RT Journal Article
SR Electronic
T1 Efficacy and safety of oral bosentan in patients with Down's syndrome and pulmonary arterial hypertension due to congenital heart disease
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P921
VO 40
IS Suppl 56
A1 Michele D'Alto
A1 Emanuele Romeo
A1 Paola Argiento
A1 Anna Correra
A1 Berardo Sarubbi
A1 Maria Pignatiello
A1 Antonietta Caronna
A1 Carmine Dario Vizza
A1 Raffaele Calabrò
A1 Maria Giovanna Russo
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P921.abstract
AB 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.View this table:Clinical and haemodynamic variables before and after oral bosentan treatment in patients with and without Down's syndromeNo 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.