• Carla Spazzolini, Jamie Mullally, Arthur J Moss, Peter J Schwartz, Scott McNitt, Gregory Ouellet, Thomas Fugate, Ilan Goldenberg, Christian Jons, Wojciech Zareba, Jennifer L Robinson, Michael J Ackerman, Jesaia Benhorin, Lia Crotti, Elizabeth S Kaufman, Emanuela H Locati, Ming Qi, Carlo Napolitano, Silvia G Priori, Jeffrey A Towbin, and G Michael Vincent.
• Section of Cardiology and Department of Cardiology and Molecular Cardiology Laboratory, Fondazione Policinico S. Matteo IRCCS and the University of Pavia, Pavia, Italy.
• J. Am. Coll. Cardiol. 2009 Aug 25;54(9):832-7.

ObjectivesThis study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy).BackgroundThe clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously.MethodsThe study population of 3,323 patients with QT interval corrected for heart rate (QTc) > or =450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life.ResultsThe risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc > or =500 ms, heart rate < or =100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p < 0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy.ConclusionsPatients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset.2009 by the American College of Cardiology Foundation

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