A 35-year-old man presents with progressive exertional dyspnoea, clubbing, and cyanosis since childhood. Echocardiography shows right-to-left shunt through a VSD and Eisenmenger physiology (pulmonary arterial pressure equal to systemic). SpO2 is 82% on room air. Which therapy is now approved and most appropriate for symptom and haemodynamic improvement in Eisenmenger syndrome?
- A Bosentan (endothelin receptor antagonist) ✓
- B Surgical closure of VSD
- C Sildenafil plus systemic anticoagulation
- D Lung transplantation as first-line therapy
Explanation
Eisenmenger syndrome represents the most severe form of pulmonary arterial hypertension associated with congenital heart disease. Surgical closure of the shunt is absolutely contraindicated once Eisenmenger physiology is established (irreversible pulmonary vascular disease). Bosentan (endothelin receptor antagonist) has been specifically approved for Eisenmenger syndrome (BREATHE-5 trial), improving exercise capacity and haemodynamics. Other PAH therapies (sildenafil, tadalafil, macitentan) are also used. Anticoagulation is not routinely given (bleeding risk from haemoptysis). Transplantation is reserved for end-stage disease refractory to all therapy.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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