title: Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down's syndrome.
abstract: Pulmonary arterial hypertension associated with congenital heart disease caused by systemic-to-pulmonary shunting was associated with a high risk of morbidity and mortality. In this retrospective study, the longer term treatment effect of bosentan on exercise capacity and quality of life (QoL) were evaluated in 58 adult patients (>18 years) with pulmonary arterial hypertension associated with congenital heart disease, including patients with Down's syndrome. All patients were evaluated at baseline and during follow-up using laboratory tests, 6-minute walk test, QoL questionnaires, and Doppler echocardiography. Treatment efficacy was analyzed separately for patients without (n = 30) and with Down's syndrome (n = 28). Median follow-up of all patients treated with bosentan was 22 months (range 3 to 36). In patients without Down's syndrome, mean 6-minute walk distance increased from 427 +/- 97 to 461 +/- 104 m (p <0.01) after 6 months of treatment, followed by a gradual return to baseline and disease stabilization. QoL improved significantly during treatment and was maintained during 18 months of follow-up (p <0.05). In patients with Down's syndrome, 6-minute walk distance and QoL were stable during treatment. In conclusion, findings suggested that in patients without Down's syndrome, longer term bosentan treatment resulted in a persistent improvement in QoL and stabilization of exercise capacity.
authors.affiliation: Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands. m.g.duffels@amc.uva.nl
authors.forename: Mariëlle G J
authors.initials: MG
authors.lastname: Duffels
authors.forename: Jeroen C
authors.initials: JC
authors.lastname: Vis
authors.forename: Rosa L E
authors.initials: RL
authors.lastname: van Loon
authors.forename: Pythia T
authors.initials: PT
authors.lastname: Nieuwkerk
authors.forename: Arie P J
authors.initials: AP
authors.lastname: van Dijk
authors.forename: Elke S
authors.initials: ES
authors.lastname: Hoendermis
authors.forename: Rianne H A C M
authors.initials: RH
authors.lastname: de Bruin-Bon
authors.forename: Berto J
authors.initials: BJ
authors.lastname: Bouma
authors.forename: Paul
authors.initials: P
authors.lastname: Bresser
authors.forename: Rolf M F
authors.initials: RM
authors.lastname: Berger
authors.forename: Barbara J M
authors.initials: BJ
authors.lastname: Mulder
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D004314:Down Syndrome / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D005080:Exercise Test / Q000379:methods*; D017079:Exercise Tolerance / Q000187:drug effects* / Q000502:physiology; D005260:Female; D005500:Follow-Up Studies; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011336:Probability; D011788:Quality of Life; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D013449:Sulfonamides / Q000627:therapeutic use*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000959:Antihypertensive Agents; D013449:Sulfonamides; D000077300:Bosentan