title: Applicability of bosentan in Dutch patients with Eisenmenger syndrome: preliminary results on safety and exercise capacity.
abstract: BACKGROUND
  The purpose of this study was to investigate the applicability of bosentan treatment in a broad selection of patients with Eisenmenger syndrome.
  
  
  METHODS
  Dutch patients with Eisenmenger syndrome in New York Heart Association functional class III, 9 (41%) male and 13 (59%) female, including 11 patients with Down syndrome (50%), aged 20 to 61 years (median 37 years), were screened for an open-label, standardised treatment protocol. Patients underwent clinical examinations, six-minute walk test (6-MWT), resting oxygen saturation measurements, cardiac MRI, Doppler echocardiography, lung function tests and exercise capacity testing by peak oxygen consumption at baseline. At 12 weeks of treatment 6-MWT and at 26 weeks 6-MWT and Doppler echocardiography were repeated.
  
  
  RESULTS
  Median follow-up of the patients who started bosentan treatment was five months (range 0.5 to 9.6 months). Oxygen saturation at baseline was 83% (range 76 to 91%) and did not decrease during treatment. Compared with baseline, 6-MWT increased after 12 weeks from 333±93 m to 384±89 m. None of the patients discontinued medication and no liver function abnormalities were observed. Of all Doppler echocardiographic and MRI parameters measured for right ventricular function, tricuspid annular peak systolic velocities using tissue Doppler imaging (TDI-S) was the only independent predictor for six-minute walk test (β=0.8, p=0.001).
  
  
  CONCLUSIONS
  Bosentan may be safely applied in patients with Eisenmenger syndrome combined with complex congenital heart disease or with Down syndrome. Our preliminary results suggest that exercise tolerance improves during the first months of bosentan treatment.
authors.forename: M G J
authors.initials: MG
authors.lastname: Duffels
authors.forename: R M F
authors.initials: RM
authors.lastname: Berger
authors.forename: P
authors.initials: P
authors.lastname: Bresser
authors.forename: H A C M
authors.initials: HA
authors.lastname: de Bruin-Bon
authors.forename: E
authors.initials: E
authors.lastname: Hoendermis
authors.forename: B J
authors.initials: BJ
authors.lastname: Bouma
authors.forename: B J M
authors.initials: BJ
authors.lastname: Mulder
keywords: Eisenmenger syndrome; congenital heart disease; medical treatment