RESUMEN
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Asunto(s)
Humanos , Masculino , Femenino , Adulto Joven , Adulto , Persona de Mediana Edad , Cardiopatías Congénitas/complicaciones , Tumores Neuroendocrinos/epidemiología , Complejo de Eisenmenger/complicaciones , Cianosis , Paraganglioma/epidemiología , Feocromocitoma/epidemiologíaRESUMEN
El bosentán, antagonista oral de los receptores de la endotelina, ha demostrado beneficio clínico en los pacientes en situación de Eisenmenger en el estudio BREATHE-5, lo que ha permitido la aprobación de su uso para tal indicación por la EMEA (Agencia Europea de Medicamentos). Sin embargo, en este estudio el seguimiento se limitó a 16 semanas y excluyó a los pacientes con cardiopatías congénitas complejas (CCC). Valoramos el efecto a largo plazo del tratamiento con bosentán en 10 pacientes con CCC en situación de Eisenmenger. Con un seguimiento clínico medio de 25 meses, todos los pacientes alcanzaron las dosis óptimas sin efectos colaterales ni variación en la saturación arterial de oxígeno en reposo o al máximo esfuerzo. Sí hubo cambios significativos en los parámetros clínicos: la clase funcional NYHA mejoró de 3,3 ± 0,7 a 2,5 ± 0,9 (p = 0,002) y la distancia recorrida en el test de la marcha de los 6 minutos (TM6M) de 266 ± 161 m a 347 ± 133 m (p = 0,015) (AU)
The BREATHE-5 study demonstrated that bosentan, an oral endothelin receptor antagonist, provides clinical benefits in patients with Eisenmenger’s syndrome. As a result, the European Medicines Agency (EMEA) approved its use for this indication. However, follow-up in that study was limited to 16 weeks and patients with complex congenital heart disease were excluded. We assessed the effect of long-term bosentan treatment in 10 patients with complex congenital heart disease and Eisenmenger’s syndrome. In the mean clinical follow-up period of 25 months, all patients reached the target dose without developing side effects and without experiencing a change in arterial oxygen consumption at either rest or maximal exercise. Moreover, there were significant changes in clinical parameters: NYHA functional class improved from 3.3 (0.7) to 2.5 (0.9) (P=.002) and the 6-minute walk distance increased from 266 (161) m to 347 (133) m (P=.015) (AU)
Asunto(s)
Humanos , Masculino , Femenino , Receptores de Endotelina , Cardiopatías Congénitas/tratamiento farmacológico , Complejo de Eisenmenger/terapia , Antihipertensivos/uso terapéutico , Puente Cardiopulmonar/métodos , Hemodinámica , Hemodinámica/fisiologíaRESUMEN
The BREATHE-5 study demonstrated that bosentan, an oral endothelin receptor antagonist, provides clinical benefits in patients with Eisenmenger's syndrome. As a result, the European Medicines Agency (EMEA) approved its use for this indication. However, follow-up in that study was limited to 16 weeks and patients with complex congenital heart disease were excluded. We assessed the effect of long-term bosentan treatment in 10 patients with complex congenital heart disease and Eisenmenger's syndrome. In the mean clinical follow-up period of 25 months, all patients reached the target dose without developing side effects and without experiencing a change in arterial oxygen consumption at either rest or maximal exercise. Moreover, there were significant changes in clinical parameters: NYHA functional class improved from 3.3+/-0.7 to 2.5+/-0.9 (P=.002) and the 6-minute walk distance increased from 266+/-161 m to 347+/-133 m (P=.015).
