Bosentan in Eisenmenger syndrome and chronic thromboembolic pulmonary hypertension

Bosentan, a dual endothelin receptor antagonist, has been used for symptomatic improvement of patients with idiopathic pulmonary arterial hypertension (PAH) and specific types of secondary (e.g. scleroderma and human immunodeficiency virus infection) PAH, but its efficacy in patients with congenital heart disease and chronic thromboembolic pulmonary hypertension is still under evaluation. In this study two patients are presented, one with common atrioventricular canal and pulmonary hypertension and the other with recurrent pulmonary thromboembolism; both improved significantly after bosentan administration.

The first patient was a 38-year-old female with trisomy 21, common atrioventricular canal and pulmonary hypertension (Eisenmenger syndrome). The second patient was a 57-year-old male, who was on anticoagulant therapy owing to an episode of pulmonary embolism that had been diagnosed 3 years earlier. Recurrent pulmonary thromboembolism was considered the most probable diagnosis. Chest spiral computed tomography examination excluded pulmonary endarterectomy as a therapeutic approach. At admission, the two patients were on New York Heart Association class IV, diagnosed by progressive rest dyspnea. Bosentan was administered to patients 1 and 2 for 6 and 9 months, respectively (62.5 mg b.i.d. for 4 weeks, then 125 mg b.i.d.).

Bosentan therapy resulted in significant clinical improvement in both patients. No hepatic dysfunction nor any other side-effect was observed.

Bosentan could be an effective therapy for pulmonary hypertension owing to congenital heart disease and chronic thromboembolic disease.