The Long QT Syndrome

The primary form of long QT syndrome (LQTS) is an infrequently occuring familial disorder in which affected members have electrocardiographic QT interval prolongation and a predisposition to syncope the fatal ventricular arrhythmias. The review includes discussions of inheritance, clinical presentation, diagnosis, and treatment of LQTS. At present, there are four madalities of treatment for LQTS patients β-blockers, pacemakers, left cervicothoracic sympathetic ganglionectomy, and implanted defibrillators. Because the clinical course of LQTS is quiet variable, therapy must be individualized for each patient.