Eisenmenger Syndrome Anita Saxena Department of Cardiology, All India Institute of Medical Sciences...

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


1958: Paul Wood’s

Croonian Lectures

coined the term

“Eisenmenger

Syndrome”

1958: Paul Wood’s

Croonian Lectures

coined the term

“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

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


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


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


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


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


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


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



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


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


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


Carefully managed, most patients lead useful livesCarefully managed, most patients lead useful lives


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

Eisenmenger Syndrome Anita Saxena Department of Cardiology, All India Institute of Medical Sciences...

Documents

Transcript of Eisenmenger Syndrome Anita Saxena Department of Cardiology, All India Institute of Medical Sciences...