Background Oral bosentan is an established treatment for pulmonary arterial hypertension (PAH).
Objective The aim of the present study was to evaluate safety, tolerability, and clinical and haemodynamic impact of bosentan in patients with PAH related to congenital heart disease (CHD).
Patients Twenty-two patients with PAH CHD-related (8M, 14F, mean age 38±10) were treated with oral bosentan (62.5 mg x 2/die for the first month and then 125 mg x 2/die).
Main outcome measures Clinical status, liver enzymes, WHO functional class, resting oxygen saturations and 6-min walk test (6MWT) were assessed at baseline and at 1, 3, 6 and 12 month. Haemodynamic evaluation with cardiac catheterization was performed at baseline and at 12-month follow-up.
Results Twelve patients had ventricular septal defect, 5 atrio-ventricular canal, 4 single ventricle and one atrial septal defect. All patients tolerated bosentan well. No major side effects were observed. After a year of therapy we observed an improvement in WHO functional class (2.5±0.7 vs 3.1±0.7; p<0.05), oxygen saturation at rest (87±6% vs 81±9; p<0.001), heart rate at rest (81±10 vs 87±14 bpm; p<0.05), distance travelled in the 6MWT (394±73 vs 320±108 m; p<0.001), oxygen saturation at the end of 6MWT (71±14 vs 63±17 %; p<0.05), Borg's index (5.3±1.8vs 6.5±1.3; p<0.001), pulmonary vascular resistances index (PVRi: 14±9 vs 22±12 WU.m2; p<0.001), systemic vascular resistances index (SVRi: 23±11 vs 27±10 WU.m2; p<0.01), PVRi/SVRi (0.6±0.5 vs 0.9±0.6; p<0.05); pulmonary (4.0±1.3 vs 2.8±0.9 l/min/m2; p<0.001) and systemic cardiac output (4.2±1.4 vs 3.4±1.1 l/min/m2; p<0.05).
Conclusions Bosentan was safe and well tolerated in adults with PAH CHD-related during 12 months of treatment. Clinical status, exercise tolerance and pulmonary haemodynamics significantly improved.
- congenital heart defects
- eisenmenger syndrome
- pulmonary hypertension
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