Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome

• Published in: International journal of cardiology Vol. 164; no. 3; pp. 323 - 326
• Main Authors: D'Alto, Michele, Romeo, Emanuele, Argiento, Paola, D'Andrea, Antonello, Sarubbi, Berardo, Correra, Anna, Scognamiglio, Giancarlo, Papa, Silvia, Bossone, Eduardo, Calabrò, Raffaele, Vizza, Carmine D, Russo, Maria G
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier Ireland Ltd 15-04-2013; Elsevier
• Subjects: Administration, Oral; Adult; Antihypertensive Agents - administration & dosage; Biological and medical sciences; Cardiology. Vascular system; Cardiovascular; Chromosome aberrations; Congenital heart disease; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Down Syndrome - complications; Down's syndrome; Exercise Tolerance - drug effects; Familial Primary Pulmonary Hypertension; Female; Follow-Up Studies; Heart; Heart Defects, Congenital - complications; Hemodynamics - drug effects; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Male; Medical genetics; Medical sciences; Middle Aged; Pneumology; Prospective Studies; Pulmonary arterial hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Sulfonamides - administration & dosage; Treatment Outcome; Young Adult; Down's syndrome; Pulmonary arterial hypertension; Congenital heart disease; Chromosomal aberration; Trisomy; Respiratory disease; Chromosome G21; Aneuploidy; Cardiovascular disease; Down syndrome; Artery; Pulmonary hypertension; Treatment; Blood vessel; Circulatory system; Cardiology; Congenital cardiopathy
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2011.07.009

Abstract Background Oral bosentan is effective in pulmonary arterial hypertension (PAH) related to congenital heart disease (CHD). In patients with Down's syndrome, 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 Down's syndrome. 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 Down's syndrome patients showed an improvement in WHO functional class (Down: 2.5 ± 0.5 vs 2.9 ± 0.6, p = 0.005; controls: 2.5 ± 0.5 vs 2.9 ± 0.5, p = 0.000002), 6-minute walk distance (Down: 288 ± 71 vs 239 ± 74 m, p = 0.0007; controls: 389 ± 80 vs 343 ± 86 m, p = 0.00003), and hemodynamics (pulmonary flow, Down: 4.0 ± 1.6 vs 3.5 ± 1.4 l/m/m2 , p = 0.006; controls: 3.5 ± 1.4 vs 2.8 ± 1.0 l/m/m2 , p = 0.0005; pulmonary to systemic flow ratio, Down: 1.4 ± 0.7 vs 1.0 ± 0.4, p = 0.003; controls: 1.1 ± 0.7 vs 0.9 ± 0.3, p = 0.012; pulmonary vascular resistance index, Down: 15 ± 9 vs 20 ± 13 WU m2 , p = 0.007; controls: 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.