What is the management approach for Eisenmenger syndrome?

Management of Eisenmenger Syndrome

Patients with Eisenmenger syndrome should be managed exclusively in specialized centers with expertise in adult congenital heart disease and pulmonary arterial hypertension to reduce morbidity and mortality. 1

Diagnostic Evaluation

A comprehensive evaluation of patients with Eisenmenger syndrome should include:

• Detailed anatomical assessment of the underlying congenital defect
• Documentation of shunt direction and severity of pulmonary arterial hypertension
• Assessment of:
  • Oxygen saturation (finger and toe oximetry)
  • Complete blood count with indices
  • Iron studies (ferritin)
  • Renal and hepatic function tests
  • 6-minute walk test
  • Pulmonary function tests
  • ECG and chest X-ray
  • Echocardiography for ventricular function assessment

Cardiac catheterization should be performed only at specialized centers with expertise in adult congenital heart disease and pulmonary arterial hypertension 1.

Pharmacological Management

Pulmonary Vasodilator Therapy

• The endothelin receptor antagonist (ERA) bosentan is indicated as first-line therapy for WHO functional class III patients with Eisenmenger syndrome 1
• Other ERAs, phosphodiesterase type-5 inhibitors, and prostanoids should be considered for symptomatic patients 1
• Combination therapy may be considered in patients not responding adequately to monotherapy 1
• Calcium channel blockers are contraindicated in Eisenmenger syndrome 1

Anticoagulation

• Oral anticoagulation should be considered in patients with:
  • Pulmonary artery thrombosis
  • Signs of heart failure
  • Atrial fibrillation
  • Low bleeding risk 1

Oxygen Therapy

• Supplemental oxygen should be considered when it produces a consistent increase in arterial oxygen saturation and reduces symptoms 1
• Oxygen-responsive hypoxemia should be further investigated 1

Management of Erythrocytosis

• Therapeutic phlebotomy should only be performed when:
  • Hemoglobin > 20 g/dL
  • Hematocrit > 65%
  • Patient has symptoms of hyperviscosity
  • No evidence of dehydration 1
• Iron deficiency should be avoided and treated when present 1

Critical Precautions

1. Avoid air bubbles in intravenous lines - essential to prevent paradoxical embolism 1

2. Medication review - all medications must be rigorously evaluated for potential effects on:

   • Systemic blood pressure
   • Loading conditions
   • Intravascular shunting
   • Renal or hepatic function 1

3. Avoid destabilizing situations:

   • Volume shifts
   • Extreme fatigue
   • High-altitude exposure
   • Cigarette smoke exposure
   • Dehydration 1, 2

4. Pregnancy is absolutely contraindicated due to extremely high maternal and fetal mortality 1

5. Non-cardiac surgery and procedures should only be performed in specialized centers with expertise in Eisenmenger syndrome 1

Multisystem Approach

Eisenmenger syndrome affects multiple organ systems and requires vigilant monitoring for complications:

• Cardiac: Monitor for arrhythmias, heart failure progression
• Hematologic: Monitor for bleeding complications, thrombosis
• Neurologic: Assess for stroke risk, brain abscess
• Respiratory: Evaluate for hemoptysis, pulmonary infections
• Renal: Regular assessment of renal function

Follow-up

Patients should be seen at specialized centers at least every 6-12 months with assessment of:

• Exercise capacity
• Oxygen saturation
• Blood tests (CBC, iron studies, renal/liver function)
• Echocardiography to assess ventricular function

Common Pitfalls to Avoid

1. Never attempt surgical repair of the defect once Eisenmenger physiology is established as this can be fatal

2. Avoid routine phlebotomy which can lead to iron deficiency and paradoxically increase risk of thrombotic events

3. Avoid medications that can destabilize the delicate hemodynamic balance, particularly vasodilators that may cause systemic hypotension

4. Never use calcium channel blockers as they can cause catastrophic systemic vasodilation and decreased cardiac output 1

5. Avoid dehydration which can increase blood viscosity and risk of thrombosis

The management of Eisenmenger syndrome requires a careful balance between treating symptoms while not disrupting the patient's fragile physiological equilibrium 2, 3.