Clinical Course, Risk Stratification And Response To Beta-Blockers In Patients With Long Qt Syndrome Type 3
Circulation(2017)
摘要
Background: Long QT syndrome type 3 (LQT3) represents a rare and lethal variant of long QT syndrome (LQTS) caused by gain-of-function mutations in the SCN5A gene. Objective: 1) To describe the clinical course of LQT3 patients; 2) To identify predictors of Life-threatening Arrhythmic Events (LAE, defined as aborted cardiac arrest, sudden cardiac death); 3) To test the efficacy of beta-blockers (BB) in preventing the occurrence of LAE in LQT3 patients. Methods: Cohort study of consecutive LQT3 patients. The clinical course was determined with the Kaplan-Meier method. Predictors of LAE were determined with Cox multivariable analysis. Efficacy of BB was assessed by comparing the rate of LAE during matched time intervals of a median of 45 months [IQR: 15-118] in 67 patients (43% males). Results: 171 LQT3 patients (52% males, age at first visit 28±21 years, QTc 492± 56ms) were followed up for 9±7 years: 13/171 (8%) patients suffered a first arrhythmic event at follow up (1% per year). Univariable analysis showe...
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关键词
Long QT syndrome, Beta-blocker, Arrhythmias, treatment of, Sudden cardiac death, Gene mutations
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