Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease

• Published in: Heart (British Cardiac Society) Vol. 93; no. 8; pp. 974 - 976
• Main Authors: Diller, Gerhard-Paul, Dimopoulos, Konstantinos, Kaya, Mehmet G, Harries, Carl, Uebing, Anselm, Li, Wei, Koltsida, Evdokia, Gibbs, J Simon R, Gatzoulis, Michael A
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and British Cardiovascular Society 01.08.2007; BMJ; BMJ Publishing Group LTD; BMJ Group
• Subjects: 6-minute walk test distance; Adult; Aged; Antihypertensive Agents - therapeutic use; Biological and medical sciences; bosentan; Cardiology. Vascular system; Cardiovascular disease; Congenital diseases; congenital heart disease; Defects; Eisenmenger Complex - complications; Eisenmenger Complex - drug therapy; Eisenmenger Complex - physiopathology; Eisenmenger’s syndrome; endothelin antagonism; Exercise Tolerance; Follow-Up Studies; Heart; Heart Defects, Congenital - complications; Heart Defects, Congenital - drug therapy; Heart Defects, Congenital - physiopathology; Humans; Hypertension; Hypertension, Pulmonary - complications; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - physiopathology; Medical sciences; Middle Aged; Oxygen - blood; PAH; Patients; Physiology; Pneumology; pulmonary arterial hypertension; Pulmonary Hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Safety; SMWTd; Studies; Sulfonamides - therapeutic use; ventricular septal defect; VSD; Human; Respiratory disease; Toxicity; Cardiovascular disease; Long term; Phlebology; Artery; Pulmonary hypertension; Association; Bosentan; Efficiency; Adult; Complication; Circulatory system; Safety; Cardiology; Congenital cardiopathy
• ISSN: 1355-6037; 1468-201X
• DOI: 10.1136/hrt.2006.089185

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.