Abstract
Aim. To evaluate the long-term effects of oral bosentan in adult patients with pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD) with and without Down's syndrome.
Methods. WHO functional class, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in CHD-PAH patients with and without Down's syndrome.
Results. Seventy-four consecutive patients were enrolled: 18 with and 56 without Down's syndrome. After 12 months of bosentan therapy, both groups showed an improvement in WHO functional class, 6-minute walk distance and hemodynamics.
No differences in the efficacy of therapy were observed between the two groups.
Conclusions. Bosentan was safe and well tolerated in adult patients with CHD-related PAH with and without Down's syndrome during 12 months of treatment. Clinical status, exercise tolerance, and pulmonary hemodynamics improved, regardless of the presence of Down's syndrome.
- © 2012 ERS