Management of Pulmonary Hypertension Secondary to Eisenmenger Syndrome
The endothelin receptor antagonist bosentan is the first-line treatment for patients with Eisenmenger syndrome who are in WHO functional class III, as it has demonstrated improvements in exercise capacity, hemodynamics, and functional ability. 1
Diagnosis and Evaluation
- Eisenmenger syndrome results from congenital heart disease with systemic-to-pulmonary shunts that lead to pulmonary vascular disease, causing reversal of shunt direction and central cyanosis 1
- Clinical manifestations include dyspnea, fatigue, syncope, cyanosis, and secondary erythrocytosis 1
- Patients may also experience hemoptysis, cerebrovascular accidents, brain abscesses, coagulation abnormalities, and are at risk for sudden death 1
- Comprehensive evaluation should include:
- Oxygen saturation measurements (finger and toe oximetry) 1
- Anatomic definition of cardiac defects (using appropriate imaging) 1
- Assessment of pulmonary arterial pressure and resistance 1
- Complete blood count with indices to evaluate erythrocytosis 1
- Renal and hepatic function tests 1
- 6-minute walk test to assess exercise capacity 1
Treatment Approach
Specialized Care
- Patients with Eisenmenger syndrome should be managed in specialized centers with expertise in adult congenital heart disease and pulmonary hypertension 1
- Patient education about potential medical risk factors and behavioral modifications is essential 1
Pharmacological Management
First-line Therapy:
- Bosentan (endothelin receptor antagonist) is indicated for WHO functional class III patients with Eisenmenger syndrome (Class I recommendation, Level B evidence) 1
Alternative/Additional Options:
- Other endothelin receptor antagonists, phosphodiesterase type-5 inhibitors (such as sildenafil), and prostanoids should be considered (Class IIa recommendation, Level C evidence) 1
- Sildenafil has shown improvements in exercise capacity, systemic arterial oxygen saturation, and hemodynamic parameters in Eisenmenger patients 2
- Combination therapy may be considered in patients with inadequate clinical response to monotherapy (Class IIb recommendation, Level C evidence) 1
- Calcium channel blockers are NOT recommended in patients with Eisenmenger syndrome (Class III recommendation, Level C evidence) 1
Supportive Measures
- Supplemental oxygen therapy should be considered if it produces a consistent increase in arterial oxygen saturation and reduces symptoms (Class IIa recommendation, Level C evidence) 1
- In the absence of significant hemoptysis, oral anticoagulant treatment should be considered in patients with pulmonary artery thrombosis or signs of heart failure (Class IIa recommendation, Level C evidence) 1
- Phlebotomy with isovolumic replacement should be considered when symptoms of hyperviscosity are present, usually when hematocrit exceeds 65% (Class IIa recommendation, Level C evidence) 1
- All medications given to Eisenmenger patients should be carefully reviewed for potential effects on systemic blood pressure, loading conditions, and intravascular shunting 1
Special Considerations
Avoid circumstances that can lead to clinical deterioration:
Ensure exclusion of air bubbles in intravenous tubing during treatment 1
Non-cardiac surgery and cardiac catheterization should only be performed in centers with expertise in managing Eisenmenger syndrome 1
Advanced Options
- For patients with deteriorating clinical status despite medical therapy, lung transplantation with repair of cardiac defects or combined heart-lung transplantation may be considered 3, 4
- However, Eisenmenger patients have better survival compared to idiopathic pulmonary arterial hypertension, with a 3-year survival rate of 77% compared to 35% for untreated idiopathic PAH 1