Eisenmenger

Syndrome

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In 1897, Eisenmenger reported the case of

a 32-year-old man who had showed
exercise intolerance, cyanosis, heart
failure, and haemoptysis prior to death.
Autopsy showed a large ventricular septal
defect (VSD) and overriding aorta. This
was the first description of a link between
a large congenital cardiac shunt defect and
the development of pulmonary hypertension

Pathophysiology

Patients with large congenital cardiac or surgically created

extracardiac left-to-right shunts
increased pulmonary blood
flow
pulmonary vascular disease
pulmonary hypertension
Early stages remains reactive to pulmonary vasodilators
With continued insult becomes fixed & ultimately the level of PVR
becomes so high resulting in reversed or bidirectional shunt flow
with variable degrees of cyanosis.
Lesions with high shear rate e.g.-large VSD/PDA- pulm. Htn in
early childhood
Lesions with low shear rate- pulm. Htn in late middle age
High altitude- early onset

Approximately 50% of infants with a large, nonrestrictive VSD or PDA

develop pulmonary hypertension by early childhood.

40% of patients with VSD or PDA and transposition of the great

arteries develop pulmonary hypertension within the first year of life.

Large ASD 10% progress to pulmonary hypertension, slowly and

usually not until after the third decade of life.

All patients with persistent truncus arteriosus and unrestricted

pulmonary blood flow, and almost all patients with common
atrioventricular canal, develop severe pulmonary hypertension by the
second year of life.

10% of those with a Blalock-Taussig anastomosis (subclavian artery

to pulmonary artery) develop pulmonary hypertension compared to
30% of those with a Waterston (ascending aorta to pulmonary artery)
or a Potts (descending aorta to pulmonary artery) shunt.

Prognosis

Median survival- 80% at 10 yrs after diagnosis & 42%

at 25 yrs.
Long-term survival depends on the age at onset of
pulmonary hypertension and right ventricular function
Syncope, increased CVP, SPO2 < 85%- poor short
term outcome.
Most deaths- sudden cardiac death
Other- heart failure, haemoptysis, thromboembolism,
brain abscess & complications of pregnancy and non
cardiac surgery
Saha etal Int J cardiol. 45:199,1994

Vongpatanasin W etal Ann. Intern. Med. 128:745,1998

History

Pulmonary hypertension- Breathlessness, Fatigue,

Lethargy, Severely reduced exercise tolerance with
a prolonged recovery phase, Presyncope, Syncope
Heart failure- Exertional dyspnea, Orthopnea, PND,
Edema, Ascites, Anorexia, Nausea
Erythrocytosis- Muscle weakness, Anorexia,
Myalgias, Fatigue, Lassitude, Paresthesias of the
digits and lips, Tinnitus, Blurred or double vision,
Scotomata, Slowed mentation
Bleeding tendency
Palpitations- often due to AF/flutter
Haemoptysis- pulmonary infarction, rupture of
pulmonary vessels or aortopulmonary collateral
vessels

Cardiovascular findings

Central cyanosis (differential cyanosis in the case of a PDA)

Clubbing
JVP- dominant A-wave
central venous pressure may be elevated.
Precordial palpation- right ventricular heave, palpable S2.
Loud P2
High-pitched early diastolic (Graham steell) murmur of
pulmonic insufficiency
Right-sided fourth heart sound
Pulmonary ejection click
The continuous murmur of a PDA disappears when
Eisenmenger physiology develops; a short systolic murmur
may remain audible.

Other findings

Respiratory - cyanosis and tachypnea.

Hematologic - bruising and bleeding; funduscopic
abnormalities related to erythrocytosis include engorged
vessels, papilledema, microaneurysms, and blot
hemorrhages.
Abdominal - jaundice, right upper quadrant tenderness, and
positive Murphy sign (acute cholecystitis).
Vascular - postural hypotension and focal ischaemia
(paradoxical embolus).
Musculoskeletal - clubbing, hypertrophic osteoarthropathy
Ocular signs include conjunctival injection, rubeosis iridis,
and retinal hyperviscosity change

Lab investigations

Complete blood count

Red cell mass is increased with erythrocytosis.

Bleeding time is prolonged by platelet dysfunction, VWF dysfunction
Biochemical profile

Increased conjugated bilirubin

Increased uric acid
Urea and creatinine sometimes elevated
Erythrocytic hypoglycemia is an artifactually low blood glucose level caused by
increased in vitro glycolysis in the setting of increased red cell mass.

Iron studies

Erythrocytosis increases hematocrit and hemoglobin concentration.

Phlebotomy-related iron deficiency decreases the mean corpuscular volume and
mean corpuscular hemoglobin concentration.

Reduced serum ferritin due to phlebotomy-related iron store reduction

Increased total iron binding capacity

Urine biochemical analysis reveals proteinuria.

Arterial blood gases

Reduced resting PaCO2 due to resting tachypnea and reduced PaO2 due to right-toleft shunting
Mixed respiratory and metabolic acidosis

Chest radiograph

Right ventricular and right atrial enlargement

Features of pulmonary hypertension - dilated main pulmonary
artery, increased hilar vascular markings, and pruned
peripheral vessels

Electrocardiogram

Almost always abnormal results and includes signs of

right heart hypertrophy in addition to abnormalities
associated with the underlying defect
Tall R wave in V1, deep S wave in V6, ST and T wave
abnormalities
P pulmonale
Atrial and ventricular arrhythmias
Incomplete right bundle branch block is present in 95% of
ASDs.
Vertical frontal plane QRS axis usually is present with
ostium secundum ASD.
Left axis deviation commonly is present with ostium
primum ASD.

Echocardiogram

Transthoracic echocardiogram
The structural cardiac defect responsible for the shunt can be
defined by the 2-dimensional imaging.
The location of cardiac shunt can be demonstrated by color
Doppler or venous agitated saline contrast imaging.
The pressure gradient across the defect can be estimated.
Estimated pulmonary artery systolic and diastolic pressures
Identification of coexistent structural abnormalities
Left and right ventricular size and function
Identification of surgical systemic-to-pulmonary shunts
The addition of supine bicycle ergometry can demonstrate
increased right-to-left shunting with exercise.
Transesophageal echocardiogram is useful for imaging posterior
structures, including the atria and pulmonary veins.

Apical 4-chamber transthoracic view demonstrating an

ostium ASD with enlarged right-side chambers.

Cardiac catheterization
Severity of pulmonary vascular hypertension
Conduit patency and pressure gradient
Coexisting coronary artery anomalies (rare)
Degree of shunting

Medical Treatment
Fluid balance and climate control
Avoid sudden fluid shifts or dehydration, which may increase right-toleft shunting.
Avoid very hot or humid conditions, which may exacerbate
vasodilatation, causing syncope and increased right-to-left shunting.
Oxygen supplementation
Use is controversial
Oxygen therapy has been shown to have no impact on exercise
capacity and survival in adult patients with Eisenmenger syndrome
Sandoval etal Am J Respir Crit Care Med. 2001 Nov 1;164(9):1682-7

Continuous home oxygen therapy better than nocturnal

supplementation
Better results in children and at early stages. Bowyer etal Br Heart J. 1986 Apr;55(4):385-90
Most useful as a bridge to heart-lung transplantation.

Medical Treatment

Erythrocytosis - rule out dehydration. Then, if symptoms of hyper

viscosity and the haematocrit is greater than 65%, venesect 250500 mL of blood and replace with an equivalent volume of isotonic
sodium chloride (or 5% dextrose if in heart failure).
For resuscitation in the event of massive acute bleeding, replace
losses with FFP, cryoprecipitate, and platelets.
Infective endocarditis prophylaxis
Encourage good oral hygiene
Anticoagulation- increased risk of bleeding, hence not routinely

used. Silversides et al J Am Coll Cardiol 2003 Dec 3; 42(11): 1982-7

Digoxin, diuretics for right heart failure

Medical Treatment
Pulmonary vasodilator therapy
Long-term prostacyclin therapy- Improvement in haemodynamics,
suturation & 6 minute walk test. Rosenzweig etal, Circulation 1999 Apr 13; 99(14): 1858-65
Fernandes etal Am J Cardiol 2003 Mar 1; 91(5): 632-5

Bosentan, an endothelin receptor antagonist Christensen,Am J Cardiol 2004 Jul 15; 94(2): 261-3
Schulze-Neick et al Am Heart J 2005 Oct; 150(4): 716

Treatment with prostacyclin analogues and/ or endothelin receptor

antagonists delayed the need for transplantation. Adriaenssens, Eur Heart J 2006 Jun;
27(12): 1472-7

Sildenafil- Singh TP etal Am Heart J 2006 Apr; 151(4): 851

Pregnancy
To be avoided
Therapeutic abortion in first trimester

Surgical options
Heart lung transplant
Procedure of choice if repair of the underlying cardiac defect is not
possible.
Performed successfully for the first time in 1981.
Reported actuarial survival rates are 68% at 1 year, 43% at 5 years,
and 23% at 10 years.
The main complications are infection, rejection, and obliterative
bronchiolitis
Bilateral lung transplantation
Preferable procedure if the cardiac defect is simple (e.g.- ASD)
Repair of the underlying cardiac defect is required
Better than single-lung transplantation in terms of mortality, New
York Heart Association functional class, cardiac output, and
postoperative pulmonary edema.
Advantages over heart-lung grafting include no transplant coronary
artery disease or cardiac rejection.

Corrective surgery options

Repair of the primary defect is contraindicated
in the context of established severe pulmonary
hypertension.
Corrective surgery may be possible if a
significant degree of left to- right shunting
remains and if responsiveness of the
pulmonary circulation to vasodilator therapy
can be demonstrated.
Limitation - transient dynamic right ventricular
outflow tract obstruction.

Activity
Intense athletic activities carry the risk of sudden death.
Exercise prescription can be individualized based on
exercise testing that documents a level of activity that
meets the following 3 criteria:
Oxygen saturations remain greater than 80%.
No symptomatic arrhythmias.
No evidence of symptomatic ventricular dysfunction
Diet
Excessive sodium intake to be avoided

Anaesthetic
considerations

Eisenmenger pts pose a difficult challenge

as they have lost the ability to adapt to
sudden changes in haemodynamics
because of fixed pulmonary vascular
disease
Colon-Otero G etal Mayo Clin Proc 1987;62:37985.

Preoperative assessment

Assessment of medical condition

Assessment of anotomical defect and physiology
Non-cardiac/ cardiac surgery/ pregnancy for labour
analgesia

Goals
Prevent further increase in Rt to Lt shunt
Maintain CO
Prevent arrhythmias
Avoid hypovolemia, PVR, SVR
Marked increase in SVR should also be
avoided as excessive systemic vasoconstriction
can precipitate acute LVH

What To Do?

Prevention of prolonged fasting & dehydration

Sedation to reduce preop anxiety and oxygen consumption
Keep phenylephrine/ Norepinephrine infusion,
anticholinergic, antiarrythmics ready
Monitoring- Pulse oximetry, ECG, ETCO2, Arterial catheter
for IBP monitoring and serial ABG monitoring, CVP, AWP.
(PAC- better to avoid)
TOE- to know status of the shunt, to guide fluid therapy by
looking at ventricular function, to measure pulmonary artery
pressure. Bouch DC, Anaesthesia. 2006 Oct;61(10):996-1000
Avoid factors known to increase PVR viz. cold, hypercarbia,
acidosis, hypoxia,

Air Bubble precautions

To prevent paradoxical air embolism

Remove all bubbles from iv tubing
Connect the iv tubing to the venous cannula while there is free
flowing in fluid .
Eject small amount of solution from syringe to clear air from the
needle hub before iv injection
Aspirate injection port before injection to clear any air
Hold the syringe upright to keep bubbles at the plunger end
Do not leave a central line open to air
Use air filters
? No N2O.

Which anaesthetic technique to use?

Regional blocks - low mortality (5% vs 18% for

G.A.).Mortality more dependent on the surgical procedure
rather tan anaesthetic technique. Martin JT et al, Reg Anesth Pain Med. 2002 SepOct;27(5):509-13.

General anaesthesia
Induction with high dose opioid (short acting) technique or
with ketamine, etomidate or low dose thiopentone
Cardiostable inhalational agent- isoflurane, sevoflurane,
xenon. Hofland J Br J Anaesth. 2001 Jun;86(6):882-6.
Muscle relaxation with atracurium, vecuronium
TIVA with propofol, remifentanil. Kopka A, Acta Anaesthesiol Scand. 2004 Jul;48(6):782-6
Some patient may not tolerate positive pressure ventilation
and PEEP well

Anaesthetic technique

Single shot SAB contraindicated rapid drop in SVR

Low-dose bupivacaine-fentanyl spinal anesthesia has been
successfully used for lower extremity surgery in a nonparturient with
Eisenmenger's syndrome Chen CW et al, J Formos Med Assoc. 2007 Mar;106(3 Suppl):S50-3
Graded epidural can be safely used
Ropivacaine, Levobupivacaine theoretically better- less
cardiotoxicity
Continuos spinal anaesthesia with slow increments of doses titrated
against the haemodynamic and anaesthetic effects. Cole PJ, Br J Anaesth. 2001
May;86(5):723-6.

Pulmonary vasodilator therapy intraop.

100 % oxygen
Nitric oxide- 5 -20 ppm. Bouch DC etal, Anaesthesia. 2006 Oct;61(10):996-1000
Prostacycline- infusion or nebulization

Postoperative care

Observation on a monitored bed in ICU/HDU for 24 hours or

overnight atleast because of their predisposition to develop
ventricular/ supraventricular tachycardia, bradyarrhythmia and
myocardial ischemia
Meticulous attention to fluid balance to prevent hypovolumia
Monitoring of blood pressure preferably invasive, Oxygen saturation
and CVP
Position slowly- risk of postoperative postural hypotension with
secondary increase in right to left shunting
Prevention of venous stasis by early ambulation and by applying
effective elastic stocking or periodic pneumatic compression.
Adequate pain management adverse hemodynamics and possibly
hypercoagulable state

Eisenmenger and
pregnancy

Pts with Eisenmenger do not tolerate

pregnancy well because
Decreased SVR during pregnancy
Decreased FRC & increased oxygen
consumption exacerbate maternal hypoxemia
decreased O2 delivery to fetus IUGR & fetal
demise

Risks related with pregnancy

Spontaneous abortions- 20- 30%

Premature delivery- 50%

IUGR- 50% of born.

Maternal death- 30-45% intrapartum or first post partum weak

Successful first pregnancy doesnt preclude maternal death during
subsequent pregnancy Gleicher N: Obstet Gynecol Surg 34:721, 1979
Factors influencing mortality- thromboembolism, hypovolumia,
preeclampsia
Mortality is similar with ceasarean section or vaginal delivery
Mortality reaches to 80% in presence of preeclampsia

Avila WS: Eur. Heart J. 16:460,1995

In O.T.

General measures- preparation and monitoring same as

described before+ left uterine displacement, anti aspiration
prophylaxis, preparation for neonatal resuscitation
If vaginal delivery planned- give labour analgesia
CSE technique preferred- Intrathecal fentanyl/ sufentanil + very
low dose L.A. in first stage of labour, then small, incremental
dose of L.A.
Use of continuous spinal anaesthesia and postop analgesia also
reported. Sakuraba s, J Anesth. 2004;18(4):300-3.

G.A
Post op monitoring

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anaesthesia.co.in@gmail.com

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