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Eisenmenger Syndrome
Anita Saxena
Department of Cardiology,All India Institute of Medical
SciencesNew Delhi, India 110029
Anita Saxena
Department of Cardiology,All India Institute of Medical
SciencesNew Delhi, India 110029
Eisenmenger SyndromeEisenmenger Syndrome
1887 : Victor Eisenmenger described history and postmortem details of 32 year old man with VSD and pathological features of PAH
1887 : Victor Eisenmenger described history and postmortem details of 32 year old man with VSD and pathological features of PAH
Eisenmenger SyndromeEisenmenger Syndrome
1958: Paul Wood’s
Croonian Lectures
coined the term
“Eisenmenger
Syndrome”
1958: Paul Wood’s
Croonian Lectures
coined the term
“Eisenmenger
Syndrome”
Eisenmenger SyndromeEisenmenger 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
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 SyndromeUnderlying 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 B2
Exact mechanism not clear
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
Pulmonary Arterial Hypertension
Hyperkinetic Obstructive (Eisenmenger’s)
Heart Size Large Normal
Parasternal impulse Hyperkinetic
ForcibleClick Absent Present
S2 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
At what age a large VSD
Eisenmengerize?
1. < 6 months
2. 2 years
3. 10 years
4. 20 years
1
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
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 EvaluationHistory of symptoms of L R shunt in infancyCyanosis, erythrocytosis, headacheMildly symptomatic with dyspnoea, fatigueHistory of syncope, hemoptysis,CVA
Clinical EvaluationHistory of symptoms of L R shunt in infancyCyanosis, erythrocytosis, headacheMildly symptomatic with dyspnoea, fatigueHistory of syncope, hemoptysis,CVA
ES - Underlying CHDES - 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
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
Eisenmenger’s Physiology: Clinical Assessment
Cyanosis: generally mildAbsence of cardiomegaly, heart failureMinimal left parasternal liftConstant ejection click of PAHAbsence of significant shunt murmursPulmonary regurgitation murmur may be audible
Cyanosis: generally mildAbsence of cardiomegaly, heart failureMinimal left parasternal liftConstant ejection click of PAHAbsence of significant shunt murmursPulmonary regurgitation murmur may be audible
ES: Underlying CHDES: 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 -
Noninvasive Assessment
Eisenmenger SyndromeNoninvasive Evaluation
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
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
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
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
Eisenmenger Syndrome:
Natural history
Eisenmenger Syndrome:
Natural history
Identify the false statement1. Prognosis of ES is good2. Survival better than IPAH3. With recent advances,
pregnancy better tolerated4. Heart failure most common
cause of death
Identify the false statement1. Prognosis of ES is good2. Survival better than IPAH3. With recent advances,
pregnancy better tolerated4. Heart failure most common
cause of death
Question 4
Eisenmenger Syndrome
Natural HistoryLife 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%
Natural HistoryLife 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
(Sa02<85%)
Lange RA et al, 1998
Predictors of Poor outcome
History of syncope
Elevated right heart filling
pressure
Severe hypoxemia
(Sa02<85%)
Lange RA et al, 1998
Eisenmenger’s 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
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 TherapyConventional Therapy
Digitalis, diureticsAnti-arrhythmic drugsAnticoagulantsLong term oxygen therapyAvoidance of dehydration, high altitude, infections and IV linesAvoidance of pregnancy
Digitalis, diureticsAnti-arrhythmic drugsAnticoagulantsLong term oxygen therapyAvoidance of dehydration, high altitude, infections and IV linesAvoidance of pregnancy
Targeted Therapy:Pulmonary Vasodilators
Targeted Therapy:Pulmonary Vasodilators
Prostanoids: Epoprostenol infusion
Phosphodiesterase-5 inhibitors: Sildenafil, tadalafil
Endothelin receptor antagonists: Bosentan (BREATH-5 trial)
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
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
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
Transplantation1982 : Combined heart-lung
transplantation introduced by Reitz et al1990 : Single lung transplantation with
repair of cardiac defect successfully
performed by Fremes et alLung transplant has advantages of
better donor availabilityAvoidance of cardiac allograft rejectionAbsence of coronary vasculopathy
Transplantation1982 : Combined heart-lung
transplantation introduced by Reitz et al1990 : Single lung transplantation with
repair of cardiac defect successfully
performed by Fremes et alLung transplant has advantages of
better donor availabilityAvoidance of cardiac allograft rejectionAbsence of coronary vasculopathy
Management of Eisenmenger Syndrome
Lung TransplantationActuarial 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
Lung TransplantationActuarial 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
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
• 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 lesionsACC/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
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 SyndromePerioperative Risk for Noncardiac Surgery in Eisenmenger Syndrome
Risk to Fetus: if Sao2 < 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
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
gestationOutcome of pregnant women with
Eisenmenger syndrome has not changed in last three decades
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 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
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 SyndromeEisenmenger 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%
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 Down’s, 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
Is generally established by 2-4 yrs of age
Accelerated onset in Down’s, 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 livesCarefully managed, most patients lead useful lives
Eisenmenger SyndromeEisenmenger Syndrome
ConclusionPatients 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
ConclusionPatients 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