Eisenmenger syndrome is a form of pulmonary hypertension caused by long-standing left-to-right shunting at the atrial or ventricular level, leading to reversed or bidirectional shunting. It most commonly presents in the second to third decade of life and is associated with uncorrected congenital heart defects. Management focuses on avoiding risks that could destabilize the patient's balanced physiology, such as pregnancy, surgery, travel to high altitudes, and medications like vasodilators. While life expectancy has improved with advanced medical care, mortality remains high due to complications like heart failure, stroke, hemorrhage and sudden death.

1. Dr Dinesh Pandey
MBBS, DNB (MED), Fellow DNB Cardiology
Eisenmenger syndrome

3. 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)

5. Following
3
accounts
for 70-
80% of
cases

6. Eisenmenger Syndrome – A progressive disease. Exact mechanism not clear

7. 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

8. Eisenmenger Syndrome
Presents by: 2nd/3rd decade
Established by: 2-3yrs
Early ES: AVSD, Truncus, d-TGA, Single Ventricle

9. 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

10. Clinical differences between Hyperkinetic and obstructive PAH

11. Echocardiography in Pediatric and Adult Congenital Heart Disease, 2nd Ed

12. Clinical Evaluation
• History of symptoms of L R shunt in infancy
• Erythrocytosis, Headache
• Dyspnoea
• Fatigue : low fixed cardiac output
• Syncope : Arrhythmia/low CO
• Chest Pain : RV ischemia
• Hemoptysis
• CVA : Paradoxic embolism/ICH/Thrombosis
• SCD : Arrhythmia

13. • ES: Clinical Assessment
• Cyanosis : variable
generally mild – moderate
• Clubbing : mild
• BP, Pulse : N
• No or Minimal cardiomegaly
• RV apex
• Minimal left parasternal lift/palpable P2

14. Auscultation
• S1: N
• S2 : Loud P2
• RVS4 +
• RVS3 +/-
• Constant ejection click at PA
• Absence of significant shunt murmurs
• Pulmonary regurgitation murmur may be audible

16. ECG:
• NSR
• RAE ( peaked p)
• RVH ( tall R in V1-V2)
• Sometime qR in V1

24. Echocardiography
• Defines the large defect
• 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

25. Cardiac cath :
• 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

27. Natural History
• Usually healthy childhood, and gradually become progressively cyanotic with
each succeeding decade.
• Complications tend to occur from 3rd decade onward, and congestive heart
failure, the most common, usually occurs after 40 years
• Most survive to adulthood with 77% & 42% survival rate at 15 and 25 years
age.
• Common modes of death are sudden death(30%), CHF(25%),
hemoptysis(15%), pregnancy, following surgery, and infectious causes (brain
abscess, endocarditis) account for most remainder.

28. • Congestive heart failure
• Arrhythmias (atrial
fibrillation/flutter)
• Infectious endocarditis
• Bleeding disorders
• Brain abscess
• Erythrocytosis
• Hyperuricemia/gout
• Paradoxical emboli
• Pulmonary artery calcification &
aneurysm
• Progressive valvular disease
• Renal dysfunction
• Stroke or transient ischemic
attack
• Sudden death
Clinically, patients gradually develop the following
complications of advanced pulmonary vascular disease:

29. Policy of “non-
intervention”,
unless
absolutely
necessary
Avoid
destabilizing
the “balanced
physiology”

32. Eisenmenger Syndrome Predictors of Poor outcome
• History of syncope
• Elevated right heart filling pressure
• Severe hypoxemia (Sa02<85%)

33. 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

34. Conventional Therapy
• Digitalis, diuretics
• Anti-arrhythmic drugs
• Iron supplimentation
• Anticoagulants
• Long term oxygen therapy
• Avoidance of dehydration, high altitude, infections and IV lines
• Avoidance of pregnancy

35. Targeted Therapy: Pulmonary Vasodilators
• Prostanoids: Epoprostenol infusion
• Phosphodiesterase-5 inhibitors: Sildenafil, tadalafil
• Endothelin receptor antagonists: Bosentan (BREATH-5)

39. 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
wt in kg x 80 x [Current Hct- optimal Hct]
Amount of Blood Removed = -------------------------------------------------------
current Hct

40. Management of Eisenmenger Syndrome
Transplantation
1982 : Combined heart-lung transplantationintroduced 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

41. 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

42. Perioperative Risk for Noncardiac Surgery
High risk conditions
• Pulm hypertension
• Cyanotic CHD
• NYHA class III or IV
• Severe ventricular dysfunction (EF<35%)
• Severe left heart obstructive obstruction
Moderate risk conditions
• Intra cardiac shunt lesions

43. • 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
• Antibiotics given to prevent infective endocarditis

46. Eisenmenger Syndrome & Pregnancy
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

47. Eisenmenger 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%

48. Risks for Eisenmenger Syndrome
• Pregnancy (contraindicated)
• General anesthesia
• Dehydration
• Hemorrhage
• Cardiac and noncardiac surgery
• Drugs (vasodilator, diuretics, oral pill, NSAIDs)
• Anemia commonly due to iron deficiency
• Intravenous lines (air embolism,infection)
• Altitute exposure
• Pulmonary infection

49. Conclusion:
• Patients with Eisenmenger Syndrome can live up to 5th and 6th decade of with
informed medical care , Patient education and protection from special risks.
• 20% of death are related to avoidable errors.
• ES is pulmonary hypertensive disease caused by left to right shunting of blood.
• The severity of pulmonary vascular is an important Prognostic factor.

50. • Corrective surgery may cause pulmonary crisis: It should be performed
in selected patient.
• The Principle intervention is non –intervention.
• For quality of life, complications must be managed.
• Pregnancy , non cardiac surgery, travailing: be cautious.
• Transplantation is an effective choice of Treatment.