Cardiology in review
-
Cardiology in review · Jul 2004
ReviewCongenital and acquired long QT syndrome. Current concepts and management.
Congenital long QT syndrome (LQTS) is a rare but potentially lethal disease, characterized by prolongation of QT interval, recurrent syncope, and sudden death. In the pregenomic era (1959-1991), sympathetic imbalance was thought to be responsible for this disease. Since 1991 (postgenomic era), 7 LQTS genes have been discovered and more than 300 mutations have been identified to account for approximately 70% of patients affected. ⋯ For patients with a history of drug-induced LQTS, care must be taken to avoid further exposure to QT-prolonging drugs or conditions. Molecular genetic analysis could be useful to unravel subclinical mutations or polymorphisms. Physicians not only need to be aware of the pharmacodynamic and pharmacokinetic interactions of various important drugs, but also need to update their knowledge.