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
Keywords: Beta blockers, channellopaties, long QT syndrome, therapy, pharmacology, Ventricular arrhythmias.
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