Long QT syndrome: diagnosis and management.

BACKGROUND: Long QT syndrome (LQT) is characterized by prolongation of the QT interval, causing torsade de pointes and sudden cardiac death. The LQT is a disorder of cardiac repolarization caused by alterations in the transmembrane potassium and sodium currents. Congenital LQT is a disease of transmembrane ion-channel proteins. Six genetic loci of the disease have been identified. Sporadic cases of the disease occur as a result of spontaneous mutations. The acquired causes of LQT include drugs, electrolyte imbalance, marked bradycardia, cocaine, organophosphorus compounds, subarachnoid hemorrhage, myocardial ischemia, protein sparing fasting, autonomic neuropathy, and human immunodeficiency virus disease.

METHODS: Data on the diagnosis and management of LQT were thoroughly reviewed.

RESULTS AND CONCLUSIONS: The diagnosis of LQT primarily rests on clinical and electrocardiographic features and family history. The clinical presentations range from dizziness to syncope and sudden death. Genetic screening is available primarily as a research tool. Short-term treatment of LQT is aimed at preventing the recurrences of torsades and includes intravenous magnesium and potassium administration, temporary cardiac pacing, withdrawal of the offending agent, correction of electrolyte imbalance, and, rarely, intravenous isoproterenol administration. The long-term treatment is aimed at reducing the QT-interval duration and preventing the torsades and sudden death and includes use of oral beta-adrenergic blockers, implantation of permanent pacemaker/cardioverter-defibrillator, and left thoracic sympathectomy. Sodium channel blockers are promising agents under investigation. Electrocardiograms are recorded for screening of family members. The data favor treating asymptomatic patients, if <40 years old at the time of diagnosis, with beta-adrenergic blockers.