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Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease.

Abstract
Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) due to systemic to pulmonary shunting is associated with a high risk of morbidity and mortality. In this study we evaluated 4 years treatment effect of bosentan on exercise capacity and quality of life and survival rates in 64 adult patients with PAH associated with CHD, including patients with Down syndrome (DS). All patients were evaluated at baseline and during follow-up with laboratory tests, 6-minute walk test, quality of life questionnaires, and Doppler echocardiography. In total, 13 patients (20%) died during 4-years of follow-up; 4 patients with DS and 9 patients without DS. Mean follow-up of all patients treated with bosentan was 3.5 ± 1.2 year. We analyzed treatment efficacy separately within patients without DS (n=34) and patients with DS (n=30). Mean 6-minute walking distance (6 MWD) in patients without DS significantly increased at 6 months from 417 ± 108 to 458 ± 104 m (+41 m; p=0.002) and significant improvement continued to exist during at least 2.5 years of follow-up (p=0.003). Moreover, stroke volume increased significantly (p=0.02). In the patients with DS, 6-MWD, stroke volume and quality of life remained stable during treatment. In this study we demonstrate a prolonged beneficial effect of bosentan treatment on exercise capacity, stroke volume and quality of life in patients without DS. However the mortality rate of 20% of patients after 4 years of follow-up remains high.
AuthorsJeroen C Vis, Marielle G Duffels, Pepijn Mulder, Rianne H A C M de Bruin-Bon, Berto J Bouma, Rolf M F Berger, Elke S Hoendermis, Arie P J van Dijk, Barbara J M Mulder
JournalInternational journal of cardiology (Int J Cardiol) Vol. 164 Issue 1 Pg. 64-9 (Mar 20 2013) ISSN: 1874-1754 [Electronic] Netherlands
PMID21723630 (Publication Type: Journal Article)
CopyrightCopyright © 2011 Elsevier Ireland Ltd. All rights reserved.
Chemical References
  • Antihypertensive Agents
  • Sulfonamides
  • Bosentan
Topics
  • Adult
  • Antihypertensive Agents (therapeutic use)
  • Bosentan
  • Down Syndrome (complications)
  • Familial Primary Pulmonary Hypertension
  • Female
  • Heart Defects, Congenital (complications, mortality)
  • Humans
  • Hypertension, Pulmonary (complications, drug therapy, mortality)
  • Male
  • Middle Aged
  • Sulfonamides (therapeutic use)
  • Survival Rate
  • Time Factors

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