Channelopathy in athletes

Channelopathies are primary inherited electrical disorders (prevalence 2–4%). Long QT syndrome (LQTS), short QT syndrome (SQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and Brugada syndrome (BrS) predispose to life-threatening ventricular arrhythmia (VT/VF). Several triggers can provoke VT/VF, such as exercise (LQTS,CPVT), vagotonia (BrS), hyperthermia (LQTS and BrS), immersion in cold water (LQTS), or electrolyte/volume depletion. It is a challenge for the screening physician to recognize the signature ECG changes of channelopathy at rest (LQTS, SQTS, BrS) or during exercise (CPVT) to identify the disease and assess the risk of VT/VF. Eligibility decision-making should involve cardiological expertise in channelopathy and determination of phenotypic and genotypic expression, and is not solely based on the assumption that channelopathies induce VT/VF. This chapter discusses the clinical presentation, management, and risk assessment in athletes of the four major channelopathies (LQTS, SQTS, CPVT, and BrS).