title: Long-term bosentan treatment of complex congenital heart disease and Eisenmenger's syndrome.
abstract: The BREATHE-5 study demonstrated that bosentan, an oral endothelin receptor antagonist, provides clinical benefits in patients with Eisenmenger's syndrome. As a result, the European Medicines Agency (EMEA) approved its use for this indication. However, follow-up in that study was limited to 16 weeks and patients with complex congenital heart disease were excluded. We assessed the effect of long-term bosentan treatment in 10 patients with complex congenital heart disease and Eisenmenger's syndrome. In the mean clinical follow-up period of 25 months, all patients reached the target dose without developing side effects and without experiencing a change in arterial oxygen consumption at either rest or maximal exercise. Moreover, there were significant changes in clinical parameters: NYHA functional class improved from 3.3+/-0.7 to 2.5+/-0.9 (P=.002) and the 6-minute walk distance increased from 266+/-161 m to 347+/-133 m (P=.015).
authors.affiliation: Servicio de Cardiología, Hospital Universitario de Guadalajara, Guadalajara, España.
authors.forename: Eva
authors.initials: E
authors.lastname: Díaz-Caraballo
authors.forename: Ana E
authors.initials: AE
authors.lastname: González-García
authors.forename: Margarita
authors.initials: M
authors.lastname: Reñones
authors.forename: Angel
authors.initials: A
authors.lastname: Sánchez-Recalde
authors.forename: Francisco
authors.initials: F
authors.lastname: García-Río
authors.forename: José M
authors.initials: JM
authors.lastname: Oliver-Ruiz
mesh_terms: D000328:Adult; D000077300:Bosentan; D004541:Eisenmenger Complex / Q000150:complications* / Q000188:drug therapy*; D065128:Endothelin Receptor Antagonists; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000151:congenital* / Q000188:drug therapy*; D006801:Humans; D008297:Male; D013449:Sulfonamides / Q000627:therapeutic use*; D013997:Time Factors
chemical_list: D065128:Endothelin Receptor Antagonists; D013449:Sulfonamides; D000077300:Bosentan