[The treatment of the long QT syndrome]

General principles of long QT syndrome (LQTS) management are presented first. The management of pause-dependent LQTS includes the following steps: A. recognition of torsades de pointes type of ventricular tachycardia and of the ecg warning signs, B. termination of the arrhythmia and the ecg warning signs by interventions which do not shorten the QT interval and by interventions which also shorten the QT interval. The management of adrenergic-dependent LQTS includes the following steps: A. individual risk stratification, B. avoidance of deleterious sympathetic stress, C. recognition of the arrhythmia and of the ecg warning signs, D. termination of the arrhythmia, E. prevention of relapses using beta-blockers, high left cervico-thoracic sympathectomy, permanent pacemakers and automatic implantable cardioverter-defibrillators.