Bosentan for the treatment of pulmonary arterial hypertension associated with congenital heart defects

Bosentan is an effective first-line therapy in New York Heart Association (NYHA) III patients with idiopathic pulmonary arterial hypertension (PAH). Pre-clinical data support the rationale for the potential benefit of bosentan in PAH associated with congenital heart disease (CHD).We performed a retrospective analysis of patients with PAH-associated CHD who were treated with bosentan on top of conventional therapy. Bosentan was started at 62.5 mg...