Cardiovascular System I

Objectives
Present the clinical features and
emergency management of
cardiovascular disorders, including:
Recognize congenital and acquired heart
disease.
Outline management of ductal dependent
lesions.
Identify patients with myocarditis.
Congenital Heart Disease:
Recognition and Stabilization
Rapid cardiopulmonary assessment to
recognize and manage life-threatening
illness caused by heart disease
Understand the physiology of different
conditions to optimize treatment plans.
Critical Concepts
Dysrhythmias can cause serious
cardiovascular compromise.
Structural congenital heart disease can
present in many different ways at many
different ages.
Acquired heart disease can be subtle
yet life-threatening.
Case Study 1:
Rapid Breathing
10-day-old infant is brought to ED by
mother for rapid breathing and not
eating well.
Product of normal spontaneous vaginal
delivery
Spent 2 days with mother in hospital
Uneventful course, including circumcision
Birth weight 3.2 kg
Case Study 1 (continued)
Slow to breastfeed since birth
Would gasp and cry after sucking for a short
time. Difficulty feeding.
3 to 4 wet diapers per day
No congestion, no fever
No vomiting with feedings
2 yellow seedy stools since passing
meconium after birth
Initial Assessment (1 of 2)
PAT:
Abnormal appearance, abnormal
breathing, abnormal circulation
Vital signs:
HR 170, RR 70, BP 82/40, T 37C (rectal),
Wt 3.4 kg, O
2
sat 90% on room air
Initial Assessment (2 of 2)
A: No evidence of obstruction
B: Elevated RR and labored
C: Pale, diaphoretic, tachycardia, weak
pulse, cyanosis
D: GCS grossly normal but in distress and
inconsolable
E: No signs of head injury, fractures, or
bruising
Detailed Physical Exam
Lung sounds equal bilaterally with rales in
both bases
Hyperactive precordium with a gallop rhythm
Pulses weak in distal and lower extremities
Distended abdomen with liver palpable 4 cm
below right costal margin
Question
What is your general impression of this
patient?
General Impression
Impending cardiopulmonary failure
(compensated shock)
Cyanosis, diaphoresis
Pale, tachycardia
What are your initial management
priorities?
Management Priorities (1 of 3)
ABCs
Give 15L O
2
by nonrebreather mask or
100% O
2
by BMV, or perform endotracheal
intubation.
Start IV, obtain blood glucose.
ECG and monitor rhythm on cardiac monitor
CXR
Administer fluid challenge: 10 cc/kg NS
Management Priorities (2 of 3)
Administer prostaglandin E
1
(PGE
1
):
0.05 to 0.1 mcg/kg/min
Intubate to protect against apnea and
relieve stress from work of breathing.
Consider furosemide (0.5 to 1 mg/kg).
Sepsis work-up and then antibiotics
Defer lumbar puncture.
Management Priorities (3 of 3)
Cardiology consultation or transfer to
pediatric cardiology center emergently:
Echocardiogram
If blood pressure and perfusion do not
improve, add inotropic agent:
Dobutamine: 2 to 20 mcg/kg/min
Epinephrine: 0.1 to 1.5 mcg/kg/min
Case Discussion (1 of 2)
This infant is in CHF.
Poor feeding and easy fatigability
Gallop rhythm and enlarged liver
Diminished pulses
Shock:
Altered mental status, compensated shock
(tachycardia, diaphoresis, respiratory distress,
normal BP in upper extremities)
Case Discussion (2 of 2)
Possible ductal dependent lesion:
Right age for presentation of shock
triggered by closure of the ductus
arteriosus
Measure blood pressure in four
extremities
Assess oxygenation response to
supplemental oxygen
Case Progression: Version 1
BP differential noted in lower extremities.
Oxygenation improves to 99% with
supplemental oxygen.
CXR shows cardiomegaly and pulmonary
edema.
Echocardiogram demonstrates coarctation of
the aorta.
Infant improves with PGE
1
infusion,
diuretics, and inotropes.
Case Progression: Version 2
Oxygenation fails to improve with
supplemental oxygen (remains 90%).
Oxygenation declines further to <80%.
CXR is nonspecific.
Echocardiogram demonstrates transposition
of the great vessels.
Infant improves with PGE
1
infusion.
Surgical intervention is scheduled.
Background: Structural
Congenital Heart Disease
Congenital heart disease: 5 to 8 cases per
1,000 live births
Child with congenital anomaly usually does
not show cardiovascular problems in utero.
Changes at birth place great stress on
infants cardiovascular system.
Some cyanotic heart conditions are highly
dependent on shunting through ductus
arteriosus. Closure can be terminal event.
Clinical Features: Your First Clue
Age
Progressive deterioration (mild)
followed by suddenly progressing to
critical condition
Cyanosis
Congestive Heart Failure (CHF)
Consider concurrent sepsis
Diagnostic Studies (1 of 3)
Radiology:
Pulmonary hypoperfusion: pulmonic stenosis,
TOF, TA
CHF (if large VSD present to allow high-output
failure, e.g., increased right-sided flow)
Some classic CXR appearances (more classic if
condition is permitted to worsen):
TGA: Egg on side
TAPVR: Snowman
TOF: Boot shaped
Diagnostic Studies (2 of 3)
ECG:
Right axis (RVH): Normal for newborns
Left axis: Hypoplastic right heart, tricuspid
atresia, endocardial cushion defect (AV canal)
ST-T changes, strain, ischemia
Dysrhythmia
Prolonged QT
Low voltage
Diagnostic Studies (3 of 3)
Laboratory:
Glucose: Any child in distress needs to have
hypoglycemia excluded.
CBC: Look for anemia, signs of sepsis.
Electrolytes: Congenital adrenal hyperplasia,
salt-wasting form
Arterial blood gas: Hyperoxia text

Fetal Circulation (1 of 2)
Placenta oxygenates
blood and returns to
right atrium (RA) via
IVC.
Preferentially shunts
across FO to LA.
LV ejects most
oxygenated blood to
carotids and coronaries.
Fetal Circulation (2 of 2)
Superior vena cava (SVC) returns
deoxygenated blood to RA where it mixes
with oxygenated blood from the placenta.
Preferentially enters RV.
RV ejects into PA.
No pulmonary capillary flow, so PA is
shunted into the descending aorta via the
ductus arteriosus.
Coarctation of the Aorta (1 of 2)
Coarctation
of the Aorta
(2 of 2)
Transposition
of the Great
Arteries
Differential Diagnosis: What
Else? (1 of 2)
Other cyanotic and acyanotic
congenital structural heart disease
Ductal dependent coarctation
Hypothermia
Sepsis
TORCH
Differential Diagnosis: What
Else? (2 of 2)
Congenital adrenal hyperplasia (CAH)
Hypoglycemia
Shaken baby syndrome/intracranial
lesion
Catastrophic gastrointestinal process,
e.g., volvulus
Normal CV System Function
Represented by vital signs (O
2
sat included)
Factors affecting cardiac output (perfusion):
Heart rate
Stroke volume
Contractility
Vascular resistance
Children <8 years predominantly increase
their HR to increase cardiac output (unable
to increase stroke volume until >10 years).
Normal Vital Signs For Age
HR RR BP (systolic)
Newborn 90-180 40-60 60-90
1 month 110-180 30-50 70-104
3 months 110-180 30-45 70-104
6 months 110-180 25-35 72-110
1 year 80-160 20-30 72-110
2 years 80-140 20-28 74-110
4 years 80-120 20-26 78-112
6 years 75-115 18-24 82-115
8 years 70-110 18-22 86-118
10 years 70-110 16-20 90-121
12 years 60-110 16-20 90-126
14 years 60-110 16-20 92-130
Transition from Fetal
Circulation
Placental circulation is interrupted at birth:
Increase in systemic arterial blood pressure
Spontaneous respirations
Decreased pulmonary vascular resistance,
increasing pulmonary blood flow
Foramen ovale closes.
Ductus arteriosus closes.
This initial rapid change slows down over
first 24 hours of life.
Cyanotic Heart Disease (CHD)
Cyanotic: Refractory to oxygen
Right to left shunting
Some lesions (e.g., TGA) are highly
dependent on a shunt (VSD, PDA)
Cyanosis usually presents shortly after
birth.
Cyanotic CHD
5 Ts:
Truncus arteriosus
Tetralogy of Fallot (TOF)
Transposition of the great arteries (TGA)
Tricuspid atresia
Total anomalous pulmonary venous return
(TAPVR)
Severe aortic stenosis
Hypoplastic left heart
Severe coarctation of the aorta
Tetralogy of
Fallot (TOF)
Pulmonic stenosis
Aortic override
VSD
RVH
Right-to-left
shunting through
VSD dependent
on severity of
pulmonic stenosis
Tricuspid
Atresia
RV is
hypoplastic.
Right-to-left
shunt
through
VSD
Total
Anomalous
Pulmonary
Venous
Return
(TAPVR)
Cyanosis
Respiratory disorder
Hemoglobin disorder
Acrocyanosis (normal newborns): Cold
stress and peripheral vasoconstriction
Generalized or central cyanosis often
due to cyanotic congenital heart
disease. Often worsened by crying.
Central Cyanosis vs.
Acrocyanosis
Hyperoxia Test
Administer 100% oxygen.
Significant increase in PaO
2
seen with
pulmonary and hemoglobin disorders.
In CHD, PaO
2
will not increase or it will
increase slightly.
Deoxygenated blood bypasses lungs and goes
directly to left side of heart, diluting the fully
oxygenated blood coming from lungs with
deoxygenated blood.
CHD
Increased pulmonary vascularity:
Total anomalous pulmonary venous return
Truncus arteriosus
Transposition of the great arteries
Other complex lesions without pulmonic stenosis
Decreased pulmonary vascularity:
Tetralogy of Fallot
Ebsteins anomaly
Hypoplastic right heart, tricuspid atresia
Complex lesions with pulmonic stenosis
Prostaglandin E
1
Keeps the ductus open
0.05 to 0.1 mcg/kg/min with an
increase to 0.2 mcg/kg/min over
several minutes
Side effects: Apnea, pulmonary
congestion, fever, hypotension,
seizures, and diarrhea
Consider elective intubation.
Noncyanotic CHD
May present with CHF or heart
murmurs heard during physical exam
Left-to-right shunts
Excess pulmonary vascularity
ASD, VSD, PDA
Obstructive lesions
Aortic stenosis, coarctation of the aorta,
mitral stenosis, pulmonic stenosis
Clinical Features
CHF: Tachypnea, tachycardia,
diaphoresis, decreased feeding,
hepatomegaly, murmurs, gallop
rhythms, pulmonary edema
Decreased activity or poor sleeping
with respiratory distress
Diagnostic Studies
CXR: Cardiomegaly, pulmonary
vascular congestion
ECG: Abnormal axis, ST segment
changes
Echocardiogram: Definitive anatomic
diagnosis, degree of congestive heart
failure (chamber sizes, contractility)
Management of CHF
Give oxygen, assisted ventilation if needed.
Elevate head and shoulders 45 degrees.
Monitors, pulse oximetry
Obtain IV access.
Send laboratories.
CXR and ECG
Furosemide, nitroglycerin, digoxin
Inotropes (dobutamine) for signs of shock
Case Study 2:
Chest Pain, SOB
10-year-old boy presents with chief
complaint of chest pain and shortness
of breath.
5 days of cold and cough symptoms
He has been lying around a lot and has
missed 1 week of school.
Usually a very active child but complains
that he is just too tired to play
Initial Assessment
PAT:
Abnormal appearance, abnormal
breathing, abnormal circulation
Vital signs:
HR 130, RR 44, BP 90/65, T 37.8C, O
2

sat 90% on room air, increases to 100%
on O
2
Initial Assessment
A: Patent
B: Intermittently shallow and deep;
rapid respiratory rate
C: Pale; pulse rapid, thready, and
weak
D: No focal deficits, GCS 15
E: No signs of injury
Focused History
O: Chest hurts for several days.
P: Provoked by cough and exertion; short of
breath whenever he gets up and walks
Q: Burning, pressure
R: Substernal, some radiation to shoulders
S: 3 to 8 out of 10
T: Pressure and SOB last almost all day,
exacerbations with exertion last 15 to 30
min.
Detailed Physical Exam
Neck: Jugular venous distention supine
Lungs: Diminished breath sounds with
occasional end expiratory wheeze with deep
breaths
Cardiac: Distant heart sounds, no murmurs,
S
3
gallop rhythm
Abdomen: Distended with palpable spleen
and liver
Neuro: No focal deficits
Question
What is your general impression of this
patient?

What are your initial management
priorities?
General Impression
Child is in respiratory distress and in
cardiogenic shock.
Demonstrates abnormal appearance with
increased work of breathing and signs of
shock.
Management Priorities
ABCs
Give O
2
by nonrebreather mask.
Obtain IV access.
Check rhythm on cardiac monitor.
Obtain blood glucose, lab studies.
Consider reducing preload and afterload
with nitrates.
Consider diuretic therapy.
May need inotropic support.
Case Discussion:
Differential Diagnosis
Acquired cardiac problem:
Respiratory illness during winter months
causing secondary myocarditis
Congenital heart lesion that had been
asymptomatic until this illness:
Anomalous coronary artery or valvular
disease
Pericarditis
Clinical Features: Your First Clue
Consider myocarditis in any child with:
Weakness
SOB
Chest pain
Especially if associated with preceding
prodromal viral illness
Distant heart sounds: Silent Chest
Enlarged heart on CXR
Diagnostic Studies: Myocarditis
Radiology:
CXR will reveal
cardiomegaly
and prominent
vasculature,
perhaps even
pulmonary
edema
Laboratory:
May not add
much
Not specific
Differential Diagnosis: What Else?
Pericarditis
Hypertensive crisis
Anomalous coronary artery and myocardial
ischemia/infarction
Valvular disease
Structural cardiac disease (e.g., VSD, ASD)
Renal failure (e.g., glomerulonephritis)
Rheumatic fever
Management: Myocarditis
Gentle diuretic therapy
Afterload reduction
Possibly inotropic support
Echocardiogram
Intrinsic cardiac lesion?
Muscle hypertrophy?
Pericardial effusion?
Decreased contractility?
Case Progression: Version 1
CXR: Cardiomegaly
Echocardiogram: Poor cardiac contractility
Diagnosis: Myocarditis
Maintained on inotropes and pressor agents
Recovered to a point that he could be
discharged 2 weeks later
Will be followed closely to assess the degree
to which he regains cardiac function
Case Progression: Version 2
CXR: Cardiomegaly
Echocardiogram: Poor cardiac contractility
Diagnosis: Myocarditis
Deteriorates in ED:
Progressive shock
Requires inotropic support
Develops V-tach and V-fib
Extracorporeal membrane oxygenation (ECMO)
Myocarditis
Inflammatory disease of the myocardium:
Direct infection of the myocardium (e.g., viral
myocarditis)
Toxin production (e.g., diphtheria)
Immune response as a delayed sequela of an
infection (postviral or postinfectious myocarditis)
A common type of myocarditis is acute rheumatic
fever (ARF).
Acute Rheumatic Fever:
Jones Criteria
Major criteria:
Carditis: Most commonly valvulitis
Migratory polyarthritis
Chorea, erythema marginatum,
subcutaneous nodules
Minor criteria:
Fever, elevated CRP or ESR, prolonged
PR interval, arthralgia
Pericarditis
Pericardial inflammation
Viral versus bacterial
Bacterial causes include
pneumococcus, S. aureus, H.
influenzae type B
Cardiac tamponade possibly requiring
pericardiocentesis
Pericarditis: Clinical Features
Chest pain
Respiratory distress, CHF, or tamponade
Precordial "knock" or rub (like the sound of
shoes walking on snow)
The classic signs include exercise
intolerance, fatigue, jugular distension, lower
extremity edema, hepatomegaly, poor distal
pulses, diminished heart tones, and pulsus
paradoxus.
Endocarditis
An infection of the endothelial surface of the
heart, with a propensity for the valves
Increased risk in children with artificial valves
and patches, and patients with central lines
90% of cases are caused by gram-positive
cocci.
Alpha strep, Staph aureus, pneumococcus,
group A hemolytic streptococci
Endocarditis Clinical Features
Fever
Tachycardia, CHF, dysrhythmia, cardiogenic
shock
History of recent cardiac surgery or
indwelling vascular catheter
Heart murmur
Petechiae, septic emboli, or splenomegaly
Kawasaki Disease
Vasculitis: Propensity for coronary
aneurysms
Aneurysms may subsequently scar,
resulting in coronary stenosis (early
onset coronary artery disease).
Coronary artery thrombosis and
myocardial infarction
Myocarditis, dysrhythmia
Kawasaki Disease:
Clinical Features
High fever
Conjunctivitis
Cervical lymphadenopathy
Gingivostomatitis
Polymorphous rash
Swelling of the hands with erythema of
the palms
The Bottom Line
Assessment of congenital heart
disease can be challenging; however,
applying assessment skills with an
understanding of normal physiology as
well as pathophysiology of
cardiovascular disorders in children will
assist the clinician in management.