Short QT and atrial fibrillation: A KCNQ1 mutation–specific disease. Late follow-up in three unrelated children

Abstract

Short QT syndrome (SQTS) is an inherited, rare, potentially lethal disease characterized by ventricular repolarization alterations, predisposing individuals to atrial fibrillation (AF), syncope, and high incidence of sudden cardiac death (SCD), often during the first years of life.1,2 SQTS was first described in a family in which some members showed an abnormally short QT interval and 1 of them suffered from AF. An additional patient had a short QT interval, recurrent ventricular arrhythmias, and, ultimately, SCD.