PT  - JOURNAL ARTICLE
AU  - Michele D'Alto
AU  - Emanuele Romeo
AU  - Paola Argiento
AU  - Anna Correra
AU  - Berardo Sarubbi
AU  - Maria Pignatiello
AU  - Antonietta Caronna
AU  - Carmine Dario Vizza
AU  - Raffaele Calabrò
AU  - Maria Giovanna Russo
TI  - Efficacy and safety of oral bosentan in patients with Down's syndrome and pulmonary arterial hypertension due to congenital heart disease
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - P921
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/P921.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/P921.full
SO  - Eur Respir J2012 Sep 01; 40
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.