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QUARTERLY FOCUS ISSUE: HEART RHYTHM DISORDER: CLINICAL RESEARCH

Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy

Carla Spazzolini, DVM^_*, Jamie Mullally, BS, Arthur J. Moss, MD^,_*, Peter J. Schwartz, MD^_*,, Scott McNitt, MS, Gregory Ouellet, BS, Thomas Fugate, BS, Ilan Goldenberg, MD, Christian Jons, MD, Wojciech Zareba, MD, PhD, Jennifer L. Robinson, MS, Michael J. Ackerman, MD, PhD^||, Jesaia Benhorin, MD^¶, Lia Crotti, MD, PhD^_*, Elizabeth S. Kaufman, MD^#, Emanuela H. Locati, MD, PhD^_**, Ming Qi, PhD, Carlo Napolitano, MD, Silvia G. Priori, MD, PhD, Jeffrey A. Towbin, MD and G. Michael Vincent, MD

^_* Section of Cardiology and Department of Cardiology and Molecular Cardiology Laboratory, Fondazione Policinico S. Matteo IRCCS and the University of Pavia, Pavia, Italy
Heart Research Follow-up Program, Cardiology Division, Department of Medicine, University of Rochester Medical Center, Rochester, New York
Department of Pathology, University of Rochester Medical Center, Rochester, New York
Laboratory of Cardiovascular Genetics, IRCCS Istituto Auxologico, Milan, Italy
^|| Department of Medicine, Pediatrics, and Molecular Pharmacology, Mayo Clinic College of Medicine, Rochester, Minnesota
^¶ Bikur Cholim Hospital, University of Jerusalem, Jerusalem, Israel
^# The Heart and Vascular Research Center, MetroHealth Campus, Case Western Reserve University, Cleveland, Ohio
^_** Cardiovascular Department De Gasperis, Niguarda Hospital, Milan, Italy
Molecular Cardiology, Fondazione S. Maugeri-University of Pavia, Pavia, Italy
Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
Department of Medicine, University of Utah School of Medicine, Salt Lake City, Utah

Manuscript received March 9, 2009; revised manuscript received May 13, 2009, accepted May 17, 2009.

^_* Reprint requests and correspondence: Dr. Arthur J. Moss, Heart Research Follow-up Program, Box 653 University of Rochester Medical Center, Rochester, New York 14642 (Email: heartajm{at}heart.rochester.edu).

Objectives: This 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).

Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously.

Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) 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.

Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc 500 ms, heart rate 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.

Conclusions: Patients 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.

Key Words: genetics • infants • long QT syndrome • risk stratification

Abbreviations and Acronyms   ACA = aborted cardiac arrest   ECG = electrocardiogram   HR = hazard ratio   LQTS = long QT syndrome   QTc = QT interval corrected for heart rate   SCD = sudden cardiac death   SIDS = sudden infant death syndrome

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