Management of individuals with long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy may involve exercise restriction and/or β-blocker therapy.

This study assessed the practices of a group of paediatric electrophysiologists regarding the management of genotype-positive/phenotype-positive and genotype-positive/phenotype-negative individuals with these conditions.

An online survey was circulated to members of the Pediatric and Congenital Electrophysiology Society in May, 2014. The survey included questions addressing the respondents’ approach regarding exercise recommendations and prescription of β-blocker therapy.

A total of 45 cardiologists completed the survey. The majority of respondents restricted symptomatic patients from competitive sports; however, only approximately half restricted phenotype-negative mutation carriers from this level of activity. Recommendations were less consistent regarding other types of activities. A trend was identified regarding physician physical activity and exercise recommendations for phenotype-negative mutation carriers. Less-active physicians were more likely to restrict exercise. β-blocker therapy was discussed by the majority of respondents for symptomatic patients and a significant number of asymptomatic patients.

Exercise restriction for patients with long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy varies based on several factors including phenotype, type of exercise, guidelines referred to, and physicians’ own level of activity.