Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Issue 8 (16th July 2007)

• Record Type: Journal Article
• Authors: Diller, Gerhard-Paul; Dimopoulos, Konstantinos; Kaya, Mehmet G; Harries, Carl; Uebing, Anselm; Li, Wei; Koltsida, Evdokia; Gibbs, J Simon R; Gatzoulis, Michael A
• Is Part Of: Heart. Volume 93:Issue 8(2007)
• Page Start: 974
• Page End: 976
• Publication Date: 2007-07-16
• Subjects: PAH, pulmonary arterial hypertension -- SMWTd, 6-minute walk test distance -- VSD, ventricular septal defect; bosentan -- congenital heart disease -- Eisenmenger's syndrome -- endothelin antagonism -- pulmonary arterial hypertension; Heart -- Diseases -- Treatment -- Periodicals; Cardiology -- Periodicals; 616.12
• DOI: 10.1136/hrt.2006.089185
• Languages: English
• ISSNs: 1355-6037

Abstract

Abstract : Objective: To examine long-term safety and efficacy of bosentan—an oral dual endothelin receptor antagonist—in patients with pulmonary hypertension associated with congenital heart disease or Eisenmenger's syndrome. Design: Retrospective study. Setting: Tertiary cardiology referral centre. Patients: All adult patients with pulmonary arterial hypertension associated with congenital heart disease treated with bosentan at the Royal Brompton Adult Congenital Heart Centre were included. Main outcome measures: Oxygen saturation, functional (WHO) class, 6-minute walk test distance and liver enzymes were analysed. Results: Eighteen patients (14 female) with pulmonary arterial hypertension associated with congenital heart disease (15 patients with Eisenmenger's syndrome) with a mean (SD) age of 41 (9) years (range 23–69) were included. Median follow-up was 29 months (range 1–39). One patient died during follow-up. Patients tolerated bosentan well and no significant rise in liver transaminases was seen. Arterial oxygen saturation remained stable throughout follow-up. Mean (SD) functional class (p = 0.001) and the 6-minute walk test distance improved compared with baseline (284 (144) vs 363 (124) m, 380 (91) m and 408 (114) m at baseline, 0–6 months, 6–12 months and 1–2 years of treatment, respectively; p<0.05 for each). Conclusions: Bosentan appears to be safe and well tolerated in adults with pulmonary arterial hypertension associated with congenital heart disease or Eisenmenger's syndrome during mid- to long-term follow-up. In addition, functional class and the 6-minute walk test distance improved and this effect was maintained for up to 2 years of bosentan treatment.