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Updated: Jan 28, 2026
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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
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Amiloidosis cardíaca por cadenas ligeras sistémica con bloqueo auriculoventricular
1Department of Cardiology, The Third Affiliated Hospital of Zhejiang Chinese Medical University, Hangzhou, China.
Frontiers in cardiovascular medicine
|January 26, 2026
Resumen
Los pacientes con amiloidosis sistémica de cadenas ligeras (AL) pueden experimentar eventos cardíacos tardíos a pesar del tratamiento. La monitorización continua con un monitor cardíaco implantable es crucial para detectar arritmias potencialmente mortales y guiar la implantación de marcapasos.
Área de la Ciencia:
- Cardiología
- Hematología
- Electrofisiología
Sus antecedentes:
- La amiloidosis sistémica de cadenas ligeras (AL) es un trastorno raro de las células plasmáticas.
- La afectación cardíaca es común (60-75%), con mal pronóstico sin tratamiento.
- El bloqueo de la conducción auriculoventricular (AV) afecta al 3-5% de los pacientes con AL.
Objetivo del estudio:
- Destacar el riesgo de eventos electrofisiológicos tardíos en pacientes con amiloidosis AL.
- Enfatizar la utilidad de los monitores cardíacos implantables (DAI) en el manejo de estos pacientes.
Principales métodos:
- Informe de caso de un hombre de 50 años con amiloidosis cardíaca AL.
- El paciente logró la remisión hematológica y la mejora cardíaca después de la terapia y el trasplante de células madre.
- Se utilizó un monitor cardíaco implantable (DAI) para detectar asistolia, lo que llevó a la implantación de un marcapasos.
Principales resultados:
- A pesar del tratamiento y la recuperación cardíaca, el paciente experimentó síncope debido a asistolia.
- El DAI detectó un evento de asistolia de 7 segundos.
- La implantación de marcapasos resolvió el síncope y redujo las arritmias auriculares.
Conclusiones:
- Las mejoras hematológicas y cardíacas no garantizan la estabilidad electrofisiológica en la amiloidosis AL.
- Es esencial la vigilancia de la enfermedad de conducción oculta en pacientes de alto riesgo.
- Los DAI son vitales para detectar bradiarritmias intermitentes y guiar la intervención.
Palabras clave:
bloqueo auriculoventricularamiloidosis cardíacamonitor cardíaco implantabletipo de cadena ligerasistémicaMás Videos Relacionados
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