Determination of Genotype and Phenotypes in Pediatric Patients With Biventricular Noncompaction.

ABSTRACT:

Background

Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy characterized by prominent trabeculations. Detailed characteristics of biventricular noncompaction (BiVNC) remain unknown. This study aimed to elucidate the clinical characteristics and genetic landscape of BiVNC.

Methods and results

We recruited children with left ventricular noncompaction from Japanese multi-institutional centers from 2013 to 2021. Left ventricular noncompaction was classified as BiVNC, congenital heart disease, arrhythmia, dilated cardiomyopathy, or normal function. In these patients, cardiomyopathy-associated genes were screened. A total of 234 patients (127 male; mean age, 4 months [range, 0-6.6 years]) were enrolled in this study, of whom 25 had BiVNC; 55, normal fu

SUBMITTER: Hirono K 

PROVIDER: S-EPMC11935668 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature