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Cardiomiopatía hipertrófica con disfunción sistólica ventricular izquierda: información del registro SHaRe
Peter Marstrand1,2, Larry Han3, Sharlene M Day4
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA (P.M., N.K.L., C.Y.H.).
Circulation
|April 2, 2020
Resumen
La cardiomiopatía hipertrófica con disfunción sistólica del ventrículo izquierdo (HCM-LVSD) afecta al 8% de los pacientes. Aunque variable, el 75% experimenta eventos adversos, y el 35% se enfrenta a un resultado compuesto dentro de 8,4 años.
Área de la Ciencia:
- Cardiología
- La genética
- Investigación de la insuficiencia cardíaca
Sus antecedentes:
- La cardiomiopatía hipertrófica con disfunción sistólica ventricular izquierda (HCM-LVSD) se define por una fracción de eyección < 50%.
- El pronóstico y la historia natural de HCM-LVSD siguen siendo poco conocidos debido a su rareza.
Objetivo del estudio:
- Para describir la historia natural de HCM-LVSD.
- Identificar los factores predictivos del pronóstico y el desarrollo de incidentes de HCM-LVSD.
Principales métodos:
- Se utilizaron datos de 6793 pacientes con CMH en 11 centros internacionales especializados (Registro de HaRe).
- Utilizó modelos de riesgos proporcionales de Cox para analizar los resultados y los factores de riesgo.
Principales resultados:
- HCM-LVSD ocurrió en el 8% (553 pacientes) de la cohorte.
- El 75% de los pacientes con HCM-LVSD experimentaron eventos adversos; el 35% alcanzó un resultado compuesto (muerte, trasplante, LVAD) con un tiempo medio de 8,4 años.
- Los predictores del resultado compuesto incluyeron múltiples variantes sarcoméricas, fibrilación auricular y LVEF < 35%. Los predictores de la incidencia de HCM-LVSD incluyeron el tamaño de LV, el grosor de la pared, LVEF 50-60%, la mejora tardía del gadolinio y las variantes sarcoméricas.
Conclusiones:
- HCM-LVSD afecta a una minoría significativa de pacientes con HCM, a menudo con resultados graves.
- Los factores genéticos, específicamente las variantes sarcoméricas (filamento múltiple o delgado), juegan un papel crucial tanto en el pronóstico como en la progresión de la enfermedad.
- Las características clínicas y los hallazgos de las imágenes cardíacas también son predictores clave para los eventos adversos y el desarrollo de la enfermedad en HCM-LVSD.
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