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Title: | Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease | Authors: | Baptista, Rui Castro, Graça Silva, António Marinho da Monteiro, Pedro Providência, Luís Augusto |
Keywords: | Bosentan; Congenital heart disease; Pulmonary hypertension; Bosentan; Cardiopatia congénita; Hipertensão pulmonar | Issue Date: | Feb-2013 | Serial title, monograph or event: | Revista Portuguesa de Cardiologia | Volume: | 32 | Issue: | 2 | Abstract: | Background: Bosentan is recommended for symptomatic patients with Eisenmenger syndrome
due to simple congenital lesions such as atrial and ventricular septal defects (VSD). However, its
long-term efficacy and safety in patients with pulmonary arterial hypertension (PAH) associated
with complex congenital heart disease (CHD) is unknown.
Objectives: We examined the short- and long-term effects and safety profile of bosentan in
patients with PAH and complex CHD.
Methods: We followed 14 patients with PAH and complex CHD for a mean of four years. Demographic
parameters, exercise capacity assessed by the six-minute walking test (6MWT) and
oxygen saturation were assessed at baseline, six months and at follow-up.
Results: Mean age was 37.1±11.7 years; 90% were in WHO class III or IV. The most common diagnosis
was pulmonary atresia with VSD (35.7%), followed by truncus arteriosus (28.6%), patent
ductus arteriosus (21.4%) and transposition of the great arteries (14.3%). After six months of
treatment, six-minute walking distance (6MWD) increased from 371.9 to 428.4 m (p=0.005)
and functional class was improved (p=0.005). After four years, one patient discontinued bosentan
due to side effects and four patients were started on sildenafil, after a mean 38 months of
bosentan treatment. Mean 6MWD for patients on bosentan monotherapy (n=8) was 440.1±103.8
m, whereas for patients on bosentan-sildenafil combination therapy (n=4) it was 428.8±96.9
m, after four years of therapy. Two patients died during follow-up.
Conclusions: Bosentan was safe and was associated with improved exercise capacity in patients
with PAH and complex CHD. This improvement was sustained for up to four years and the safety
profile was similar to simple CHD patients. Introdução: O bosentano é recomendado em doentes com hipertensão arterial pulmonar (HAP) associada a lesões congénitas simples, como comunicações interventriculares (CIV). Contudo, a sua eficácia e segurança a longo prazo em doentes com HAP associada a cardiopatias congénitas complexas (HAP-CCC) é desconhecida. Objectivos: Avaliámos a eficácia e seguranc¸a a curto e longo-prazo do bosentano na HAP-CCC. Métodos: Estudaram-se 14 doentes com HAP-CCC, relativamente a parâmetros demográficos, capacidade funcional avaliada pelo teste de marcha de seis minutos (TM6M) e saturação de oxigénio, antes de iniciar terapêutica, aos seis meses e durante o período de seguimento clínico a longo-prazo (quatro anos). Resultados: Noventa por cento dos doentes encontravam-se em classe OMS III ou IV, com idade média de 37,1 ± 11,7 anos. O diagnóstico mais frequente foi a atrésia pulmonar com CIV (35,7%), seguida de truncus arteriosus (28,6%), canal arterial patente (21,4%) e transposição de grandes vasos (14,3%). Após seis meses de tratamento, o TM6M aumentou de 371,9 para 428,4 metros (p = 0,005) e a classe funcional melhorou (p = 0,005). Após quatro anos, um doente suspendeu bosentano devido a efeitos secundários e quatro doentes iniciaram sildenafil, após uma duração média de monoterapia de 38 meses. Após quatro anos de terapêutica, nos doentes em monoterapia com bosentano (n = 8) o TM6M foi de 440,1 ± 103,8 metros, enquanto que nos doentes com bosentano-sildenafil (n = 4) foi de 428,8 ± 96,9 metros. Dois doentes faleceram durante o período de seguimento clínico. Conclusões: O bosentano foi seguro e esteve associado a melhoria na capacidade funcional em doentes com HAP-CCC até aos quatro anos de seguimento clínico. |
URI: | https://hdl.handle.net/10316/102695 | ISSN: | 0870-2551 | DOI: | 10.1016/j.repc.2012.02.023 | Rights: | openAccess |
Appears in Collections: | FMUC Medicina - Artigos em Revistas Nacionais |
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