1. Eisenmenger Syndrome
By Abid H Laghari

2. • Eisenmenger syndrome is pulmonary hypertension with a
reversed central shunt
• An uncorrected large left-to-right shunt causes irreversible
rise in PVR leading to reversal of or bidirectional shunt flow
with resultant hypoxemia
• Eisenmenger syndrome is not a congenital defect, but a
pathophysiologic condition

3. NEJM 2000; 342(5); 334-342

4. • Around 12 different congenital intracardiac or extracardiac
defects can cause Eisenmenger syndrome:
• Following 3 account for 70–80% of cases
• VSD
• Atrioventricular septal defect
• PDA

5. • Other congenital heart diseases which can cause
Eisenmenger syndrome:
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–
–
–
–
–
ASD
Truncus arteriosus
Aortopulmonary window
Univentricular heart without PS
D-transposition of the great vessels with VSD
Surgically created aorto-pulmonary connections
Braunwald E. Heart Disease

6. • With large shunts, the PVR develops relatively quickly, usually
within first two years of life
• In patient with ASD may have Eisenmenger syndrome in
adulthood

7. Presentation and course in childhood
• Children may be asymptomatic or have only mild dyspnea
• Reduced exercise capacity, dyspnea and fatigue develop
gradually as pulmonary blood flow decreases, and hypoxemia
increases due to bidirectional shunting

8. Course in adulthood
• Many individuals with Eisenmenger syndrome survive into
adulthood with 80% survival at 10 years, 77% survival at 15
years and 42% at 25 years after diagnosis
• Variables associated with poor prognosis include :
- Syncope
- Elevated RA pressure
- Severe resting hypoxemia (<80% transcutaneous oxygen
saturation)

9. The causes of death in Eisenmenger pts:
• Sudden death (30%)
• Congestive heart failure (25%)
• Hemoptysis (15%)

10. The causes of death contd.
Other (30%) including:
– Pregnancy
– Perioperative following non-cardiac surgery
– Infective endocarditis
– Brain abscess
– Non-cardiac causes

11. • While individuals with Eisenmenger syndrome may remain
relatively stable for long periods of time, it is essential to
appreciate that their hemodynamic state is very delicately
balanced
• This balance is easily upset, often with disastrous results

12. Examination in Eisenmenger Syndrome
• Central cyanosis with digital clubbing
• May have differential cyanosis and clubbing
• Hypoxemia with resting oxygen saturation <90%
• Lungs are usually clear

13. • RV heave, palpable P2, right sided S4, and occasionally
pulmonary ejection click
• Murmurs likely to be heard include a high-pitched diastolic
decrescendo murmur of pulmonic insufficiency and a
holosystolic murmur of TR
• Murmurs related to the defects connecting the systemic and
pulmonary circulations are not usually heard

14. Diagnostic Testing
• Goals
– For the diagnosis of heart defect
– For evaluating the severity
– For stratification, predictable prognostic factors? For surgery?
• Choices
– Electrocardiography
• RAE, RVH, right axis deviation, arrhythmia
– Chest X ray
• Cardiomegaly, dilated pulmonary arteries, pulmonary artery calcification
– Echocardiography: TEE is preferred
• Heart defect, direction of shunting, pulmonary hypertension
– Cardiac catheterization
– Open lung biopsy

23. • It is important to be certain that the diagnosis of Eisenmenger
syndrome is correct
• One does not want to miss the opportunity to identify
individuals who have reversibility of their pulmonary vascular
disease that may enable a surgical repair of the defect
• The cardiac catheterization is performed to establish that the
PVR is elevated and responsiveness to administration of
oxygen, nitric oxide, suldinafil , Ca Channel Blockers

24. Catheter and surgical management
• Once Eisenmenger physiology has developed, catheter or
surgical interventions have a limited role in management
• Surgery to repair the underlying congenital anomaly is not
recommended for two reasons:
1 - The risk of surgery is exceedingly high
2 – Those who survive the surgery have increased mortality

25. • Heart–lung transplantation is an option, but long waiting is a
problem
• In some instances , lung transplantation with repair of the
intracardiac defect may be an option
• Lung transplantation has the advantage of better donor
availability, a shorter waiting period, and avoidance of
problems associated with heart transplantation (vasculopathy
and rejection)

26. The following may lead one to consider surgical or
transcatheter options:
• Progressive deterioration of functional class
• Recurrent syncope
• Refractory right heart failure
• Supraventricular tachyarrhythmias
• Worsening hypoxemia

27. Expected abnormalities
• A number of abnormal findings are expected in Eisenmenger
syndrome pts and should not raise undue concern unless they
represent a significant change from past values
• Oxygen saturation at rest usually ranges in 80s
• If checked shortly after exertion , it will be lower (mid 70%
range)
• The baseline value should be established after a few minutes
of rest

28. • Hct , PLt
• INR and APTT are mildly prolonged
• Uric acid and bilirubin are elevated
• Proteinuria , usually less than 1 G/24 hours (this is
glomerular in origin and related to the hypoxemia)
• Mildly elevated serum Cr and hematuria can also be found

29. Recommendations for Medical Therapy of
Eisenmenger Physiology
Class I
1.
It is recommended that patients with Eisenmenger syndrome avoid the
following activities or exposures, which carry increased risks:
a. Pregnancy. (Level of Evidence: B)
b. Dehydration. (Level of Evidence: C)
c. Moderate and severe strenuous exercise, particularly isometric exercise (Level of
Evidence: C)
d. Acute exposure to excessive heat (eg, hot tub or sauna). (Level of Evidence: C)
e. Chronic high-altitude exposure (particularly at an elevation greater than 5000 feet
above sea level). (Level of Evidence: C)
f. Iron deficiency. (Level of Evidence: B)

30. Recommendations for Medical Therapy of
Eisenmenger Physiology cont:
2. Patients with Eisenmenger syndrome should seek prompt
therapy for arrhythmias and infections. (Level of Evidence: C)
3. Should have hemoglobin, platelet count, iron stores,
creatinine, and uric acid assessed at least yearly. (Level of
Evidence: C)
4. Should have assessment of digital oximetry, both with and
without supplemental oxygen therapy, at least yearly. The
presence of oxygen-responsive hypoxemia should be
investigated further. (Level of Evidence: C)

31. Recommendations for Medical Therapy of
Eisenmenger Physiology cont:
5. Exclusion of air bubbles in intravenous tubing is
recommended as essential during treatment of adults with
Eisenmenger syndrome. (Level of Evidence: C)
6. These pts should undergo noncardiac surgery and cardiac
catheterization only in centers with expertise in the care of
such patients (Level of Evidence: C)

32. Medical Therapy of
Eisenmenger Physiology cont:
Hypoxemia:
• While it seems obvious that inhaled O2 would help, no studies
show a mortality or morbidity benefit from chronic O2
administration
• Inhaled O2 can be used if the patient feels comfortable with it
(reduced dyspnea, reduced fatigue, improved sleep)
• However, the adverse effects of mucosal dryness leading to
mucous bleeding and the cumbersome equipment cause most
patients to chose not to chronically use O2

33. Hyperviscosity syndrome:
• Viscosity is affected by the concentration of RBCs and their
deformability
• A high Hct alone may not cause these symptoms
• The major etiology for reduced deformity is thought to be iron
deficiency which causes RBCs to change from deformable
biconcave disks to more rigid microspheres
• Blood loss related to phlebotomy, hemoptysis, epistaxis and
menses are common causes of iron deficiency

34. Important considerations in individuals with symptoms
suggestive of hyperviscosity syndrome
• High Hct in the absence of symptoms does not require
phlebotomy
• Exclude dehydration as a cause of Hct
• Exclude iron deficiency , If present, treat with oral iron
• Phlebotomy may be appropriate if symptoms are severe and
none of the above factors apply

35. Phlebotomy
• The goal of phlebotomy is to treat the symptoms of the
hyperviscosity syndrome and not to obtain a specific Hct
• Prompt relief of symptoms after the phlebotomy confirms
that hyperviscosity was the likely etiology
• If the symptoms do not resolve promptly, consider other
alternative causes and do not repeat the phlebotomy

36. Medical Therapy of
Eisenmenger Physiology cont:
Bleeding:
• These pts are at risk of bleeding from the relatively benign
easy bruising to life-threatening massive intra-pulmonary
hemorrhage and hemoptysis
• Most bleeding is, however minor, involves the
mucocutaneous tissues, and responds to conservative
management

37. • Significant bleeding can be treated with vitamin K, FFPs,
platelets or cryoprecipitate
• Phlebotomy may improve platelet function, increase platelet
count and improve various coagulation abnormalities
• Phlebotomy can be considered prior to elective surgery to
decrease the risk of bleeding

38. Cerebrovascular and other embolic events:
• Mechanisms include hemorrhage, emboli and infection with
formation of a cerebral abscess
• Iron deficiency is the major risk factor for cerebrovascular
events
• The risk–benefit ratio of aspirin or warfarin needs to be
considered in each patient

40. Gout
• Rare
• Pathophysiology ??
– Increase resorption of uric acid
– Increase production of uric acid and impaired excretion
• Treatment
– Colchicine
– Avoid NSAIDs

41. Pulmonary hypertension:
• Pulmonary vasodilator agents such as prostacyclin analogs,
endothelin antagonists and phosphodiesterase inhibitors have
been found to reduce PVR and improve functional capacity
• Limited data cite some individuals so responsive to these
agents that surgical correction of the defect was possible
• Alternatively, in patients with progressive heart failure, these
agents have been used as part of a bridge to transplantation

42. Recommendations for Follow-Up
Class I
1. Patients with CHD-related PAH should:
a. Have coordinated care under the supervision of a trained CHD and
PAH care provider and be seen by such individuals at least yearly
(Level of Evidence: C)
b. Have yearly comprehensive evaluation of functional capacity and
assessment of secondary complications (Level of Evidence: C)
c. Discuss all medication changes or planned interventions with their
CHD-related PAH caregiver(Level of Evidence: C)

43. Recommendations for Reproduction
Class I
1. Women with severe CHD-PAH, especially those with
Eisenmenger physiology, and their partners should be
counseled about the absolute avoidance of pregnancy in
view of the high risk of maternal death, and they should be
educated regarding safe and appropriate methods of
contraception. (Level of Evidence: B)

44. 2. Women with CHD-PAH who become pregnant should:
a. Receive individualized counseling from cardiovascular and
obstetric caregivers collaborating in care and with expertise
in management of CHD-PAH. (Level of Evidence: C)
b. Undergo the earliest possible pregnancy termination after
such counseling. (Level of Evidence: C)
3. Surgical sterilization carries some operative risk for women
with CHD-PAH but is a safer option than pregnancy (Level of
Evidence: C)

45. Class IIb
1. Pregnancy termination in the last 2 trimesters of pregnancy
poses a high risk to the mother
- It may be reasonable, however, after the risks of
termination are balanced against the risks of continuation
of the pregnancy (Level of Evidence: C)

46. During pregnancy deaths are commonly due to:
•
•
•
•
•
Thromboembolism (44%)
Hypovolemia (25%)
Pre-eclampsia (18%)
Worsening heart failure
Progressive hypoxemia

47. Non-cardiac surgery in Eisenmenger patients
• Non-cardiac surgery in Eisenmenger patients carries a high
morbidity and mortality risk (up to 19%)
• Surgery should be avoided when possible, but is commonly
needed for acute cholecystitis (due to bilirubin stone
formation from the hyperbilirubinemia)
• Necessary operations should be done in a center familiar with
the high risks of performing surgery on these patients

48. Perioperative morbidity and mortality
The mortality and morbidity are related to:
• Sudden fall in SVR leading to worsening hypoxemia due to
progressive right to left shunting
• Hypovolemia and dehydration
• Excessive bleeding
• Perioperative arrhythmias
• Thrombophlebitis/DVT/paradoxical emboli

49. Take Home Messages
• Eisenmenger syndrome is a pulmonary hypertensive disease
caused by left-to-right shunting of blood
• The severity of pulmonary vascular resistance is a important
prognostic factor
• Corrective surgery may cause pulmonary crisis. It should be
performed in selected patients
• The principle of intervention is non-intervention
• For quality of life, complications must be managed
• Pregnancy, noncardiac surgery, travelling: be cautious
• Transplantation is an effective choice of treatment

50. THANKS FOR YOUR ATTENTION