Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy.

ABSTRACT:

Aims

This study aimed to explore the incidence of severe cardiac events in paediatric arrhythmogenic right ventricular cardiomyopathy (ARVC) patients and ARVC penetrance in paediatric relatives. Furthermore, the phenotype in childhood-onset ARVC was described.

Methods

Consecutive ARVC paediatric patients and genotype positive relatives ≤18 years of age were followed with electrocardiographic, structural, and arrhythmic characteristics according to the 2010 revised Task Force Criteria. Penetrance of ARVC disease was defined as fulfilling definite ARVC criteria and severe cardiac events were defined as cardiac death, heart transplantation (HTx) or severe ventricular arrhythmias. Childhood-onset disease was defined as meeting definite ARVC criteria ≤12 years of age.

Results<

SUBMITTER: Smedsrud MK 

PROVIDER: S-EPMC9712025 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature