Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience.

ABSTRACT:

Background and objectives

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVT-causative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.

Methods

We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated.

Results

RYR2 variants were identified in 17 patients, and 14 were classified as pathogenic or l

SUBMITTER: Lee J 

PROVIDER: S-EPMC11685342 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature