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Eisenmenger Syndrome
Anita Saxena
Department of Cardiology,
All India Institute of Medical Sciences
New Delhi, India 110029
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Eisenmenger Syndrome
1887: Victor Eisenmengerdescribed history and
postmortem details of 32year old man with VSD andpathological features ofPAH
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Eisenmenger Syndrome
1958: Paul Woods
Croonian Lecturescoined the term
Eisenmenger
Syndrome
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Eisenmenger Syndrome
Definition:
Pulmonary hypertension at or nearsystemic level with reversed orbidirectional shunt between thepulmonary and systemic circulation
and pulmonary vascular resistanceabove 800dyn/cm-5 (10 Wood Units)
Paul Wood, Br Med J, 1958
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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
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Eisenmenger Syndrome A
progressive disease
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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 B2
Exact mechanism not clear
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Pulmonary Arterial Hypertension
Hyperkinetic Obstructive(Eisenmengers)
Heart Size Large Normal
Parasternal
impulse Hyperkinetic Forcible
Click Absent Present
S2 ASD wide & fixed wide & fixed
VSD wide & variable SinglePDA paradoxic split normal split
Shunt murmur present short/absent
Flow murmur Present Absent
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Question 1
At what age a large VSD Eisenmengerize?
1. < 6 months
2. 2 years
3. 10 years
4. 20 years
1
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Eisenmenger SyndromeClinical 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
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Eisenmenger Syndrome
Clinical Evaluation
History of symptoms of L R shunt
in infancyCyanosis, erythrocytosis, headache
Mildly symptomatic with dyspnoea,
fatigue
History of syncope, hemoptysis,CVA
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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
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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 murmursPulmonary regurgitation murmur may
be audible
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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 (S2) Single Narrow/normal Wide & fixed
Parasternal heave - - Yes
TR murmur - - Yes
PR murmur - Yes -
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Noninvasive Assessment
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Eisenmenger SyndromeNoninvasive 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
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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 02, tolazoline
and nitric oxide should be assessed
Limit the use of contrast agent to minimal
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Eisenmenger Syndrome:Natural history
Identify the false statement
1. Prognosis of ES is good2. Survival better than IPAH
3. With recent advances, pregnancy
better tolerated4. Heart failure most common cause of
death
Question 4
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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%
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Life expectancy reduced by about 20 years
Unwarranted surgical closure hastens death
Policy of non-intervention, unless absolutely necessary
Avoid destabilizing the balanced physiology
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ES Survival better than IPAH
I t f l ft t i l d f ti i l i
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Landzberg, M. J. et al. J Am Coll Cardiol 2006;47:D33-D36
Impact of left ventricular dysfunction on survival inEisenmenger syndrome
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Dimopoulos, K. et al. Circulation 2010;121:20-25
Cumulative mortality rate curve (with 95% CIs)
Overall population (n=229) According to functional class
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Long Term Survival in Eisenmenger physiology
Diller G et al. Eur Heart J 2006;27:1737-1742
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Eisenmenger Syndrome
Predictors of Poor outcome
History of syncope
Elevated right heart filling pressure
Severe hypoxemia (Sa02
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EisenmengersSyndrome
Is Preventable
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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
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Conventional Therapy
Digitalis, diuretics
Anti-arrhythmic drugs
Anticoagulants
Long term oxygen therapy
Avoidance of dehydration, highaltitude, infections and IV lines
Avoidance of pregnancy
T d Th
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Targeted Therapy:Pulmonary Vasodilators
Prostanoids: Epoprostenol infusion
Phosphodiesterase-5 inhibitors:
Sildenafil, tadalafil
Endothelin receptor antagonists:
Bosentan (BREATH-5 trial)
1. Fernandes SM, et al 20032. Chou EM, et al 20073. Mukhopadhyay S, et al 20064. Galie N, et al 20065. Gatzoulis MA, et al 2008
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Dimopoulos, K. et al. Circulation 2010;121:20-25
Survival in Eisenmenger SyndromePatients on Advanced Therapy (n=287)
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Bosentan in ES
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Bosentan in ES: BREATH 5
Gatzoulis MA, Int J Cardio 2008
Ei S d R l f
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Eisenmenger Syndrome: Role ofPhlebotomy
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 anemiaand cerebrovascular accidents
Discourage routine phlebotomy
M t f Ei
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Management of EisenmengerSyndrome
Transplantation
1982 : Combined heart-lung transplantation
introduced by Reitz et al
1990 : Single lung transplantation with repair ofcardiac defect successfully performed by
Fremes et al
Lung transplant has advantages ofbetter donor availability
Avoidance of cardiac allograft rejection
Absence of coronary vasculopathy
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Management of EisenmengerSyndrome
Lung Transplantation
Actuarial survival rates : At 1 year 70-80%,
At 4 years
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Perioperative Risk for Noncardiac Surgery
High risk conditionsPulm hypertension
Cyanotic CHD
NYHA class III or IVSevere ventricular dysfuntion (EF
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Associated with a mortality rate of 14% -19%
Local anesthesia is preferred to general
anesthesia
Prolonged fasting and volume depletion shouldbe avoided
Small air bubbles in IV lines should be removed
Early ambulation is encouragedAntibodies given to prevent infective
endocarditis
Perioperative Risk for Noncardiac Surgery inEisenmenger Syndrome
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Risk to Fetus: if Sao2< 85%, chances of live fetus only 12%
Caesarian section only for obstetric reasons
Pregnancy and congenital heart disease
C li i D i P i W i h CHD
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Complications During Pregnancy in Women with CHD
Drenthen W, et al . Outcom e of pregnancy in women w ith co ngeni ta l
heart d isease: a literatu re review. J Am Coll Card iol 2007;49:2303-11
C li i D i P i W i h CHD
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Drenthen W, et al . Outcom e of pregnancy in wom en with c ong eni tal
heart d isease: a literatur e review. J Am Co ll Cardio l 2007;49:2303-11
Complications During Pregnancy in Women with CHD
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Management of EisenmengerSyndrome
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 gestationOutcome of pregnant women with Eisenmenger
syndrome has not changed in last threedecades
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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
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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%
Eisenmenger S ndrome
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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 poorlytolerated
Pregnancy carries 30-50% maternal mortality
Closure of the defect is detrimental onceobstructive PAH has developed
Eisenmenger Syndrome
Carefully managed, most patients lead useful lives
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Eisenmenger Syndrome
Conclusion
Patients with Eisenmenger syndrome can liveupto fifth and sixth decades with informed
medical care, patient education andprotection from special risks
20% of death are related to avoidable errors
Doing nothing may be a positive action forgood in such patients
Jane Somerville, 1998
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