congenital long-QT syndrome

congenital long-QT syndrome

A rare autosomal dominant heart disorder causing prolongation of the QT interval in the ECG with ventricular arrhythmias, syncope, and the risk of sudden death. The most common forms, types 1 and 2, are caused by mutations respectively in the potassium channel genes KCNQ1 and KCNH2. Alleles for the syndrome are transmitted to daughters more often than to sons.