Clinical Features and Outcomes of Pediatric <i>MYH7</i>-Related Dilated Cardiomyopathy.

ABSTRACT:

Background

Although genetic variants in MYH7 are the most frequent cause of pediatric genetic dilated cardiomyopathy (DCM), there are no studies available describing this entity. We sought to describe clinical features, analyze variant location, and explore predictors of bad prognosis in pediatric MYH7-related DCM.

Methods and results

We evaluated clinical records from 44 patients (24 men; median age at diagnosis, 0.54 [interquartile range, 0.01-10.8] years) with pathogenic/likely pathogenic variants in MYH7 diagnosed with DCM at pediatric age (<18 years) followed at 13 international centers. We also explored risk factors associated with a composite end point of end-stage heart failure defined as heart transplantation or heart failure-related death. Twen

SUBMITTER: de Frutos F 

PROVIDER: S-EPMC11935667 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature