Eisenmenger Syndrome

Anita Saxena

Department of Cardiology,
All India Institute of Medical Sciences
New Delhi, India 110029
Eisenmenger Syndrome
1887 : Victor Eisenmenger
described history and
postmortem details of 32
year old man with VSD and
pathological features of
PAH
Eisenmenger Syndrome
1958: Paul Woods
Croonian Lectures
coined the term
Eisenmenger
Syndrome
Eisenmenger Syndrome
Definition:
Pulmonary hypertension at or near
systemic level with reversed or
bidirectional shunt between the
pulmonary and systemic circulation
and pulmonary vascular resistance
above 800dyn/cm
-5
(10 Wood Units)
Paul Wood, Br Med J, 1958
Eisenmenger Syndrome
Underlying Basic Lesions
Type of lesion Somerville 98 Daliento et al 98
(n=132) (n=188)

Ventricular Septal Defect 45 71
Atrial Septal Defect 6 21
Patent ductus arteriosus 12 36
Atrio ventricular septal defect 16 23
Truncus arteriosus 15 11
Single ventricle 13 9
Transposition of great arteries 5 8
Others 20 9
Eisenmenger Syndrome A
progressive disease
Eisenmenger Syndrome
Mechanism of abnormal pulm vascular response
Stimulation of insulin like growth factor
Impaired relaxation of pulmonary arterioles
Increased endothelin production
Elevated plasma thromboxane B
2

Exact mechanism not clear
Pulmonary Arterial Hypertension

Hyperkinetic Obstructive
(Eisenmengers)
Heart Size Large Normal
Parasternal
impulse Hyperkinetic Forcible
Click Absent Present
S
2
ASD wide & fixed wide & fixed
VSD wide & variable Single
PDA paradoxic split normal split
Shunt murmur present short/absent
Flow murmur Present Absent
Question 1
At what age a large VSD Eisenmengerize?
1. < 6 months
2. 2 years
3. 10 years
4. 20 years
1
Eisenmenger Syndrome
Clinical Groups
Cyanosis since birth: TGA, Truncus,
Univentricular hearts
Failure to thrive in infancy A settled
phase Symptomatic adolescent: Large
VSD, PDA, AVSD
Insidious presentation: AP Window
Eisenmenger Syndrome
Clinical Evaluation
History of symptoms of L R shunt
in infancy
Cyanosis, erythrocytosis, headache
Mildly symptomatic with dyspnoea,
fatigue
History of syncope, hemoptysis,CVA
ES - Underlying CHD
Which one of the following clinical sign
is unlikely in VSD ES (uncomplicated)
1. Single S2
2. Palpable second sound
3. Cardiomegaly
4. Absent parasternal heave

Question 2
Eisenmengers Physiology: Clinical
Assessment
Cyanosis: generally mild
Absence of cardiomegaly, heart failure
Minimal left parasternal lift
Constant ejection click of PAH
Absence of significant shunt murmurs
Pulmonary regurgitation murmur may
be audible
ES: Underlying CHD
Characteristic VSD PDA ASD
Usual age of ES < 2 years < 2 years 20 40 years
Differential
Cyanosis
- Yes (50%) -
Cardiomegaly - - Yes
Second H S (S
2
) Single Narrow/normal Wide & fixed
Parasternal heave - - Yes
TR murmur - - Yes
PR murmur - Yes -
Noninvasive Assessment
Eisenmenger Syndrome
Noninvasive Evaluation
Echocardiography is very useful
Defines the large defect (PDA may be difficult)
Estimates PA pressure by TR/PR jets
Contrast echo demonstrates R L shunting
TEE is safe and may be required in adults for
precise delineation of the abnormality
Eisenmenger Syndrome:
Invasive Evaluation
Cardiac cath can be safely performed
It must be done in borderline cases to assess
operability
Response of pulmonary vasculature to
pulmonary vasodilators like 0
2
, tolazoline
and nitric oxide should be assessed
Limit the use of contrast agent to minimal
Eisenmenger Syndrome:
Natural history
Identify the false statement
1. Prognosis of ES is good
2. Survival better than IPAH
3. With recent advances, pregnancy
better tolerated
4. Heart failure most common cause of
death
Question 4
Eisenmenger Syndrome
Natural History
Life expectancy reduced by about 20 years
Survival Pattern:
At one year 97%
At 5 years 87%
At 10 years 80%
At 15 years 77%
At 25 years 42%
Life expectancy reduced by about 20 years
Unwarranted surgical closure hastens death
Policy of non-intervention, unless absolutely necessary
Avoid destabilizing the balanced physiology
ES Survival better than IPAH
Landzberg, M. J. et al. J Am Coll Cardiol 2006;47:D33-D36
Impact of left ventricular dysfunction on survival in
Eisenmenger syndrome
Dimopoulos, K. et al. Circulation 2010;121:20-25
Cumulative mortality rate curve (with 95% CIs)
Overall population (n=229) According to functional class
Long Term Survival in Eisenmenger physiology
Diller G et al. Eur Heart J 2006;27:1737-1742
Eisenmenger Syndrome
Predictors of Poor outcome
History of syncope
Elevated right heart filling pressure
Severe hypoxemia (Sa0
2
<85%)
Lange RA et al, 1998
Eisenmengers
Syndrome
Is Preventable
Eisenmenger Syndrome
Management Strategies
Drug treatment
Phlebotomy
Transplantation : Heart lung / lung

Counsel against special risks
Pregnancy
Hormone contraceptives
Noncardiac surgery
High altitude/flying
Sudden emotional upset
Conventional Therapy
Digitalis, diuretics
Anti-arrhythmic drugs
Anticoagulants
Long term oxygen therapy
Avoidance of dehydration, high
altitude, infections and IV lines
Avoidance of pregnancy
Targeted Therapy:
Pulmonary Vasodilators
Prostanoids: Epoprostenol infusion
Phosphodiesterase-5 inhibitors:
Sildenafil, tadalafil
Endothelin receptor antagonists:
Bosentan (BREATH-5 trial)
1. Fernandes SM, et al 2003
2. Chou EM, et al 2007
3. Mukhopadhyay S, et al 2006
4. Galie N, et al 2006
5. Gatzoulis MA, et al 2008
Dimopoulos, K. et al. Circulation 2010;121:20-25
Survival in Eisenmenger Syndrome
Patients on Advanced Therapy (n=287)
Bosentan in ES
Bosentan in ES: BREATH 5
Gatzoulis MA, Int J Cardio 2008
Eisenmenger Syndrome: Role of
Phlebotomy
Indication for Isovolumic Phlebotomy
Symptomatic hyper viscosity (PCV >0.65,
Hb>20gm%)

Important issues to remember
Symptoms of hyper viscosity resemble those of
iron deficiency
Phlebotomy may result in iron deficiency anemia
and cerebrovascular accidents
Discourage routine phlebotomy
Management of Eisenmenger
Syndrome
Transplantation
1982 : Combined heart-lung transplantation
introduced by Reitz et al
1990 : Single lung transplantation with repair of
cardiac defect successfully performed by
Fremes et al
Lung transplant has advantages of
better donor availability
Avoidance of cardiac allograft rejection
Absence of coronary vasculopathy

Management of Eisenmenger
Syndrome
Lung Transplantation
Actuarial survival rates : At 1 year 70-80%,
At 4 years <50%, At 10 years <30%
Indications for transplant
History of syncope
Refractory right heart failure
Poor exercise tolerance
Severe hypoxemia
Perioperative Risk for Noncardiac Surgery
High risk conditions
Pulm hypertension
Cyanotic CHD
NYHA class III or IV
Severe ventricular dysfuntion (EF<35%)
Severe left heart obstructive obstruction
Moderate risk conditions
Intracardiac shunt lesions
ACC/AHA guidelines 2008
Associated with a mortality rate of 14% -19%
Local anesthesia is preferred to general
anesthesia
Prolonged fasting and volume depletion should
be avoided
Small air bubbles in IV lines should be removed
Early ambulation is encouraged
Antibodies given to prevent infective
endocarditis
Perioperative Risk for Noncardiac Surgery in
Eisenmenger Syndrome
Risk to Fetus: if Sao
2
< 85%, chances of live fetus only 12%
Caesarian section only for obstetric reasons
Pregnancy and congenital heart disease
Complications During Pregnancy in Women with CHD
Drenthen W, et al. Outcome of pregnancy in women with congenital
heart disease: a literature review. J Am Coll Cardiol 2007;49:2303-11
Drenthen W, et al. Outcome of pregnancy in women with congenital
heart disease: a literature review. J Am Coll Cardiol 2007;49:2303-11
Complications During Pregnancy in Women with CHD
Management of Eisenmenger
Syndrome
Avoidance of Pregnancy
Pregnancy is absolutely contraindicated
Maternal mortality is 36%-45%
Mortality often occurs in post-partum period
Fetal loss occurs in over 60%
Termination is indicated in early gestation
Outcome of pregnant women with Eisenmenger
syndrome has not changed in last three
decades
Eisenmenger Syndrome
Management of Pregnancy
Prolonged bed rest after 20th wks gestation
Oxygen therapy
Digoxin and diuretics if CHF present
Prolonged use of anticoagulants - Heparin
Careful monitoring of volume status, oxygen
saturation and hematocrit is necessary
Eisenmenger Syndrome
Basic Events Leading to Death

Right ventricular failure 30%
Sudden death?vent arrhythmia 25%
Cardiovascular surgery 12%
Cerebrovascular accidents/abscess 10%
Hemoptysis 9-15%
Noncardiac surgery 6%
Pregnancy related 5%
Heart lung/lung transplants 4%
Is generally established by 2-4 yrs of age
Accelerated onset in Downs, Cyanotic CHD
Median survival is 40-45 yrs of age
Anesthesia, surgery, dehydration poorly
tolerated
Pregnancy carries 30-50% maternal mortality
Closure of the defect is detrimental once
obstructive PAH has developed
Eisenmenger Syndrome

Carefully managed, most patients lead useful lives
Eisenmenger Syndrome
Conclusion
Patients with Eisenmenger syndrome can live
upto fifth and sixth decades with informed
medical care, patient education and
protection from special risks
20% of death are related to avoidable errors

Doing nothing may be a positive action for
good in such patients
Jane Somerville, 1998