Pharmacological and non-pharmacological management of the XXXX : the rationale.

@entity1 @entity1164 @entity1169 @entity944 @entity1457 @entity1163 @entity737 @entity74 @entity5894 @entity299 @entity1162 @entity1167 @entity943 @entity884
The @entity1162 (LQTS) is a @entity74 characterized by a prolongation of the QT interval on the ECG and occurrence of life-threatening @entity943 especially, but not only, under conditions of increased sympathetic activity. Symptomatic untreated @entity1 are at high risk for @entity1164 . Twelve LQTS genes have been identified and most of them encode cardiac ion channels. Very effective therapies are available and in carefully treated @entity1 mortality is around 0.5-1% over 20 years. The initial treatment should always involve b-blockers, with @entity1169 and @entity5894 being the two most effective ones. With few exceptions all mutation carriers should be treated because of the risk of @entity944 during the first @entity299 . Approximately 20% of @entity1 continue to have @entity1163 despite the b-blockers and the most rationale next level of therapy is represented by Left Cardiac Sympathetic Denervation (LCSD), which is highly effective and can complement any other therapy. One important limitation of LCSD is that, without valid reasons, it is available only in a few selected centers. Whenever @entity1163 recurs despite LCSD, or whenever an aborted @entity1167 has occurred, it becomes logical to resort to the @entity737 ( @entity737 ). The latter, however, is burdened by a high rate (31%) of adverse events including severe ones such as @entity884 , @entity1457 , and by the need of frequent battery replacements. A scoring system, based on simple clinical variables, can identify the @entity1 more and less likely to benefit from @entity737 implantation.
