PURPOSE: Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down’s syndrome (DS), the effect of bosentan is largely unknown. Aim of the study was to evaluate the long-term effects of bosentan in adult patients with CHD-related PAH with and without DS.
METHODS: WHO functional class, resting oxygen saturation, 6-minute walk test (6MWT) and hemodynamics were assessed at baseline and after 12 months of bosentan therapy in patients with CHD-related PAH 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 with and without DS patients showed an improvement in WHO functional class (DS: 2.5±0.5 vs 2.9±0.6, p=0.005; no DS: 2.5±0.5 vs 2.9±0.5, p=0.000002), 6-minute walk distance (DS: 288±71 vs 239±74 m, p=0.0007; no DS: 389±80 vs 343±86 m, p=0.00003), and hemodynamics (pulmonary flow, DS: 4.0±1.6 vs 3.5±1.4 l/m/m2, p=0.006; no DS: 3.5±1.4 vs 2.8±1.0 l/m/m2, p=0.0005; pulmonary to systemic flow ratio, DS: 1.4±0.7 vs 1.0±0.4, p=0.003; no DS: 1.1±0.7 vs 0.9±0.3, p=0.012; pulmonary vascular resistance index, DS: 15±9 vs 20±13 WU.m2, p=0.007; no DS: 20±10 vs 26±15 WU.m2, p=0.002). 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.
CLINICAL IMPLICATIONS: Oral bosentan is safe and effective in patients with CHD-related PAH and Down's syndrome.
DISCLOSURE: The following authors have nothing to disclose: Michele D'Alto, Emanuele Romeo, Paola Argiento, Anna Correra, Berardo Sarubbi, Silvia Papa, Maria Russo, Carmine Vizza, Eduardo Bossone, Raffaele Calabrò
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