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.
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.
Authors: Nazzareno Galiè; Adam Torbicki; Robyn Barst; Philippe Dartevelle; Sheila Haworth; Tim Higenbottam; Horst Olschewski; Andrew Peacock; Giuseppe Pietra; Lewis J Rubin; Gerald Simonneau; Silvia G Priori; Maria Angeles Alonso Garcia; Jean-Jacques Blanc; Andrzej Budaj; Martin Cowie; Verconcia Dean; Jaap Deckers; Enrique Fernandez Burgos; John Lekakis; Bertil Lindahl; Gianfranco Mazzotta; Keith McGregor; João Morais; Ali Oto; Otto A Smiseth; Joan Albert Barbera; Simon Gibbs; Marius Hoeper; Marc Humbert; Robert Naeije; Joanna Pepke-Zaba Journal: Eur Heart J Date: 2004-12 Impact factor: 29.983
Authors: Ingram Schulze-Neick; Nadine Gilbert; Ralf Ewert; Christian Witt; Ekkehard Gruenig; Beate Enke; Mathias M Borst; Peter E Lange; Marius M Hoeper Journal: Am Heart J Date: 2005-10 Impact factor: 4.749
Authors: Nazzareno Galiè; Maurice Beghetti; Michael A Gatzoulis; John Granton; Rolf M F Berger; Andrea Lauer; Eleonora Chiossi; Michael Landzberg Journal: Circulation Date: 2006-06-26 Impact factor: 29.690
Authors: R N Channick; G Simonneau; O Sitbon; I M Robbins; A Frost; V F Tapson; D B Badesch; S Roux; M Rainisio; F Bodin; L J Rubin Journal: Lancet Date: 2001-10-06 Impact factor: 79.321
Authors: Olivier Sitbon; David B Badesch; Richard N Channick; Adaani Frost; Ivan M Robbins; Gérald Simonneau; Victor F Tapson; Lewis J Rubin Journal: Chest Date: 2003-07 Impact factor: 9.410
Authors: M G J Duffels; M N van der Plas; S Surie; M M Winter; B Bouma; M Groenink; A P J van Dijk; E Hoendermis; R M F Berger; P Bresser; B J M Mulder Journal: Neth Heart J Date: 2009-09 Impact factor: 2.380