Author/Authors :
Duffels، نويسنده , , Mariëlle G.J. and Vis، نويسنده , , Jeroen C. and van Loon، نويسنده , , Rosa L.E. and Nieuwkerk، نويسنده , , Pythia T. and van Dijk، نويسنده , , Arie P.J. and Hoendermis، نويسنده , , Elke S. and de Bruin-Bon، نويسنده , , Rianne H.A.C.M. and Bouma، نويسنده , , Berto J. and Bresser، نويسنده , , Paul D. Berger، نويسنده , , Rolf M.F. and Mulder، نويسنده , , Barbara J.، نويسنده ,
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 Downʹs 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 Downʹs syndrome (n = 28). Median follow-up of all patients treated with bosentan was 22 months (range 3 to 36). In patients without Downʹs 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 Downʹs syndrome, 6-minute walk distance and QoL were stable during treatment. In conclusion, findings suggested that in patients without Downʹs syndrome, longer term bosentan treatment resulted in a persistent improvement in QoL and stabilization of exercise capacity.
