Bosentan in the Treatment of Pulmonary Arterial Hypertension in Adults with Congenital Heart Defects: Preliminary Experience of a Multicenter Cooperation Project

Introduction: Bosentan is effective in the treatment of primary pulmonary arterial hypertension (PAH). As yet there are no published data concerning its use in adult PAH patients with congenital heart defects. Methods: Thirty-four patients (age 45±15 years; 23 pts with Eisenmenger syndrome) at 6 specialized centers were treated with a bosentan dose of 62,5–250mg/d. Before and after 16,4±5,7 months of therapy, clinical, laboratory, echocardiographic and hemodynamic parameters were measured. Results: The walking range improved from 359±109 m to 423±92 m (p=0,002); this correlated with an improvement of the mean NY…