a saxena eisenmenger jan 2012

8/11/2019 A Saxena Eisenmenger Jan 2012

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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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1887: Victor Eisenmengerdescribed history and

postmortem details of 32year old man with VSD andpathological features ofPAH


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1958: Paul Woods

Croonian Lecturescoined the term

Eisenmenger

Syndrome


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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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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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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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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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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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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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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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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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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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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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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


Carefully managed, most patients lead useful lives


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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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a saxena eisenmenger jan 2012

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