Clinical manifestations, genetic profiles, and sudden cardiac arrest in pediatric hypertrophic cardiomyopathy: Challenges of risk prediction for initial sudden cardiac arrest presentations.

ABSTRACT:

Background

Sudden cardiac arrest (SCA) is a leading cause of death in pediatric hypertrophic cardiomyopathy (HCM).

Objective

The study sought to analyze the clinical and genetic characteristics of pediatric HCM and assess the applicability of current SCA risk prediction models.

Methods

We enrolled individuals diagnosed as HCM before 20 years of age, between 2000 and 2020, excluding those secondary to hemodynamic causes and those associated with genetic syndromes other than RASopathies.

Results

Among 91 patients (31 female, 60 male), SCA occurred in 13 (14.3%) patients, with 6 (46%) cases presenting as the initial symptom. These 6 patients were older and had lower left ventricular mass z scores compared with those who experienced SCA later during follow-u

SUBMITTER: Chiu SN 

PROVIDER: S-EPMC12302196 | biostudies-literature | 2025 Jul

REPOSITORIES: biostudies-literature