's nodes, splinter hemorrhages, Roth's spots, new
heart murmurs, hepatosplenomegaly, pleural rub, or pericardial rub. Confirmation of the diagnosis is made by the
retrieval ofthe causative organism from blood cultures. It
is not uncommon to require that several sets of blood cultures be obtained before the culprit organism is identi-
/ I55
Cardiovascular syphilis occurs in the tertiary stage of

the disease after a 15- to 30-year latency period. Men in
their late 40s are most commonly affected, although21%o
of the cases are women. Aortic aneurysm, coronary
artery disease secondary to stenosis of the coronary
ostium, and aortic regurgitation are the most common
manifestations. Most commonly syphilitic aneurysms

manifest as chest pain, and dissection has not been
reported. Compression of neighboring structures can lead
to symptoms. The ascending aorta (60%) and transverse
arch(25%) are most commonly involved. Left main coro-
nary disease due to ostial stenosis requires
bypass
surgery.
In tuberculosis, erosion of a caseous node adjacent to

the pericardium into the pericardial sac leads to tubercu-
lous pericarditis and
is
reported
to
occur during
chemotherapy. Onset is variable and can range from effrr-
sion without manifestations
of infection to fever with
chest pain. Constrictive pericarditis can develop and is

prevented by corticosteroids. Some authorities recommend early pericardiectomy before constrictive pericarditis develops because surgical treatment of constrictive
pericarditis is technically difficult.
Lyme's disease, also known as Lyme borreliosis, is a
systemic infection with the spirochete Borrelia burgdor-
Approximately 50% of patients present with an

expanding erythematous lesion, erythema migrans.
feri.
fied. Generally, three
sets ofcultures are obtained at least

2 hours apart, while the patient is not experiencing rigors,
fever, or chills. Imaging of the heart by standard transtho-
Fever, lymphadenopathy, fatigue, and headache are common in this early phase of Lyme borreliosis. During stage
2, over weeks to months, there is neurologic, cardiac, and
racic echocardiography, MRI, ultrafast CT, or transesophageal echocardiography may allow visualization of
joint involvement. Transient myocarditis with intermittent heart block of varying degree is seen in approxi-
the vegetative process.
mately 10% of patients during stage 2. Cardiac manifestations are usually self-limite4 even without treatment,
Management of infective endocarditis is based on the

organism obtained from blood culture. If symptoms are
severe and the patient is presenting with signs of sepsis
or is demonstrating signs of seeding to metastatic foci,
therapy should be initiated and directed toward the most
likely causative organism based on the above discusslon.
Late (Rheumatic Fever Secondary to Group A

Streptococcal Infection)
(2.
0. 1.
2)
Acute rheumatic fever, which is caused by a systemic

immunologic reaction to group A Streptococcus pyogenes
pharyngitis, is a clinical syndrome of fever, arthritis, rash,
chorea, and carditis. A lymphocytic cellular infiltration of
the myocardium, endocardium, and pericardium is seen.
Frank carditis is uncommon, but when it occurs congestive heart failure and death may ensue. Heart block, pericardial effirsions, heart murmurs, and cardiomegaly may
occur during acute rheumatic fever. Acutely, there can be
aortic and mitral regurgitation, while aortic and mitral
stenosis are characteristically long-term sequelae.
although temporary pacemaker insertion may be needed

in some patients. Neurologic manifestations dominate
stage 3, which occurs months to years after the initial
infection, although chronic dilated cardiomyopathy has
been seen dunng this period.
Kawasaki disease, the mucocutaneous lymph node
syndrome, is thought to be an infectious disease. During
the first phase there is fever, conjunctival congestion, dry
and cracking lips, strawberry tongue, red swollen soles
and palms, rash, and lymphadenopathy. Stage 2 consists
of desquamation of hands and feet, arthralgias and arthritis, and carditis. The 2o/o mortality results primarily from
cardiac involvement, with aneurysms, myocarditis,
arrhythmias, mitral valve incompetence, myocardial
infarction (MI), and sudden death. During the convalescent phase cardiac manifestations may appear and even
be fatal.
Myocarditis can complicate diphtheria, with disturbances of the cardiac conduction system. Cardiac disturbances follow peripheral nerve conduction abnormalities
and are caused by the action of a toxin released by

Corynebacterium diphtheriae infecting the nasal or pha-
156 /
EMnRcsNCy MrorcrNn:
Tur Coru
CuRnrculul,r
ryngeal mucosa, or larynx. Cardiovascular collapse can

occur. The mortality of diphtheria with ST:T wave abnormalities is 28o/o versus 60/o to l0% with no ST:T wave
abnormalities.
Myocarditis can also complicate severe cases of

typhoid fever, with hypotension, muffled heart sounds,
and tachycardia. Arrhythmias, low voltage, and T:wave
inversions or flattening may be seen on the ECG. Myocardial necrosis, degeneration, and falty infiltration are
seen ln autopsy speclmens.
Parasites can invade the myocardium and cause

myocarditis. Trypanosoma cruzi, T. gambiense, and T
rhodesiense, as well as Trichonelli spiralis and Tbxoplasma
gondii are known to affect the heart. Chagas's disease,
which is caused by T. cruzi and is endemic from Mexico to
northern Argentina and Chile, can affect the heart in both

the acute and chronic phases of the disease. The most common cause of death in acute Chagas's disease is congestive
heart failure secondary to severe myocarditis. Chronic disease presents as cardiac disease years after the initial infection, with arrhythmias and congestive heart failure. The
disease has a predilection for the right ventricle, and signs
of right-sided heart failure are present commonly. Prognosis is poor after the development of heart failure.
African trypanosomiasis is caused by the parasites T.
gambiense and T. rhodesiense. These parasites are transmitted through bites of the tsetse fly, are the causative
agent of sleeping sickness, and can affect the heart. A
pancarditis characterized by mononuclear cell infiltrations frequently occurs in the acute phase of infection.
Fibrosis and destruction of myocytes develops. Both the
cardiac conducting system and autonomic innervation
can sustain damage.
The acquired immunodeficiency syndrome (AIDS),

by the human immunodeficiency virus (HIV),
results in a breakdown of cellular immunity leading to
opportunistic infections with numerous agents. In one
autopsy study 52Yo of AIDS patients had mild focal
myocarditis, which was asymptomatic. No pathogen was
identified in 82% of the cases with myocarditis.
T gondii is a ubiquitous parasite that causes disease in
both immunocompetent and immunocompromised individuals. Patients with AIDS are at high risk to develop
caused
toxoplasmosis and can have severe disseminated disease

involving the heart, lung, or brain. Immunocompetent
patients usually have a benign, self-limited infection,
though rarely disseminated disease is seen in the absence
of immune deficiency. Cardiac involvement manifests as
myocarditis. Cryptococcal myocarditis can also occur in
AIDS patients.
Endocrine and Metabolic Diseases (2.10.2) (See 13.3)

Derangements of the endocrine system can adversely
affect the cardiovascular system. Hyperthyroidism leads
to
increased inotropy and chronotropy and decreased

peripheral vascular resistance. Disturbances of rhythm
can occur, with sinus tachycardia, paroxysmal atrial
tachycardia, and atrial fibrillation. Hyperthyroidism can
present as cardiac disease, with atrial fibrillation a common presentation. Every patient with new-onset superventricular tachycardia should be assessed for hyperthyroidism. Bounding pulses are the rule, and a jugular
venous hum as well as a dynamic apical pulse can sometimes be detected. Heart failure is more common in older
patients but can be seen in younger patients. Nonspecific
ST segment and T wave changes can be seen.
Bradycardia is a hallmark of hypothyroidism. Heart

failure is rare but can occur and is associated with cardiomyopathy or pericardial effirsions. In autopsy cases
myocardial fibers can be swollen and vacuolated. Low
voltage P waves and QRS complexes, nonspecific T wave
changes, and conduction disturbances are the electrocardiographic manifestations. Thyroid replacement therapy
can exacerbate angina pectoris in patients with coronary
artery disease who develop hypothyroidism, and must be
instituted carefully with concomitant medical therapy for
in hypothyroidism have found slowed myocardial relaxation, septal

cardiomyopathy, and pericardial effirsions.
Hypertension with congestive heart failure can result
from elevated serum levels ofadrenal cortical hormones,
whether from Cushing's disease, Cushing's syndrome, or
iatrogenic administration of corticosteroids. In a study of
patients over 40 years of age with Cushing's syndrome,
95Yo had hypertension and almost 50% had congestive
heart failure. Hypertension and atherosclerosis were the
cause of death in 40% of patients dying from Cushing's
angina. Echocardiographic evaluations
syndrome. Long-term corticosteroid therapy may

increase a patient's risk ofatherosclerosis.
Hypotension is the major cardiovascular manifestation
of adrenocortical insufficiency. In patients with Addison's
disease, which is chronic pnmary adrenocortical insufficiency, over 90oh have systolic blood pressures less than
110 mm Hg. Dizziness on standing and syncope can occur.
In acute
adrenocortical insufficiency, or adrenal crisis,

shock and vascular collapse are the major manifestations.
Adrenal crisis occurs in periods of stress such as surgery
trauma, or infection, and in undiagnosed or inadequately
heated patients with adrenal insufficiency. Hyperaldosteronism presents with hypertension and hypokalemia. If
present, symptoms are those of hypokalemia and relate to
the degree ofpotassium depletion.
Cardiomegaly
and hypertension are seen
in
acromegaly, which arises from hypersecretion of growth

hormone by pituitary tumors. Cardiomegaly is seen at
autopsy but does not lead to overt cardiac disease. Hypertension responds to the usual treatment regimens. Congestive heart failure, when it occurs, may be related to
hypertension rather than a direct effect of growth hormone. Mortality can result from cardiovascular disease.
Cerolovescur/,R DTsoRDERS
Diabetes mellitus leads to early onset of coronary artery
should be monitored closely and have an electrocardiographic evaluation. Peripheral vascular disease secondary
to atherosclerosis also occurs at an earlier age and with
increased frequency in diabetes, and involvement of long
segments of smaller arteries is characteristic and difficult
to treat. Clinical complications are intermittent claudication, and ulcers and gangrene ofthe feet.
Beriberi is due to thiamine deficiency and causes highoutput cardiac failure due to reduced peripheral vascular
resistance from arteriolar vasodilation. It is thought that
effects of thiamine deficiency on sympathetic nuclei
causes the loss of sympathetic tone. Enriched bread has
all but eliminated beriberi in Western countries, though it
is seen in alcoholics, individuals who eat unusual diets,
and Orientals who eat a diet high in polished rice. Physical findings are those of biventricular failure with peripheral edema (wet beriberi). In some cases a mixed sensory
and motor peripheral neuropathy dominates the clinical
picture (dry beriberi). Cardiac enlargement, pleural effi.rsions, low voltages on the electrocardiogram, a third
heart sound, and a widened pulse pressure are character-
istic physical findings.

Scurvy, the vitamin C-deficiency disease, has been
associated with sudden death. A cardiomyopathy with
fatty degeneration of the myocardium has been described.
Emergency intravenous vitamin C has been advocated as
a treatment for the heart disease associated with scurvy,
and reversal
of
electrocardiographic abnormalities has
been reported.
The protein deficiency disease kwashiorkor is associated with bradycardia, Tlwave changes, and a prolonged
QT interval, with histologic changes of muscle fiber vacuolation and intracellular edema. Wasting of the fibers of
the cardiac conducting system has been described in children dying of kwashiorkor, and cases of sudden death
among these children may be due to abnormalities of
conduction.
Pellagra, the deficiency disease ofniacin, leads to dys-
pnea on exertion, tachycardia, palpitations, and edema.

Since this deficiency is often associated with beriberi, it
is difficult to separate the cardiac abnormalities between
the two conditions. Abnormal T waves and ST segments
resolve with niacin replacement.
The cardiac abnormalities seen in hypervitaminosis D
are those of hypercalcemia, with deposition of calcium in
necrotic myocardial cells.
A cardiomyopathy of children in a region of China
with selenium poor soil has been described. Necrosis and
fibrosis is seen on pathologic examination. Selenium
supplementation is reported to prevent this disease.
157
Rheumatologic (2.10.3)
disease and increased mortality from myocardial infarction
(MI) and sudden death. It is often said that individuals with

diabetes are prone to have silent MIs, that is, MIs without
chest pain. A patient with diabetes mellitus with acute gastrointestinal complaints or severe nonspecific symptoms
Allergic myocarditis
is an acute cardiomyopathy
caused by a hypersensitivity reaction, most commonly to
a drug. Penicillin, methyldopa, sulfonamides, and tetracycline have been causative. Exposure to allogeneic
serum can also lead to allergic myocarditis. The characteristic pathologic of allergic myocarditis is cellular infiltrates of eosinophils and large mononuclear cells. Pathologic abnormalities are limited to the myocardium,
sparing the endocardium and pericardium. Drug-induced
acute pericarditis has been associated with hydralazine
and procainamide.
The collagen vascular diseases have a number of cardiovascular manifestations. Raynaud's phenomenon is a
vascular syndrome seen in rheumatoid arthritis, systemic
lupus erythematosis (SLE), and systemic sclerosis. Raynaud's phenomenon is often a presenting manifestation of
collagen vascular diseases that heralds systemic disease
by years, and is a reversible ischemia of the hands and
sometimes feet that is triggered by cold exposure and
other factors. The hands turn white and then cyanotic. On
rewarming they become erythematous. Primary Raynaud's phenomenon can occur, and pharmacologic prophylaxis with various arterial dilators has had mixed sucCCSS.
In a recent
evaluation
of
patients
with SLE with
echocardiography, Doppler cardiography, and stress testing,75%o had cardiac disease. There were valvular heart
lesions (27%), pericardial effusions (19%), pulmonary

hypertension (16%), and myocardial infarction (7o/o,
including women less than 40 years of age). Pericardial
effirsions can be massive and require emergency pericardiocentesis. Myocardial infarction in young women is
thought to be secondary to vasculitis. Treatment with corticosteroids has been found to potentiate coronary atherosclerosis in lupus patients. Vahular heart disease with
leaflet scarring, thickening of chordae tendineae, and

chordae tendineae rupture leads to aortic and mitral
regurgitation sometimes requiring valve replacement.
Lupus patients presenting with acute chest pain or
other cardiovascular complaints need a thorough evaluation for pericardial and pleural effirsions, valvular heart
disease, vasculitis, and myocardial infarction.
Clinical cardiac disease is less common in patients
with rheumatoid arthritis, though autopsy studies show
cardiac involvement. Severe clinical pericarditis with
effusions and tamponade occurs rarely, though autopsy
studies show involvement of the pericardium in 40% of
patients. Clinical valvular heart disease and conduction
defects are uncommon but can be seen in rheumatoid
arthritis.
Systemic sclerosis (scleroderma) is associated with
cardiac fibrosis in greater than 50o/o of autopsy specimens. Pericarditis, ventricular failure, and conduction
abnormalities are most often seen, and cardiac involve-
58 /
ErrrencrNcyMnolclNt: Tnr Conr CunrucuLUM
ment is a poor prognostic sign. Clinical heart failure due

to fibrosis of the myocardium occurs in less than 10% of
patrents.
The hypereosinophilia syndrome is a condition of high

levels of circulating eosinophils with no apparent cause.
It is thought to be a regulation disorder of eosinophils
related to the chronic leukemias. Tissue damage results
from major basic protein and other eosinophil products
designed to destroy parasites. The heart and nervous system are particularly r,ulnerable to damage, and cardiac
manifestations include endomyocardial fibrosis, car-
duction abnormalities including complete heart block

(the most frequent, documented in 23%o to 30% of
patients with myocardial sarcoidosis), ventricular
arrhythmias, first-degree atrioventricular block, bundle
branch block (with right bundle branch block more common than left bundle branch block), supraventricular
tachycardia, and sudden cardiac death. The average sur-
vival after the onset of cardiac symptoms has been

reported to be I to 2 years. Treatment should be specific
for the clinical manifestation and should include steroids
unless a definite contraindication exists.
diomyopathy, and valvular heart disease.
In amyloidosis, infiltration of tissues with amyloid

protein occurs. Amyloidosis may be a primary disorder or
secondary to multiple myeloma, infections, heredity, or
chronic inflammatory diseases such as rheumatoid arthritis. There can be cardiomegaly with diffirse deposition of
amyloid in the myocardium. Congestive heart failure,
arrhythymias, val"ular disease, and conduction abnormalities including heart block are possible consequences.
Heart disease is a frequent complication of ankylosing
spondylitis. The most commonly encountered lesions are
aortic incompetence and conduction defects. Aortic valve
disease is seen inlohto 10% of patients with ankylosing
spondylitis. A wide variety of conduction disturbances
are seen including first-, second-, and third-degree atrioventricular block, bundle branch block, fascicular block,
and Wolff-Parkinson-White syndrome. Often, aortic
incompetence or conduction disturbances may be the presenting feature of ankylosing spondylitis; therefore, in
the absence of other known causes of these defects, particularly in young men, the diagnosis of ankylosing
spondylitis should be entertained. The prevalence with
which aortic incompetence and atrioventricular blocks
are seen increases with both age and duration of the disease. Aortitis in ankylosing spondylitis is characterized
by adventitial scarring and intimal proliferation causing
thickening of the proximal aortic wall behind and above
the sinuses of Valsalva. The aortitis may extend to below
the aortic root to the base of the mitral valve and into the
ventricular septum causing a subaortic fibrous ridge.
Sarcoidosis is a multisystem, granulomatous disorder.
It is characteizedby the presence ofnoncaseating granulomas found in multiple body tissues. Myocardial
involvement has been found to occur in 20Yo to 27o/o of
patients with the diagnosis of sarcoidosis. This involvement is frequently limited to a small portion of the myocardium and is often clinically silent with only 40%o to
50% of patients with cardiac sarcoidosis at autopsy having been diagnosed with sarcoidosis during their lifetime.
The different cardiac structures involved" in decreasing
order offrequency, are the left ventricular free wall, ventricular septum, right ventricle, papillary muscles, right
atrium, and left atrium. Clinical manifestations include
congestive heart failure, pericardial tamponade from
pericardial involvement, ventricular aneurysms, and con-
Renal (2.10.4)
Cardiovasular complications are the leading cause of
death in patients suffering from end-stage renal disease
(ESRD). Cardiovascular diseases account for 30% to
50% of deaths in patients with ESRD, with heart failure
causing approximately 15% of these deaths, MIs about
l0%o, and pericarditis 3%.
ESRD contributes to cardiovascular disease in a num-
ber of ways. Hypertension and lipid abnormalities are

frequently seen in these patients. The role of uremia itself
has been questioned to contribute to increased incidence
of atherosclerosis.
Hypertension in ESRD patients may be caused by the
use of erythropoietin (when used to correct the anemia
associated with ESRD, erythropoietin can lead to the
increase in peripheral vascular resistance and increase in
blood viscosity), volume overload, elevated plasma renin
activity, or sympathetically mediated vasoconstriction.
Patients with ESRD are prone to elevated very low density lipoproteins, believed secondary to deficiencies in
lipoprotein lipase and hepatic triglyceride lipase, as well
as decreased amounts of high density lipoproteins, as a
result of their decreased synthesis.
The cause of heart failure in renal failure patients is
multifactorial. These causes can be subdivided into loading conditions, systolic dysfunction, impaired diastolic
filling, and altered neural control. Factors that contribute
to an alteration in the loading conditions on the heart

include anemia and arteriovenous fistulas (used for easier access in hemodialysis patients), which cause a high
cardiac output state, hypertension, fluid retention, and
thiamine deficiency. Impairment in systolic dysfunction
is seen with MIs, hyperkalemia, hypocalcemia, metabolic acidosis, uremic toxicity, myocardial fibrosis, and
valvular disease (such as the calcium deposition seen on
valves due to secondary hyperparathyroidism, commonly seen in chronic renal failure). Diastolic filling
may be altered by pericardial disease, left ventricular
hypertrophy, and myocardial fibrosis. Patients with

ESRD also frequently suffer from autonomic neuropathy, which leads to changes in the neural control of circulation.
CenuovRscut-qR DrsoRDERs
Uremic pericarditis is frequently encountered in both
acute and chronic renal failure patients. Echocardiography has demonstrated pericardial effrrsions in 32o/o to
560/o of patients starting dialysis who are asymptomatic.
Pericarditis develops in approximately l5o/o of ESRD
patients and has an average mortality of l3%o when it
occurs. Uremic pericarditis has been classified by some
authorities as early pericarditis as it tends to occur in
patients before they begin chronic dialysis treatment.
Uremic pericarditis is believed to be caused by the accumulation of uremic toxins, which leads to an inflammatory serositis involving the pericardium, but it may also
involve the pleura (causing fibrinous pleuritis, pleural
friction rubs, hemorrhagic pleural effusions, and pneumonitis). This form of pericarditis usually responds positively to dialysis in the vast majority of patients. A late or
dialysis-associated pericarditis is also seen. The mechanism for this form of pericarditis is much less clear. Late
pericarditis tends not to respond to dialysis and is more
severe with an increased likelihood of complications,
including pericardial tamponade. Most
commonly,
patients with a pericarditis will complain of chest pain,

which may be located anywhere on the precordium, have
a pleuritic component to it, and be aggravated by lying
supine and relieved by sitting forward. A friction rub may
be observed in up to 95% of these patients with it having
an evanescent quality that changes moment to moment;
therefore, the absence of a friction rub does not rule out
pericarditis. Hypotension during dialysis not attributable
to changes in intravascular volume, or in ESRD patients
presenting with hypotension despite signs of fluid overload may be due to a significant pericardial effusion.
Cardiac arrhythmias are often seen
in the ESRD
patient. Some studies have indicated cardiac arrest to be

the cause of death in approximately 9%o of ESRD
patients. Ventricular fibrillation has been seen to be the
terminal rhythm in 25% of deaths that occur at dialysis
centers. The cause of these arrhythmias is associated with
the constant disequalibrium of fluid, electolytes, and

acid-base status, MIs, coronary aftety disease, left ventricular hypertrophy, CHF, pericardial disease, and alteration in drug metabolism (e.g., digitalis).
Toxic Exposures (2.10.5)
A number of systemic poisonings adversely affect the

cardiovascular system.
Cocaine causes tachycardia and hypertension and is
associated with cardiac arrhythmias, angina pectoris, and
acute MI. Sudden death occurs with cocaine use. Cocaine
is a potent inducer of vasospasm, and infarctions have
/ I59
Tricyclic antidepressant overdoses generally present

with a sinus tachycardia, but in severe overdoses bradycardia and hypotension can develop. Widening of the
QRS complex is associated with arrhythmias and
seizures. Sodium bicarbonate infusion can reverse both
QRS widening and hypotension and is felt to be protective against lethal arrhythmias. Procainamide, quinidine,
and disopyramide are not recommended in treating the
arrhythmias associated with tricyclic overdose.
Monoamine oxidase inhibitors (MAOI) are used as
antidepressants. These substances inhibit the enzyme
monoamine oxidase and lead to elevated brain levels of
norepinephrine, serotonin, and dopamine. Drug interactions with a number of substances, including tyramines
found in foods, sympathetic amines, antihypertensives,
and beta-blockers can cause severe hypertension and
tachycardia. Other symptoms of these interactions
include fever, diaphoresis, and subarachnoid hemorrhage; death can result. In overdose, there is a progression
from an asymptomatic phase (0 to 12 hours) to a phase of
central catecholamine excess, with neuromuscular excitation and sympathetic hyperactivity. Tachycardia and
hypertension are the most common cardiovascular manifestations during this period. A phase of catecholamine
depletion then occurs, with cardiovascular collapse that is
poorly responsive to therapy.
Digoxin increases automaticity of myocardial cells
while decreasing conduction, so digoxin toxicity leads to
a combination of tachydysrhythmias and heart block.

Paroxysmal tachycardia with block and bidirectional ventricular tachycardia are uncommon except in the setting
of digoxin toxicity. Bradycardia,
premature ventricular
contractions, and all degrees ofheart block are seen with
digoxin toxicity. Serum digoxin levels correlate with toxicity. In an acute overdose, serum potassium is of great
prognostic significance, with mortality correlating with
potassium levels greater than 5.0 nEQL. The introduction of Digibind the Fab fragment of a digoxin-specific
sheep monoclonal antibody, has revolutionized the treatment of digoxin poisoning.
Colchicine is a plant alkaloid used in the treatment of
gout, which in overdose produces cardiogenic shock. Poisonings can occur from intentional overdose ofthe purified
medication or accidental ingestion of the plants from
which it is obtained, autumn crocus (Colchicum autumnale) and glory lily (Gloriosa superba). After ingestion, a
1- to 6-hour gastrointestinal phase occurs, followed by a
phase ofmultisystem failure. A dose of 0.8 mglkg is invariably fatal. Intractable cardiogenic shock occurs within the
firstT2 hours after ingestion and carries
a poor prognosis.
The antimalarial chloroquine in overdose has a direct

toxic effect on the myocardium. Death is rapid, with cardiac arrest occurring within I to 3 hours after ingestion.
Prognosis has traditionally been poor, but a prehospital
been reported in many organs. Myocardial infarctions

and strokes occur in association with cocaine use in
young healthy people without atherosclerosis. Rupture of
protocol with intubation, high-dose epinephrine, and
the ascending aorta has been reported.
diazepam has improved resuscitation outcome.
160 /
EnmncrNcv Menrcmr: Tun Conn Cunl:culutvt
In acute overdoses the toxicity of calcium channel

blockers and beta-blockers is an exaggeration of their
therapeutic actions oflowering blood pressure and reduc-
catalyst in the conversion of fibrinogen to fibrin, in turn

forming a stable fibrin matrix. Also, platelet receptor gly-
glucagon, pressors, and cardiac pacing are the mainstays

oftherapy, and calcium can be given for calcium channel
attaining coronary artery patency by activating the

enzyme plasminogen, converting it to plasmin, which is
capable of digesting fibrinogen, and lysing the clot. The
longer that a coronary artery is occluded by thrombus,
the more damage that is done to the myocardium. Obviously, therefore, it is best to remember that "time is mus-
ing heart rate, with the complication of negative

ionotropy. If the circulation can be supported until the
drug is metabolized" the patient will survive. Fluids,
overdoses In severe poisonings, intraaortic balloon

pump and cardiopulmonary bypass may be lifesaving.
Tachycardia, other cardiac arrhythmias, and hlpotension are the cardiac manifestations of theophylline overdose. Hypotension results from vasodilatation, and vigorous fluid resuscitation is necessary. If pressors are
needed, p-agonists should be avoided as worsening of
vasodilatation may result. Charcoal hemoperfusion and
multiples of activated charcoal greatly accelerate elimination of theophylline.
SELECTED READING
Diggs P, Vlay SC. The spectrum of infective endocarditis. Hosp Med
1994;30(1):22-27
of systemic diseases. In:

Aghababian P.Y, ed. Energency managemenl of cardiovascular disease.
Boston: Butterworth-Heinemann, 1994;293-305 O'Neill TIW, Bresnihan B. The heart in ankylosing spondylitis Ann Rhetrm
Meggs WJ. Cardiovascular manifestations
D i s 1992;5 I (6) :7 05-706.

Paston SO, Braunwald E. Renal disorders and heart disease. In: Braunwald
E, ed. Heart disease: a textbook of cardiovascular medicine Philadelphia: Saunders, 1992;1 856-1871.
Preston RA, Chakko S, Materson BJ. End-stage renal disease. In: Rapaport
E, ed. Cardiology and co-existing drsease New York: Churchill-Living-
stone, 1994;75-196.
Shammas RL, Movahed
A.
Sarcoidosis of the heart. Clin Cardiol 1993;
16(6):462472.
TREATMENT MODALTTTES
(2.1 1)
Thrombolytic Therapy (2.11.1)

Acute myocardial infarction (AMI) continues to be the
leading cause of death in the United States with an estimated 500,000 to 700,000 deaths annually. The past
decade has shown arapid evolution in the approach and
treatment of the patient presenting with AMI. The emergency physician is on the front-line of this treatment.
Thrombolytic therapy and its accompanying adjunctive
therapies as well as advances in the ability to pace the
heart are all within the armamentarium of treatment.
AMI occurs when an atherosclerotic plaque ruptures
and exposes collagen fibers, which in turn binds platelets
and causes initiation ofthe coagulation cascade. Platelets
further potentiate clot formation through several mechanisms. Platelet receptors for various proteins lead to further aggregation and adhesion. Their membranes also
bind coagulation factors V and X, potentiating the conversion of prothrombin to thrombin. Thrombin acts as a
coprotein binds with fibrinogen causing further platelet

aggregation. All of this ultimately leads to an occlusive
coronary thrombus and AMI.
Reperfusion using thrombolytic therapy is aimed at
cle." The decision to use thrombolytic therapy and which

thrombolytic to use must be made in the shortest amount
of time possible.
The National Heart, Lung, and Blood Institute's
National Heart Attack Alert Program, in conjunction with
the American College of Cardiology, the American Heart
Association, the American College of Emergency Physicians, and other organizations, has set the eligibility criteria for thrombolyic therapy in acute myocardial infarction. To be clinically eligible, the patient should have had
chest pain or chest-pain-equivalent syndrome consistent
with AMI less than 12 hours from symptom onset. Electrocardiographic evidence includes a l-mm or greater ST
elevation in two or more contiguous limb leads, or a 2mm or greater ST elevation in two or more contiguous
precordial leads, or a new left bundle-branch block or
evidence of acute true posterior myocardial infarction.
Initial stricter criteria of chest pain less than 6 hours and
a patient age younger than75 made approximately 40Yo
of patients presenting with an acute myocardial infarction
ineligible to receive thrombolytic therapy. Meta-analysis

and various trials of thrombolytic therapy have confirmed
that elderly patients are at an increased risk of stroke;
however, this risk is countered by the net reduction in
mortality when thrombolytics are used. Also, numerous
studies have indicated that the least amount of time
between the diagnosis of AMI (preferably, within 4
hours) and the delivery of the thrombolytic agent resulted
in the greatest benefit; however, there does remain a

reduction in mortality even if thrombolytics are not used
until 12 hours (and in some studies more than 12 hours)
after onset of symptoms. The ultimate goal remains to
give thrombolytic therapy as soon as possible, making the
diagnosis to drug time 60 minutes or less.
In addition to the eligibility criteria, the exclusion criteria for thrombolytic therapy are of equal importance.
The greatest risk of using thrombolytics is hemorrhage.
To reduce this risk, certain contraindications to using
thrombolytics must be considered. The absolute contraindications include active internal bleeding, altered
level of consciousness, known spinal cord or cerebral
arteriovenous malformation or tumor, recent head
trauma, intracranial or intraspinal surgery within the
previous 2 months, trauma or surgery within previous 2
Cerutovescut-AR DISoRDERS
weeks that could result in bleeding into a closed space,
persistent blood pressure of greater than 2001120 mm
Hg, known bleeding disorder, pregnancy, suspected aor-
tic dissection, or previous allergy to streptokinase. Relative contraindications to the use of thrombolytic therapy in which the risk of hemorrhage may outweigh the
benefits of treatment include active peptic ulcer disease,
history of ischemic or embolic cerebrovascular accident; current use of anticoagulants; major trauma or
surgery from 2 weeks to 2 months previously; history
of chronic, uncontrolled hypertension (treated or
untreated); or prolonged CPR. In the event that exces-
sive bleeding occurs with thrombolytic therapy, the

thrombolytic infusion should first be stopped. If needed'
to reverse the effects of the lytic therapy, cryoprecipitate, platelets, and aminocaproic acid (Amicar) should
be considered.
Current thrombolytic agents available for use include

streptokinase (SK), anistreplase (anisoylated plasminogen-streptokinase activator complex [APSAC])' urokinase, and tissue plasminogen activator (t-PA) Of these,
streptokinase and t-PA have been researched most. Streptokinase is a single-chain polypeptide with a molecular
weight of approximately 50,000 produced by B-hemolytic
streptococcal cultures. By itself, it has no enzymatic
properties. It binds to plasminogen in a 1:1 ratio causing
a change in its molecular configuration such that this
complex becomes an active enzyme, with a half-life of
ab oltt 25 minutes, which activate s plasminogen, changing
it to plasmin that acts as a proteolytic enzyme able to lyse
fibrin clots. Because it is produced by p-hemolytic streptococci, it is antigenic and has demonstrated allergic
reactions in 5oh of patients receiving it, consisting of pruritus, rash, and fever, which is easily treated with antihistamines or antipyretic agents. Significant hypotension
may occur. Also, a significant number of patients develop
antibodies to the streptokinase itself, making it inadvisable to readminister it at a later date.
Tissue plasminogen activator is a single-chain glycoprotein with a molecular weight of 70,000 and is naturally occurring. Produced in large quantities by recombinant DNA technology, t-PA selectively binds to fibrin in
the clot matrix, resulting in a complex that converts plasminogen to plasmin and thus produces a more localized
thrombolysis. It is nonantigenic and is fibrin specific,
compared to streptokinase, which lacks fibrin specificity-activating fibrin-bound and circulating plasminogen equally well. Because of this, t-PA produces higher,
early coronary patency than does streptokinase'
Streptokinase is given as a dose of 1.5 million units
infused over 60 minutes. The most common dosing regiment for t-PA currently is 100 mg (or 1.5 mg/kg), infused
over 2 hours with 15 mg given as an IV bolus over 1 to 2
minutes followed by an infusion of 50 mg over the next
60 minutes. The last 35 mg of the t-PA is given over the
next hour.
167
The findings of a large-scale trial (GUSTO) showed

that accelerated t-PA provides a survival benefit over
streptokinase. With the cost of medicine an ever-growing
concern, there is, however, a substantial difference
between the agents. The average cost for streptokinase is
one-tenth that of t-PA ($200 vs. greater than $2,000).
Ultimately, the decision as to which thrombolytic to use
should be based on rapid availability in the ED and familiarity of the physician with the agent.
Pharmacologic Agents (2.11.2)

The lysis of clot and early reperfusion through the use
of thrombolytic therapy is the cornerstone of treatment of
patients suffering from
AMI. Of those treated with
thrombolytics, however, 20oh to 30%" fall to reperfuse and

l0%o to 25o/o of patients experience reocclusion. Adjunctive pharmacologic therapy has evolved to address these
intrinsic limitations to thrombolytic therapy, the goals of
which are to (l) help maintain coronary artery patency by
preventing reocclusion and thus reinfarction, (2) prevent
ventricular remodeling, (3) improve coronary blood flow
and/or decrease myocardial oxygen demand to reduce
myocardial ischemia, (4) decrease the size of myocardial
infarction, (5) reduce days of hospitalization, and (6)
improve the quality of life in a cost-effective manner.
Such adjunctive therapy includes any one or all ofthe following: oxygen, morphine, aspirin, nitroglycerin,
heparin, p-adrenergic blockers, angiotensin-converting
enzyme (ACE) inhibitors, calcium antagonists, magnesium, and prophylactic antiarrhythmics.
Supplemental oxygen should be given to the patient
with an uncomplicated myocardial infarction at a rate of
2 to 4 L per minute via nasal cannulae. Patients suffering
from AMI frequently demonstrate a mild to moderate
hypoxemia possibly due to a ventilatiorVperfusion mismatch that is easily corrected with the addition of supplemental oxygen.
Patients suffering from AMI may also be suffering
from pain and anxiety. Morphine sulfate can be used both
as an analgesic and an anxiolytic. It can be administered
in2- to 4-mg boluses every 5 to 10 minutes as required to
a maximum of 15 mg during the first 30 to 60 minutes.
Morphine has several advantages in that in addition to

acting as an analgesic and decreasing anxiety, it also
causes arteriolar dilatation, which decreases afterload; it
causes venous dilatation, which decreases pulmonary
capillary pressure and preload; and it causes suppression
of the respiratory center, thereby decreasing the respiratory rate, reducing the sensation ofdyspnea, and reducing
the work of breathing, all of which decrease myocardial
oxygen demand.
The Second International Study of Infarct Survival
(ISIS-2) demonstrated the efficacy of aspirin when used
alone and in conjunction with thrombolytic therapy in the
162 /
EtrtncnNcv MBlrcrNn: Tnr Conn CunnrcuI-uM
treatment of AML In the study, 17 ,187 patients presenting within 24 hours of symptom onset were randomized
to receive 162.5 mg aspirin daily by mouth, 1.5 million
units of streptokinase over 60 minutes, both, or neither.
Five weeks after randomization, aspirin was demonstrated to be associated with a 49% reduction in nonfatal
reinfarction, a 46%o reduction in nonfatal stroke, and a
23%o rcduction in total vascular mortality. This was compared to a reduction in total vascular mortality of 25%oby
streptokinase alone and a 42Yo reduction when the treatments were combined. Aspirin decreases platelet aggre-
gations
by irreversibly acetylating the active site of
cyclooxygenase, which is required for the production of

thromboxane A2, aplatelet aggregation promoter. Aspirin
should be given orally at a dose of 2 mglkg (162.5 mg in
a typical male) immediately in any patient suspected of
having an AMI. It is suggested that this first dose given
in the ED should be chewed to promote faster absorption.
Following thrombolytic therapy, there is an increase in
thrombogenicity, which possibly accounts for reocclusion
after thrombolysis. Rethrombosis after thrombolysis is
mediated not only by platelet aggregation, which as previously described can be inhibited by the use of aspirin,
but also by activation of the coagulation cascade and an
increase in thrombin production. To assist in prohibiting
the later, the role of intravenous heparin in maintaining
coronary artery patency has been evaluated in four clinical trials consisting ofa total of 1,073 patients. In all four
studies, intravenous heparin consistently demonstrated an
increase in coronary artery patency. In addition, because
of the lack of systemic fibrinogenolysis with the use of tPA, which is more fibrin specific and has a shorter halflife than streptokinase, rethrombosis and reocclusion are
more common after t-PA, requiring immediate concomitant intravenous heparini zation. The prompt use of intravenous heparin is a reasonable adjunctive therapy. The
standard intravenous heparin dose regimen is 5,000 IU
bolus followed by a continuous intravenous drip starting
at 1,000 IU/hour and titrated to maintain an activated prothrombin time (aPTT) of 1.5 to 2.0 times control. Heparin
has also been demonstrated to reduce the risk of mural
thrombus in patients having suffered AMI.
Intravenous heparin is frequently used as an adjunct
with intravenous nitroglycerin in patients with acute myocardial infarction. Studies have shown that in patients
receiving the two drugs together, there may be a nitroglycerin-induced heparin resistance that may compromise adequate heparinization, necessitating increased
amounts of heparin to achieve and maintain a therapeutic
state of systemic anticoagulation.
Intravenous nitroglycerin used during the course of an

acute myocardial infarction has been shown to be beneficial in limiting infarct size, preventing infarct expansion,
and decreasing morbidity and mortality. These benefits

are attributable to several actions of nitrates. First,
nitrates are known to decrease both the preload and after-
load on the heart, which results in a decrease in the left

ventricular filling pressure and ultimately decreases myocardial oxygen demand. Second, during acute coronary
artery occlusion, nitrates have been demonstrated to
increase collateral blood flow to the area of ischemia,
which decreases infarct size. Also, the use of intravenous
nitroglycerin has been found to have platelefinhibiting
properties. These properties are not well understood, but
may be attributable to nitroglycerin's decreasing adenosine diphosphate and thrombin-mediated platelet aggregatlon.
Intravenous nitroglycerin can be used relatively safely
if patients prone to hypotension are screened out. These
patients would include those who are hypovolemic or

those who require a high filling pressure to maintain an
adequate cardiac output, such as those suffering a right
ventricular infarct. Aside from these exclusions, all other
patients suffering AMI should receive nitrates as soon as
possible. Sublingual nitroglycerin is the first line of treatment and can be given every 5 minutes, up to a total of
three doses, if systolic blood pressure remains greater
than 90 mm Hg. If the patient is experiencing continued
discomfort, significant systemic hypertension, or pulmonary congestion, then intravenous nitroglycerin may
be instituted. The initial dose is 5 to l0 pcg/min, titrating
to the desired end point (e.g., relief of discomfort) by
increasing by 5 to 10 pcg/min every 5 to 10 minutes or
until a dose of 200 pcg/min is reached. The nitroglycerin
should also be titrated against mean blood pressure with
an end point ofa reduction ofmean blood pressure ofnot
more than l0% in normotensive patients and not more
than 30o/o in hypertensive patients, but not less than 80
mm Hg. The patient should be reassessed if the systolic
blood pressure drops to less than 90 mm Hg, the mean
blood pressure drops to less than 80 mm Hg, the diastolic
blood pressure increases more than 15 mm Hg, or the
heart rate increases more than 20oh or decreases more
than 50 beats per minute. If hypotension is encountered,
discontinue the nitroglycerin and restart at 5 pcglmin
when safe.
The second best documented cardioprotective therapy
in addition to thrombolytic therapy in patients with AMI
is the use of B-adrenergic blockers. Beta-blockers assist
by reducing chest pain, limiting the extension of the
infarct, reducing the incidence of supraventricular and
ventricular arrhythmias (including ventricular fibrillation
and possibly including reperfusion dysrrhythmias frequently encountered with the use of thrombolytics), and a
reduction in the overall mortality. Beta-blockers have
been demonstrated to shift the flow of blood from the epicardium to the more ischemic endocardium. They are
capable of lowering systolic blood pressure, reducing
heart rate (which combined, contribute to decrease the
oxygen demand on the heart), increasing diastolic time
and thus lengthening perfusion time, and preventing
myocardial wall stress, thus preventing myocardial wall
Cenorovescut-{R DTsoRDERS
rupture. It is recommended that persons receiving thrombolytic therapy be given metoprolol (15 mg in three 5-mg
doses over 15 minutes) or atenolol (5 mg over 5 minutes,
wait
10 minutes, then give a second 5 mg over 5 minutes),
or propranolol
(l
mg slowly every 5 minutes to a total
of
5 mg) intravenously as a routine
adjunctive therapy
unless contraindications to its use exist. Definite contraindications to the use ofbeta-blockers exist in l5Yo of
patients suspected of suffering AMI. These absolute contraindications include (1) hypotension (blood pressure
<100 mm Hg), (2) bradycardia (heart rate <45 beats per
minute), (3) moderate to severe left ventricular heart failure (lung rales, peripheral signs of shock), (4) higher
degree ofatrioventricular block (PR interval >0.24 secs,
complete heart block, Mobitz type II AV block), (5)
severe COPD (requiring maintenance treatment with
steroids or pu-stimulants), and (6) previous adverse reaction to beta-blockers. The relative contraindications to the
use of beta-blockers include (1) history of asthma, (2)
currently taking beta-blockers, (3) currently taking calcium channel blockers, (4) severe peripheral vascular disease, and (5) brittle insulin-dependent diabetes mellitus.
Theoretically, calcium channel blockers should benefit
patients suffering from an acute myocardial infarction by
relieving coronary artery spasm; improving collateral
flow to areas of ischemia; reducing myocardial oxygen
demand by decreasing afterload, heart rate, and myocardial contractility; and preventing excessive influx of calcium ions into ischemic cells. However, of the 22trials on
the use ofcalcium channel blockers in patients suspected
of suffering an acute myocardial infarction, there has
been no improvement in mortality rate, infarct size, or
reinfarction rate. Only in the treatment of non-Q-wave
myocardial infarction has diltiazem offered any reduction
to reinfarction.
Injury to the myocardium activates the reninangiotensin and sympathetic systems. Neurohormonal
activation increases infarct expansion by increasing myocardial oxygen demand and increasing coronary arterial
vasoconstriction. They also worsen ischemia, reinfarction, congestive heart failure, and malignant arrhythmias.
Angiotensin II may also be directly toxic and have similar toxic manifestations to norepinephrine on the myocar-
dium. ACE inhibitors increase coronary blood flow

decrease cellular proliferation (hence, limit remodeling),
decrease inflammatory response to local injury, and
decrease edema formation. Starting treatment too early
with an ACE inhibitor increases the risk of hypotension,
in particular if used concurrently with streptokinase,
which acts as a vasodilator by activating the reninangiotensin system. Therefore, for safety reasons, ACE
inhibitor treatment is generally delayed until 24 hours
after AML Studies have indicated improved long-term
survivability with the use of ACE inhibitors.
Magnesium is the second most abundant intracellular
cation in the body. The average 70 kg male has a total of
163
-24 g of Mg2* in the body. Of this, only lo/o of the total

body Mg2* is found in the extracellular flui{ 60% is
found in bone, and 39%o is intracellular. It had been noted
in early clinical trial that patients suffering from AMI
were found to have a drop in their serum Mg2* levels dur-
ing the acute myocardial infarction, which returned to

baseline in 48 to 72 hours. Magnesium takes part in the
utilization of glucose, metabolism of adenosine triphosphate, muscle contraction, and the function of the cells
Na/K pump. Theoretical effects of magnesium therapy
include a reduction in the incidence of arrhythmias, systemic vasodilatation, coronary dilatation, a decrease in
platelet aggregation, improved myocardial metabolism,
protection against catecholamine-induced myocardial
necrosis, and a reduction in myocardial infarct size. However, the results of the Fourth International Study of
Infarct Survival (ISIS-4) demonstrated no clinical evidence to support the use of Mg2* in the setting of an acute
myocardial infarction, although magnesium was given
late in the sequence of events following AMI. Currently
the only use of Mg2* in advanced cardiac life support is
to control the arrhythmia of torsades de pointes.
The use of lidocaine hydrochloride in patients having
of ventricular ectopy is indisputable.

However, the role of lidocaine prophylactically during an
repetitive forms
acute myocardial infarction without the presence of

symptomatic ventricular ectopy has been more controversial. Meta-analysis of nine randomized trials demonstrated an increase in mortality in patients who had
received intravenous lidocaine prophylactically. Therefore, the use of intravenous lidocaine should be used
selectively and not as a routine prophylactic agent.
Cardiac Pacemakers
(2.1 1.3)
Temporary Q.11,3.1)
Temporary pacemakers are indicated for emergent pac-
ing. Emergent pacing is required in patients with hemodynamically unstable bradycardia, bradycardia with
malignant ventricular escape rhythms unresponsive to
drug therapy, and for termination of malignant atrial and
ventricular tachycardias. Temporary pacing is most helpful in patients whose primary problem is impulse formation or conduction disorders in the presence of normal
myocardial function.
In the setting of cardiac arrest, if emergency pacing is
to be used, it should be started early. When asystole or
pulseless electrical activity is present, pacing is usually
ineffective because considerable time has elapsed since
onset of hypoperfusion; therefore, myocardial response to
pacing will be limited.
Standby pacing is indicated when it is anticipated that
a stable patient may decompensate in the near future.
Examples of indications for standby pacing include stable
164 /
EuencnNcy
MltrcrNr: Tun Conr Cunnrculuv
bradycardias and prophylactic pacing in acute myocardial
infarction.
Transvenous pacing is the endocardial stimulation of
the right atrium andlor ventricle via using an electrode
that is introduced through a central vein. Venous access
routes include the subclavian, internal jugular, and

femoral and brachial veins. Transvenous patient electrodes may be rigid (5 to 7 French), which is placed with
the assistance of fluoroscopy; semirigid (a French); or
balloon tipped (6 French), which requires adequate
blood flow for placement. Lead configurations can be
unipolar or bipolar with distal pacing and proximal sensing leads.
A variety of venous introducers can be used to insert
transvenous pacing catheters. A soft, semifloating, flexible bipolar catheter is preferred because it takes advantage of any forward blood flow that is present. Transve-
nous pacemaking complications include
lator/pacing devices automatically blank the pacing complex, avoiding this problem.
The two pacing electrodes are placed on the anterior
and posterior thorax. The anterior electrode is placed to
the left ofthe sternum and centered as close as possible
to the point of maximum cardiac impulse. The posterior
thoracic chest electrode is placed directly behind the
anterior electrode.
To initiate pacing, begin at 80 beats per minute. In
asystolic arrest turn the current to maximum output and
then decrease it if capture is achieved. In patients not in
cardiac arrest, slowly increase the output from the minimal setting until capture is achieved. Capture is characteized. by a widening of the QRS complex and a broad T
wave. The only sure sign of electrical capture is presence
of a consistent ST segment and T wave after each pacing
spike.
vessel injury, pneumothorax, and laceration of the liver.
Complications of transcutaneous pacing include failure to capture, which may be related to electrode placement or patient size and body habitus. Two major pitfalls
are failure to recognize the presence ofunderlying treatable ventricular fibrillation, and failure to recognize that
the pacemaker is not capturing. Pain from the pacemaker
may be relieved using analgesia and muscle relaxants;
however, transcutaneous pacemaking is usually well tolerated because of improvements in electrodes. Tissue
damage also has been minimized by recent changes in
pacemaker and electrode designs. There can be capture
failure because of changes in pacing thresholds with pro-
Transthoracic pacing should never be used except as
longed pacing.
lead
dysfunction, arrhythmias, deep venous thrombosis, and

problems with the generator.
Transthoracic pacing is percutaneous placement of a
bipolar pacing wire directly into the right ventricle cavity
using a trocar needle. The transthoracic technique was
developed initially because it was faster than the transvenous method. This technique has been replaced by transcutaneous pacing
the emergency care setting.
Transthoracic pacing has a high incidence of serious
in
complications including pericardial tamponade, major

a
last alternative.
Transcutaneous pacing is stimulation of the heart using
externally applied skin electrodes that deliver an electrical impulse. Of the cardiac pacemaking methods available the transcutaneous technique is the initial technique
of choice. It is ideal for emergency care because of the
speed with which it can be initiated and is the least invasive pacing technique available. It should be considered
as a bridge until transvenous pacing can be initiated or
the underlying problem is corrected.
Most transcutaneous pacemakers are currently built
into defibrillators. Multifunctional electrodes allow for

pacing, hands-off defibrillation, and ECG monitoring
using a single pair of anterior-posterior or sternal-apex
chest wall electrodes. Transcutaneous pacemakers allow
operation in a fixed rate (nondemand or asynchronous) or
demand mode. Rate selection ranges from 30 to 180 beats
per minute; current output is adjustable usually from 0 to
200 mA.
If an ECG monitor is not integral to the pacemaker, an
output adapter to a separate monitor is required to blank
out the large spike from the pacemaker to allow interpre-
tation of the much smaller ECG complex. Without this

blanking protection the standard ECG and monitors are
Permanent (2.11.3.2)
Permanent pacemaking is indicated when symptoms
continue during temporary pacing. The power source for
modern permanent pacemakers is lithium batteries. Electrodes are run through the veins to the heart or to the epicardium through subcutaneous tissue.
In emergencies the type of pacemaker should be determined to allow for appropriate intervention or reprogramming. The pacemaker patient should carry the pacemaker identification card with that information. The
classification system for permanent pacemakers designates position I as the chamber paced; position II, the
chamber sensed; position III, the mode of response; posiprogrammable functions; and position
tion
antiarrhythmic functions. The most widely used pacemaker is
the VVI type, which is the ventricular demand inhibited
I!
response pacemaker.
Reported permanent pacemaker l-year failure rates

range from 7.4oh to l5%. Most failures occur within I
overwhelmed by the pacemaker spike, making the rhythm
month of implantation. Difficulties include inadequate

sensing, most commonly from electrode dislocation, battery failure, muscle stimulation, and inadequate capture.
difficult to interpret. Most combination monitor/defibril-
Malfunctions are discovered by patients reporting recur-
Cennrovescur-AR DISoRDERS
rent symptoms and physical examination findings of

infection or disconnection. Dysfunction may occur
because of loss of capture demonstrated by absence of a
pacing spike, sensing with rate competition, and absence
of a pacing artifact, suggesting mechanical difficulties.
Information of electrode position is determined using
chest radiography. By placing a magnet perpendicular to
the pacemaker generator the pacemaker is converted to a
reliable, fixed ventricular pacing mode.
Surgical Interventions (2.11.4)
In
1902 the first successful cardiac surgery was performed in the United States for a stab wound to the heart.
Since that time cardiovascular surgery has been performed for cardiac trauma, valvular and congenital heart
disease, aortic dissection, and coronary artery disease.
This section focuses on the indications for surgery for

coronary artery and valvular heart disease.
165
ered for revascularization. The decision as to which

revascularization method is most appropriate depends on
a number of factors, including the anatomy and severity
of coronary artery disease, left ventricular function, and
the patient's age, concomitant diseases, and personal
preference.
Recent studies have demonstrated the majority of

patients with CAD can be treated effectively with either
CABG or PTCA with little difference in death or myocardial infarction rate 3 years postprocedure. Patients
treated with PTCA, however, have a significant increase
in need for subsequent intervention and revascularization, and in the use of antianginal medications, compared
to patients treated with CABG. In addition, patients with
three-vessel coronary artery disease and left main disease
appear to benefit more from sugery than from PTCA.
From a financial standpoint the initial cost of PTCA is
60Vo to 75% less than that of CABG. However, at 3 years
the cost of the two strategies is nearly equal since PTCAtreated patients require repeated interventions secondary
to resteno sis and in c omp lete revascula ization.
Coronary Artery Disease

Coronary artery disease (CAD) is the leading cause
of
death in the United States, affecting 1.5 million people

and killing near one million individuals each year. The
death rate from myocardial infarction is decreasing, however, due to earlier recognition and improved manage-
ment, including revascularization procedures. The two

most commonly employed myocardial revascularization
procedures are coronary artery bypass grafting (CABG)
and percutaneous transluminal coronary angioplasty

(PTCA).
Annually CABG is performed in one of every 1000

in the United States. The theory behind it is
simple: limit the extent of myocardial infarction by
restoring blood flow to ischemic myocardial cells. This
persons
is accomplished by physically bypassing the obstructive

coronary lesions using saphenous veins or internal
mammary arteries as conduits. As the safety of the procedure has improve4 the indications for CABG have
broadened.
UnstableAngina
Recognition and management of unstable angina are
it is considered a warning sign of
impending myocardial infarction or sudden cardiac death.
Unstable angina is defined as (l) angina ofnew or recent
imperative, since
onset;
or (2)
angina that has changed
in
character,
become more frequent, more severe, or precipitated by

less exertion or less responsive to nitroglycerin; and (3)
angina occurring at rest.
Patients
with unstable angina who have
persistent
symptoms despite maximal medical therapy are consid-
The primary interventional therapies for acute myocar-
dial infarction (AMI) include the administration of

thrombolytic agents and PTCA. Percutaneous transluminal coronary angioplasty may serve as either the initial
management strategy (primary angioplasty), or as secondary therapy for patients who fail to reperfuse with
thrombolytic therapy (rescue angioplasty). In both situations coronary angiography is first performed to define
the anatomy and severity of CAD. Patients with threevessel disease, left main disease, lesions not accessible to
angioplasty, left ventricular dysfunction, or cardiogenic
shock are best managed with CABG. Surgery should be
performed within 8 hours of the onset of AMI; after this
period evidence suggests the risk of surgery is increased
and the benefit in terms of myocardial salvage limited. If
the postinfarction period is longer than 8 hours, surgery
is usually delayed as least 24 hours.
Another indication for emergent CABG in the setting
of AMI is the management of complications of primary
or rescue angioplasty, or rarely for complications of coro-
nary angiography. Emergency CABG is required in

approximately 3.7% of patients undergoing PTCA, primarily for extensive coronary arterial dissection and
coronary artery perforation or rupture.

Some patients with previous angiographically diagnosed three-vessel or left main disease may present to the
ED with AMI; in this situation, the surgeons may elect to
take the patient directly to the operating room for CABG.
For these patients the value of old records and coronary
catheteization reports are invaluable and may save precious time and resources.
166 /
EurRcrNcy MrorcrNn: Tun Conn CuRRrcur-uv
Valvulsr Heart Disease
Aggressive evaluation and stabilization must occur
Emergency valvular surgery is defined as surgery per-
formed for the correction of valvular disease believed

likely to result in death within 24 hours. This decision is
based on the patient's clinical presentation, circulatory
status, and response to therapy. Patients requiring emergency val'r.ular surgery are characterized by increasing
hemodynamic instability despite maximal therapy. These
patients may or may not have a history of valvular disease. Emergency surgery is most commonly performed in
patients with acute mitral or aortic regurgitation, or fracture/failure of a prosthetic valve. Acute aortic regurgitation is primarily caused by infective endocarditis and aor-
simultaneously. Blood should be sent for CBC, type and

crossmatch, and coagulation studies. The vascular
surgery service should be consulted immediately. Two
large bore IVs should be established, the patient placed
on a monitor, and oxygen administered. For hemodynamically stable patients, immediate esophagogastroduodenoscopy (EGD) should be performed. This can identify
the presence and location of a fistula in the majority of
cases. A negative EGD, however, does not exclude the
diagnosis. Ultrasound and computed tomography (CT)

may be useful by identifying a false aneurysm (sugges-
tive of a fistula), a perigraft collection of fluid, or the

leakage of oral contrast outside the bowel. Aortography is
if positive, but a normal study is not reassuring.
Hemodynamically unstable patients require emergency
tic
dissection involving the aortic root. Acute mitral

regurgitation may be secondary to infective endocarditis
useful
or secondary to acute rupture ofthe chordae tendineae or

papillary muscles, or perforation of the valve leaflets secondary to AMI (usually inferior). Emergency surgery is
rarely indicated for stenotic valvular lesions.
exploratory laparotomy. Prognosis for these patients is

primarily dependent on timely diagnosis and surgical
Vascular Reconstruction
(2.
Infection
I 1.4. 1)
Vascular grafts are oftwo types: autogenous and pros-
thetic. Autogenous or autologous grafts (commonly the

saphenous vein) are typically used as bypass conduits for
occlusion
of
small arteries. Prosthetic grafts
are
employed for the bypass of medium-sized and large arterial vessels. The incidence of complications, especially
infection and thrombosis, appears to be higher with prosthetic grafts.
Emergency physicians must be aware of three impor-
tant complications of prosthetic grafts: aortoenteric fistula formation, infection, and thrombosis.
Aortoenteric Fistula
An aortoenteric fistula is a fistula formation between a

piece ofaortic graft and the gastrointestinal (GI) tract. It
is estimated to occur in up to 4o/o of all patients following
aortic bypass surgery. Approxirnately 80% of these fistulas develop at the level of the duodenum; the remainder
can be found anywhere along the GI tract. The incidence
of fistula development is thought to be higher following
emergency surgery than elective surgery. Aortoenteric
fistulas can develop at anytime, but most patients present
with symptoms within 3 to 5 years following surgery.
Gastrointestinal bleeding is the most common presentation, and can range from mild to massive. However,
patients may present with mild abdominal pain, back
pain, fever, or sepsis. The patient may only have hemepositive stool and abdominal discomfort on examination.
Any patient with a history of aortic bypass surgery and
gastrointestinal bleeding should be suspected of having
an aortoenteric fistula until proven otherwise.
intervention.
The incidence ofprosthetic graft infection varies from

0o/o
to 50 , depending on the site of graft implantation.
The incidence is highest in the groin region, where grafts

originate or terminate with the femoral artery. Risk fac-
tors for the development of graft infection include

implantation following emergency surgery a history of
wound complication (e.g., hematoma, lymph leak), or
multiple reoperations for graft related complications. In
50% ofthe cases the initial signs ofinfection are present
within I month of surgery.
Infections developing within 4 months of implantation
are considered to be early. They are usually due to
Staphylococcus aureLts or gram-negative organisms.
Graft infections are considered to be late if the patient
presents beyond 4 months. Late graft infections are most
commonly caused by mucin-producing S. epidermidis,
although other coagulase-negative staphylococci and
fungi have also been isolated.
Patients with graft infection typically present with
fever, redness, or swelling at the site of the incision, or
bleeding at the site of anastomosis. On examination these

patients may have fever, incisional erythema, swelling, or
tenderness; a draining or pulsatile mass; or signs of sepsis. Patients with intraabdominal grafts may present more
subtly, with abdominal pain, back pain, or unexplained
fever and sepsis.
A CBC usually demonstrates leukocytosis and a left

shift. Blood cultures are positive in less than 50% of
patients with proven graft infection. Ultrasonography, CT
and MRI appear to be the most useful imaging studies in
the evaluation of potential graft infections. Ultrasound is
excellent for grafts in the inguinal region, or where the
graft lies superficial. Ultrasound can detect perigraft
Cenorovescut-\R DrsoRDERs
fluid or gas and anastomotic pseudoaneurysms, and evaluate graft patency. CT is superior to ultrasound in evaluating the retroperitoneum. CT criteria of graft infection
include perigraft fluid" focal bowel wall thickening,
abnormal appearance of perigraft soft tissue, and
pseudoaneurysm formation. MRI can provide excellent
detailed visualization of soft tissue structures and planes
and is quite useful in differentiating perigraft soft tissue
from fluid.
Management includes surgical consultation, removal
of the infected graft material,
and administration of
broad-spectrum antibiotics. Patients with infected vascular grafts have an approximate 33oh mortalily rate, and
20o/o amputation rate, despite appropriate therapy.
767
coronary ostium and an angiogram is performed to visualize the diseased vessels. A balloon catheter is positioned at the stenosis and inflated for I to 2 minutes. A
final angiogram is performed to confirm adequate dilatation. Aspirin and heparin are administered to reduce the
complications of platelet deposition and thrombus formatlon.
Balloon-induced barotrauma causes endothelial
denudation; splitting and disruption of the atherosclerotic
plaque; separation of the plaque from the underlying
media; and stretching of the media and adventitia, with
the end result being aneurysmal dilatation and improved
luminal size. The goal is to reduce the residual stenosis to
less than 30Yoto 50Vo.
At the current time PTCA is performed on patients

with angina or objective evidence of myocardial ischemia. For the practicing emergency physician, it is imper-
Thrombosis
Thrombosis of vascular grafts can be due to a number

of causes, including inadequate anticoagulation,
ative to have an understanding of the role of PTCA in the

management of acute ischemic syndromes.
embolism, vascular graft defects,
atherosclerotic
changes, or fibrointimal hyperplasia. Fibrointimal hyperplasia is the most common cause, and typically develops
within I to 2 years following surgery. It usually develops
at the distal anastomosis and extends into the native ves-
sel. Progressive atherosclerosis is the most common

ofgraft failure after 2 years postsurgery.
Patients with vascular graft thrombosis will typically
present with symptoms of ischemia, such as pain and
paresthesias. Examination may reveal decreased or
absent pulses, cyanosis, or diminished skin temperature.
cause
Noninvasive peripheral vascular studies, such as Doppler

ultrasonography, is the initial study of choice. Other diagnostic studies include arteriography, digital subtraction
angiography, and CT.
A vascular surgeon should be consulted immediately
on all patients suspected of vascular graft thrombosis.
Options for revascularization include thrombectomy,
peripheral percutaneous transluminal angioplasty, and
thrombolytic therapy. Occasionally, the patient will

require replacement of the graft. Morbidity is directly
related to the interval between the onset of symptoms and
revascularization.
Angioplasty
(2.
I 1.4.2)
The first percutaneous transluminal coronary angioplasty (PTCA) was performed in 1977 by Gruntzig. Initially performed on only a small subset of patients, the
indications have broadened considerably over the past
several years due to improved catheter technology and
operator experience. In 1991 alone the procedure was
performed on nearly 300,000 patients in the United
States.
Vascular access for PTCA is usually obtained through

the femoral artery. A guide catheter is advanced into the
Unstable Angina
Most patients with unstable angina should be initially

managed with medical therapy, including oxygen,
nitrates, aspirin, and heparin. If the patient fails to
respond, then immediate cardiac catheterization, followed by PTCA or CABG, should be performed, depending on the anatomy of disease.
In the setting of acute myocardial ischemia or infarc-
tion, three different management strategies utilizing

PTCA have been studied.
Immediate or adjunctive angioplasty refers to the routine use of PTCA within 18 to 48 hours after the administration of intravenous thrombolytic therapy in acute MI.
It was thought that angioplasty following thrombolytic
therapy might improve the success of reperfusion. Several well-controlled studies, however, have revealed that
adjunctive PTCA fails to improve left ventricular function or survival, and is associated with an increased incidence of complications, such as bleeding. At the present
time, PTCA immediately following thrombolytic therapy
for AMI should only be performed in patients with continued symptoms or objective evidence of ongoing ischemla.
Primary angioplasty is the term used when PTCA

AMI, replacing
thrombolytic therapy. Recent studies comparing primary
PTCA and thrombolytic therapy in this setting have
demonstrated a higher success rate in successful reperfusion with PTCA. In addition, patients treated primarily
with angioplasty have a lower incidence of reinfarction
and death, and are less likely to experience recurrent
serves as the initial treatment strategy for
168 /
ErranncsNcy MnnrcrNn:
Tnr Coru
CunrucuLUM
ischemia or require subsequent revascularization. For

best results, admission to balloon inflation should be
within 60 minutes.
Despite the favorable results of primary PTCA, more
research is required to determine the optimal initial man-
agement strategy for AMI. Two groups of patients, however, appear to definitely benefit from primary PTCA.
First, patients in whom thrombolytic therapy is contraindicated, or are considered to be at high risk for complications (e.g., the elderly), should be considered for pri-
mary PTCA. The second group involves patients in

cardiogenic shock secondary to AMI. Retrospective studies suggest these patients have improved survival when
treated with primary angioplasty.
For primary angioplasty to be successful as an initial
management strategy, the hospital must have a 24-hour
catheterization laboratory, a well-trained angioplasty

team, and an interventional cardiologist capable of
responding in a timely fashion.
Rescue angioplasty refers to PTCA performed in
patients who fail to reperfuse following thrombolytic
therapy. Unfortunately, it can be difficult to determine
clinically which patients fail to reperfuse with thrombolytic therapy. Currently, candidates for rescue angioplasty include patients with persistent chest pain or continued marked ST:segment elevation, hemodynamic
instability or large anterior infarction. It appears rescue
angioplasty for interior MI's may be associated with an
increased risk of complications, such as ventricular
arrhythmias and heart block. In general, although rescue
angioplasty appears beneficial, more study is needed.
C ircu
latory Augm e ntation
(2. 1 I. 4. 3
improves peripheral tissue perfusion. The rapid deflation

ofthe balloonjust prior to systole decreases aortic pressure and reduces impedance to left ventricular outflow,
thereby unloading the left ventricle. The physiologic

result is a decrease in systolic arteial pressure, left ventricular wall tension, and end-diastolic filling pressure
and volume.
IABC should be considered in patients unable to main-
tain adequate tissue perfusion secondary to impaired

myocardial function, despite optimal circulating volume
and appropriate inotropic support. Ideally these patients
should have a Swan-Ganz catheter in place to allow for
the rational administration of fluids and titration of
inotropic agents. Current clinical indications for IABC
include patients in cardiogenic shock secondary to AMI,
or ventricular of papillary muscle rupture secondary to
AMI. In centers without cardiac surgery capabiliry IABC
may be used to stabilize these patients for transfer to an
appropriate facility.
Relative contraindications to IABC include the presence of tachydysrhythmias, peripheral vascular disease,
or prosthetic aortic grafts. Patients with tachydysrhythmias should have their rate controlled by medications or
pacing prior to IABC. Aortic regurgitation is an absolute
contraindication to IABC, since the increased diastolic
pressures will increase left ventricular afterload and diastolic volume with disastrous consequences.
Intraaortic balloon pumps should only be placed by
physicians skilled and experienced in their use. In most
circumstances, this will be a cardiologist or cardiovascular surgeon; therefore, early consultation is important.
Modern IABC kits now allow for insertion of the balloon
into the femoral artery via a percutaneous route, and does
not require surgical cutdown.
Indications for Mechanical Assistance
Mechanical circulatory systems were developed

approximately 30 years ago in response to the nearly universal failure of medical therapy to successfully treat cardiogenic shock. Today mechanical circulatory support
devices include intraaortic balloon counterpulsation,
portable cardiopulmonary bypass, and ventricular assist
devices.
Intraaortic
qlloon Counterpuls ation
Intraaortic balloon counterpulsation (IABC) decreases
myocardial oxygen demand while improving oxygen

availability, accomplished by assisting the heart in series
with the cardiac cycle. The balloon is positioned in the
descending aorta, then inflated at the onset of diastole.
This causes displacement of blood into the proximal and
distal aorta, increasing diastolic aortic pressures. The
resulting diastolic augmentation of blood flow increases
the cardiac output in the range of 20%o to 50o/o, and
Portable Cardiopulmonary Bypas s

Based on the same principles of the traditions cardiopulmonary bypass machine, there are now several
commercially available portable cardiopulmonary bypass
systems that can be applied percutaneously. In addition,
the use of heparin-coated peripheral bypass circuits
decreases the incidence of thrombus formation and
allows the machines to be used in patients with active
bleeding or the potential for bleeding.
In cardiopulmonary bypass, the central venous pressures are decreased and mean arterial pressure and output
are increased. The end result is reversal of shock and
return of adequate tissue and organ perfusion.
Portable cardiopulmonary bypass machinery is usually
available in hospitals with open heart surgery programs.
For cardiopulmonary bypass to be successful, the proper
equipment and the appropriate personnel must be available. The team should consist ofphysicians, nurses, and
perfusionists trained in emergency cardiopulmonary
CenuovRscur-aR DTsoRDERS
bypass and able to respond almost immediately on a 24hour basis. The decision to place a patient on portable
cardiopulmonary bypass should be made in consultation
with a cardiothoracic surgeon. Once the decision has

been made, the team should be activated. Physicians
capable of placing catheters percutaneously for use as
dialysis access conduits can also place cannulas for extracorporeal circulation.
Clinical indications for the use of emergency portable
cardiopulmonary bypass are not well delineated. For
emergency physicians probably the most frequent indication for placing a patient on cardiopulmonary bypass is
severe hypothermia; a heating unit is incorporated into
the circuit so that in addition to the patient being perfused
and oxygenated, he or she is also rewarmed; this is best
accomplished in the operating room with a traditional
cardiopulmonary bypass machine. However, if for some
reason this is not available (e.g., already in use) then a
portable machine could be utilized; otherwise, portable
cardiopulmonary bypass should be reserved for selected
patients who were witnessed having cardiac arrest.

Patients benefit most when cardiopulmonary bypass is
instituted early after cardiac arrest, but long-term survival
is possible even when application is delayed.
169
group of devices with multiple programmable functions.

There are multiple manufacturers producing implantable
defibrillator systems. Terminology used by different
manufacturers to describe similar features is variable.
Tiered-therapy implantable defibrillators deliver highenergy defibrillation shocks plus low-energy shocks, and
antitachycardia pacing to terminate ventricular tachycardia. These newer systems also provide backup ventricular
pacing for bradydysrhythmias.
There is concern about the interaction of implantable
defibrillators with medical procedures. The presence of
an implantable defibrillator should not stop standard

resuscitation techniques. In the presence of ventricular
fibrillation, it should be assumed that the implantable
defibrillator is not operating properly, and resuscitation
should proceed with defibrillation as usual. Epicardial
defibrillation patches may increase the energy requirement for external defibrillation. Persons performing cardiopulmonary resuscitation may feel a mild electric
shock from their hand contact with the patient's body
from an implantable defibrillator discharge. These shocks
are small enough to be harmless to those administering
CPR. External defibrillation shocks may damage the
pulse generator of an implantable defibrillator; therefore,
after external defibrillation, the pulse generator should be
Ventricular As sist Devi
ce
Ventricular assist devices are usually implanted while

the patient is in the operating room and receiving cardiopulmonary bypass. Their use is primarily reserved as
a temporary bridge for patients awaiting heart transplantation. Ventricular assist devices are of two types-pulsatile and nonpulsatile. Nonpulsatile pumps are used
almost exclusively for resuscitation as a temporary measure, while pulsatile ventricular assist devices can provide
Ionger term support for patients.
Implantable DeJibrillators
(2.
I 1.4.4)
In 1995 there were more than 50,000 patients worldwide who had already received defibrillator implants.
Implantable defibrillators are effective in reducing sudden cardiac death in patients with life-threatening ventricular tachydysrhythmias. The mortality benefit from
reduction in sudden death varies widely across subpopulations. This is due to the powerful influence of other clinical factors constraining survival in typical implantable
defibrillator patients. Implantable defibrillator therapy is
cost-effective when compared to other medical interventions and could be more so if the implant is carried out
early in the course of ventricular tachycardia-ventricular
fibrillation management according to one review article.
Implantable defibrillators cardiovert, defibrillate, or
pace patients hearts during dangerous ventricular dysrhythmias. Technology now includes a sophisticated
monitored for proper function.

General surgery, radiotherapy, lithotripsy, and electroconvulsive therapy can be safely done under continuous
ECG monitoring in patients with implantable defibrillators. The device should be deactivated before the procedure is done and reactivated and assessed immediately
afterward. MRI is contraindicated in patients with

implantable defibrillators.
The capacity of implantable defibrillators to recognize
and treat dysrhythmias can be disabled by placing a mag-
net on top of the device. The causes of multiple discharges from implantable defibrillators include ventricular electric storm, inefficient defibrillation, nonsustained
ventricular tachycardia, and inappropriate shocks from
supraventricular tachyarrhythmias or oversensing of signals. Patients with multiple defibrillator shocks should be
evaluated where there are resuscitation capabilities and
ECG monitoring. The defibrillator should not be deactivated until a diagnosis is established. Patients may
require sedation for anxiety. The defibrillator should be
interrogated as soon as possible. Institutions implanting
devices should be able to provide 24-hour coverage with
personnel available to manage these problems. It may be
that in smaller institutions delays will be unavoidable.
Infections of implantable defibrillator systems are

potentially life threatening and therefore should not be
taken lightly. Infection is more common after a generator
replacement than after initial implantation. Infections
360 days after implantation are commonly caused by
staphylococci. Later infections may result from transient
bacteremia, device skin erosion, or delayed infection
170 /
EurncsNcy MlnrcrNr: THn Conr CunnrcuI-uM
acquired after surgery. Infection of an implantable defibrillator system should be suspected when either local or
general signs of inflammation develop. Implantable

defibrillator infections should never be considered to be
localized. The treatment of choice for these infections is
removal of the entire system and administration of parenteral antibiotics. Empiric oral antibiotics should never
be given to patients with signs of inflammation at the
generator pocket, or to patients with implanted defibrillators and unexplained fever. Suspected defibrillator infections should be confirmed by culture. In those patients
too sick to await culture results, intravenous vancomycin
is the empiric treatment of choice since it provides good
coverage against coagulase-negative staphylococci,
methicillin-resistant S. aureus, Propionibacterium acnes,
and diphtheroids that are common causes of defibrillator
infection.
Psychiatric syndromes have been described in patients
with implantable defibrillators and include anxiety with
secondary panic reaction; defibrillator dependence,

abuse, or withdrawal; negative effect on body image; and
imaginary shocks. The need to use psychotropic agents
may arise frequently. Benzodiazepines are useful when
mild to moderate sedation is needed.
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Flaherty JT. Role ofnitrates in acute myocardial infarction Am J Cardiol
1 992;70(8):73B-8
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GUSTO Investjgators An international randomized trial comparing four
thrombolytic strategies for acute myocardial infarction. N Engl J Med
1993;329:673-682.
Habib GB. Current status of thrombolysis in acute myocardial infarction. I.
Optimal selection and delivery of a thrombolyic drug. Chest 1995;
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Emerg Me d 1994;24(6):1 I 54-l 1 60.
Hjalmarson A, Olsson G Myocardial infarction Effects of beta-blockade
C irculation 199 I ;84(6 suppl):VI 1 0 I - I 07.
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myocardial infarction. Management options for various settings. Paslgrad Med I 993;94(8):5 1-5 4,59-62,67 .
Katz NM, Wallace R. Emergency coronary bypass surgery: indications and
results. C ardi ovasc C lin 1986;1 6:67 -7 2.
Landau C, Lange RA, Hillis LD. Percutaneous transluminal coronary
angioplasty. N Engl J Med 1994;330:981-992
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1994;12:657-677.
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.{nn Intern Med 1991;115(l):3444.
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166-176.
Emerg
Med 1993;ll

Emergency Medicine: The Core Curriculum

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Contents

Gallbladder and Biliary Tract . . . .

1.6.6 VascularDisorders .....

Rectum and Anus

Congenital Abnormalities of the

John T. Meredith and

Michael K. Kerr and

Robert L. Brown and

Robert L. Brown and

Robert L. Brown and

John E. Gough and

John E. Gough and

Richard C. Hunt, and

Diseases of the Circulation,

Peggy E. Goodman and

Robert L. Brown and

Gary S. Setnik and

John T. Meredith and

Gary S. Setnik and

David P Hightower and

Eric W Schmidt and

Eric W Schmidt and

Eric W Schmidt and

Eric W Schmidt and

Eric W Schmidt and

EricW Schmidt and

Eric W Schmidt and

Eric W Schmidt and

Eric W Schmidt and

Eric W Schmidt and

Eric W Schmidt and

EricW Schmidt and

Steven F Siraco and

Steven E Siraco and

4.0 ENDOCRTNE, METABOLIC,AND

ENVIRONMENTAL DISORDERS..... 233

Gordian W O. Fulde and

William J. Levin and

Mlliam J. Levin and

Nasal Foreign Body . . .

Thomas Nowicki and

Thomas Nowicki and

Immune Deficiency Syndromes. .

Jehovah's Witnesses and

Thomas Nowicki and

6.4.3 PosteriorPole......... 354

SYSTEMIC INFECTIOUS DISORDERS 423

Chapter Editor: John B. McCabe

Low Back Syndromes. . .

Valerie Schevon Nicoletti

NERVOUS SYSTEM DISORDERS. . . . .

Philip D. Anderson and

Chapter Editor: G. Richard Braen

Obstetrics and Disorders

Debra L. Weiner and

Paula J. Schweich and

13.1.4.4 Pyloric Stenosis

Carmen Teresa Garcia

Alison St. Germaine Brent

Tamara Ingrid Pottker and

Lower Respiratory Tract

Michael J. Fairley and

Michael J. Fairley and

(HIV) and Acquired