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Inherited ion channelopathies have come to the forefront as a significant cause of sudden cardiac death (SCD) in pediatric patients with structurally normal hearts. Implantable cardioverter-defibrillator (ICD) placement can be a life-saving primary preventative therapy, but because of actors inherent in the pediatric population, careful thought must be given to the specific indications for placement in each patient. The most common inherited ion channelopathies are long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. All have the potential to cause SCD. However, thanks to current research, more is now known about the range of phenotypes present within each disorder and also the benefits that medical therapy can provide. Risk stratification can allow clinicians to best predict which patients may most benefit from a primary preventative ICD while at the same time avoid placement in the larger group who may remain asymptomatic with the aid of medical therapy or even simply observation.

the journal of innovations in cardiac rhythm management

Assessing Candidacy for Primary Preventative Implantable Cardioverter-defibrillators in Pediatric Patients with Ion Channelopathies: Weighing the Risks and Benefits