Meng Wang, Derick R Peterson, Eleonora Pagan, Vincenzo Bagnardi, Andrea Mazzanti, Scott McNitt, David Q Rich, Christopher L Seplaki, Valentina Kutyifa, Bronislava Polonsky, Alon Barsheshet, Deni Kukavica, Spencer Rosero, Ilan Goldenberg, Silvia Priori, Wojciech Zareba
Background: Risk stratification in long QT syndrome (LQTS) patients is important for optimizing patient care and informing clinical decision making. We developed a risk prediction algorithm with prediction of 5-year absolute risk of the first life-threatening arrhythmic event [defined as aborted cardiac arrest, sudden cardiac death, or appropriate implantable cardioverter defibrillator (ICD) shock] in LQTS patients, accounting for individual risk factors and their changes over time. Methods: Rochester-based LQTS Registry included the phenotypic cohort consisting of 1,509 LQTS patients with a QTc ≥ 470 ms, and the genotypic cohort including 1,288 patients with single LQT1, LQT2, or LQT3 mutation...
2022: Frontiers in Cardiovascular Medicine