Phenotypic Manifestations of Arrhythmogenic Cardiomyopathy in Children and Adolescents.

ABSTRACT:

Background

Arrhythmogenic cardiomyopathy (ACM) is a variably penetrant disease increasingly identified in young patients.

Objectives

This study sought to describe the diverse phenotype, genotype, and outcomes in pediatric and adolescent patients.

Methods

Records from 1999 to 2016 were reviewed for individuals age <21 years with a consistent personal or family history. Patients were categorized by right ventricular (RV), left dominant (LD), or biventricular subtypes using 2010 Task Force Criteria or proposed features of LD disease, encompassing electrocardiographic, structural, histological, and arrhythmic characteristics. Genetic variants classified as pathogenic and/or likely pathogenic by 2015 American College of Medical Genetics and Genomics criteria in recognized

SUBMITTER: DeWitt ES 

PROVIDER: S-EPMC7261020 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature