Eisenmenger Syndrome

Report
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 Wood’s
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
(n=132)
Ventricular Septal Defect
Atrial Septal Defect
Patent ductus arteriosus
Atrio ventricular septal defect
Truncus arteriosus
Single ventricle
Transposition of great arteries
Others
45
6
12
16
15
13
5
20
Daliento et al ‘98
(n=188)
71
21
36
23
11
9
8
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
Pulmonary Arterial Hypertension
Hyperkinetic
Obstructive
(Eisenmenger’s)
Heart Size
Large
Normal
Parasternal
impulse
Hyperkinetic
Forcible
Click
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
1
At what age a large VSD Eisenmengerize?
1. < 6 months
2. 2 years
3. 10 years
4. 20 years
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
Question 2
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
Eisenmenger’s 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 (S2)
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 02, tolazoline
and nitric oxide should be assessed
Limit the use of contrast agent to minimal
Eisenmenger Syndrome:
Natural history
Question 4
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
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
Impact of left ventricular dysfunction on survival in
Eisenmenger syndrome
Landzberg, M. J. et al. J Am Coll Cardiol 2006;47:D33-D36
Cumulative mortality rate curve (with 95% CIs)
Overall population (n=229)
According to functional class
Dimopoulos, K. et al. Circulation 2010;121:20-25
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
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
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.
2.
3.
4.
5.
Fernandes SM, et al 2003
Chou EM, et al 2007
Mukhopadhyay S, et al 2006
Galie N, et al 2006
Gatzoulis MA, et al 2008
Survival in Eisenmenger Syndrome
Patients on Advanced Therapy (n=287)
Dimopoulos, K. et al. Circulation 2010;121:20-25
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
Perioperative Risk for Noncardiac Surgery in
Eisenmenger Syndrome
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
Pregnancy and congenital heart disease
Risk to Fetus: if Sao2 < 85%, chances of live fetus only 12%
Caesarian section only for obstetric reasons
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
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
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
Sudden death?vent arrhythmia
Cardiovascular surgery
Cerebrovascular accidents/abscess
Hemoptysis
Noncardiac surgery
Pregnancy related
Heart lung/lung transplants
30%
25%
12%
10%
9-15%
6%
5%
4%
Eisenmenger Syndrome
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 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

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