Cardiac Channelopathies in the Pediatric Patient: Long QT Syndrome.

Congenital long QT syndrome is the most common inherited arrhythmia syndrome and is characterized by prolongation of the QT interval in the absence of structural heart disease or external factors. Disease will often manifest during childhood resulting in life-threatening arrhythmias or sudden cardiac death. Diagnosis involves detailed personal and family history in combination with clinical testing. Genetic testing has a high diagnostic yield, though a negative result does not rule out the presence of disease. The incidence of arrhythmia events is significantly decreased with trigger avoidance and appropriate medical therapy allowing sports participation in the majority of patients.