Amiloidosis cardiaca, manifestaciones clínicas y enfoque diagnóstico
Palabras clave:
Amiloidosis, amiloidosis por cadena ligera de inmunoglobulina, amiloidosis hereditaria relacionada con transtiretina, amiloidosis familiar.Resumen
La amiloidosis es una enfermedad heterogénea caracterizada por la acumulación extracelular de proteínas fibrilares con estructura en láminas β, que pueden depositarse en distintos órganos y generar manifestaciones clínicas variables según el sitio afectado. Esta heterogeneidad clínica convierte su diagnóstico en un desafío médico. La afectación cardíaca representa la principal causa de morbilidad y mortalidad en estos pacientes, siendo la amiloidosis cardíaca la forma más común de miocardiopatía restrictiva. La amiloidosis AL presenta una incidencia estimada de 1 caso por cada 100 000 personas-año y constituye cerca del 78 % de los nuevos diagnósticos. La amiloidosis ATTR representa entre el 10 % y el 20 % de los casos, con formas hereditarias (ATTRm) y adquiridas (ATTRwt), mientras que la amiloidosis AA, de origen inflamatorio y adquirido, corresponde aproximadamente al 6 % de los casos anuales. El diagnóstico temprano es fundamental, dado que alrededor del 75 % de los subtipos de amiloidosis comprometen el corazón. La identificación oportuna y el inicio precoz del tratamiento son determinantes para disminuir la progresión de la enfermedad y limitar el daño orgánico, especialmente el cardíaco.
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