Contemporary Outcomes in Patients With Long QT Syndrome.

Background: Long QT syndrome (LQTS) is a potentially lethal cardiac channelopathy with a 1% to 5% annual risk of LQTS-triggered syncope, aborted cardiac arrest, or sudden cardiac death.

Objectives: This study sought to evaluate LQTS outcomes from a single center in the contemporary era.

Methods: The authors conducted a retrospective study comprising the 606 patients with LQTS (LQT1 in 47%, LQT2 in 34%, and LQT3 in 9%) who were evaluated in Mayo Clinic's Genetic Heart Rhythm Clinic from January 1999 to December 2015.

Effects on repolarization using dynamic QT interval monitoring in long-QT patients following left cardiac sympathetic denervation.

Background: Videoscopic left cardiac sympathetic denervation (LCSD) is an adjunct therapy for reduction of arrhythmia-induced events in patients with long-QT syndrome (LQTS). LCSD reduces LQTS-triggered breakthrough cardiac events. The temporal effects of QTc changes post-LCSD have not been studied.

Left cardiac sympathetic denervation in long QT syndrome: analysis of therapeutic nonresponders.

Background: Long QT syndrome (LQTS) is a potentially lethal but highly treatable cardiac channelopathy. Treatment options include pharmacotherapy, device therapy, and left cardiac sympathetic denervation (LCSD). Here, we sought to determine the characteristics of LQTS patients who have had ≥1 LQTS-related breakthrough cardiac event (BCE) after LCSD.