Primary prevention with the implantable cardioverter-defibrillator in high-risk long-QT syndrome patients.

ABSTRACT: Aims:Prospective data regarding the role of implantable cardioverter-defibrillator (ICD) for the primary prevention of sudden cardiac death in patients with long QT syndrome (LQTS) is scarce. Herein, we explore the prospective Rochester LQTS ICD registry to assess the risk for appropriate shock in primary prevention in a real-world setting. Methods and results:We studied 212 LQTS patients that had ICD implantation for primary prevention. Best-subsets proportional-hazards regression analysis was used to identify clinical variables that were associated with the first appropriate shock. Conditional models of Prentice, Williams, and Peterson were utilized for the analysis of recurrent appropriate shocks. During a median follow-up of 9.2?±?4.9?years, there were 42 patients who experienced at least one appropriate shock and the cumulative probability of appropriate shock at 8?years was 22%. QTc???550 ms [hazard ratio (HR) 3.94, confidence interval (CI) 2.08-7.46; P?

SUBMITTER: Biton Y 

PROVIDER: S-EPMC6365807 | biostudies-literature | 2019 Feb

REPOSITORIES: biostudies-literature