Medical Therapy for Long QT Syndrome.

Long QT syndrome (LQTS) is an arrhythmogenic disorder characterized by repolarization abnormalities with a tendency to cause life threatening cardiac events. The first manifestation of the syndrome may be sudden death, therefore, early diagnosis and therapy is of great importance. LQTS can be congenital or acquired. The latter is most commonly seen in hospitalized patients and such individuals have an easily recognizable and reversible precipitating factor (electrolyte disturbances, certain drugs etc.). Congenital LQTS, a less prevalent condition, is considered to be part of the greater family of disorders called channelopathies. Extensive research over the past decade allowed better understanding of the underlying molecular mechanisms and unmasking of the genetic substrate of these patients. Both pharmacological and interventional therapies (left cardiac sympathetic denervation, ICDs) have been used in the treatment of congenital long-QT syndrome. This review will focus solely on the pharmacologic therapy of this potentially lethal inherited disorder summarizing current knowledge regarding b-blocker use, taking into account novel and more targeted approaches such as gene-specific therapy.