What is the management of Eisenmenger syndrome?

From the Guidelines

The management of Eisenmenger syndrome focuses on improving quality of life and preventing complications rather than reversing the condition.

Key Considerations

• Treatment includes pulmonary vasodilator therapy with medications such as bosentan (starting at 62.5 mg twice daily for 4 weeks, then increasing to 125 mg twice daily), sildenafil (20 mg three times daily), or tadalafil (40 mg once daily) 1.
• Supplemental oxygen therapy should be used for patients with oxygen saturations below 90% 1.
• Iron deficiency should be treated with oral or intravenous iron supplementation.
• Diuretics may help manage right heart failure symptoms.
• Phlebotomy should only be performed for symptomatic hyperviscosity with hematocrit >65%, not as routine therapy.
• Anticoagulation is controversial and should be individualized 1.
• Pregnancy must be avoided due to high maternal mortality risk.
• Patients should avoid dehydration, high altitude, excessive physical exertion, and vasodilators that can cause systemic hypotension.
• Regular follow-up with a pulmonary hypertension specialist is essential 1.

Pathophysiology and Diagnosis

Eisenmenger syndrome is characterized by elevated pulmonary vascular resistance driving right-to-left intracardiac or great arterial shunting leading to systemic arterial desaturation 1. The risk of development of Eisenmenger syndrome is influenced by concomitant congenital syndromes, anatomic location of congenital defects, size of anatomic defects, genetic factors, and environmental exposures 1. Accurate diagnosis is necessary to guide therapy, and advanced imaging and cardiac catheterization are crucial prerequisites to optimize therapy for adults with Eisenmenger syndrome 1.

Treatment Goals

These approaches aim to reduce pulmonary vascular resistance, improve right ventricular function, prevent thromboembolism, and maintain adequate oxygen delivery to tissues in this complex condition where pulmonary hypertension has led to right-to-left shunting 1.

Recent Guidelines

The 2018 AHA/ACC guideline for the management of adults with congenital heart disease recommends bosentan as beneficial in symptomatic adults with Eisenmenger syndrome with ASD or VSD, and combination therapy with bosentan and PDE-5 inhibitors is reasonable in symptomatic adults with Eisenmenger syndrome if symptomatic improvement does not occur with either medication alone 1.

From the Research

Definition and Overview of Eisenmenger Syndrome

• Eisenmenger syndrome is a condition characterized by severe pulmonary vascular disease resulting from a congenital heart defect, leading to reversed or bidirectional shunting and chronic cyanosis 2.
• It is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease 3.
• The syndrome is marked by a large nonrestrictive systemic left-to-right shunt that triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, ultimately resulting in shunt reversal 3.

Management of Eisenmenger Syndrome

• Management strategies include the use of pulmonary arterial hypertension therapies to improve quality of life, exercise tolerance, and outcomes, although the optimal timing for their introduction and escalation remains uncertain 2.
• Advanced pulmonary arterial hypertension therapies are indicated in patients with Eisenmenger syndrome and World Health Organization functional class II or higher symptoms to improve functional capacity, quality of life, and survival 3.
• Multidisciplinary care is essential, and standardized practical guidelines for assessment, management, risk stratification, and follow-up are necessary for this fragile and vulnerable population 3.
• Specific recommendations regarding monotherapy or combination therapy are provided according to functional class and clinical response 3.
• Referral of Eisenmenger patients to specialized centers with interdisciplinary management strategies is required to optimize quality of life and outcome 4.

Therapeutic Options

• Therapies targeted against the pathophysiological pathways that underlie pulmonary arterial hypertension, such as endothelin receptor antagonists (e.g., bosentan), phosphodiesterase type V inhibitors (e.g., sildenafil), and prostacyclin analogs, have shown promise in improving hemodynamics and exercise capacity in patients with Eisenmenger syndrome 5.
• The use of combinations of targeted therapies may offer an alternative option for treatment of patients with Eisenmenger syndrome 5.

Complications and Co-morbidities

• Eisenmenger syndrome is associated with significant morbidity and mortality, and patients are at risk of developing co-morbidities such as coagulation disorders, renal dysfunction, hypertrophic osteoarthropathy, heart failure, and reduced quality of life 2, 4.
• Multiorgan disease is an integral manifestation of Eisenmenger syndrome, involving the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems 3.