Author + information
- Received October 4, 2012
- Revision received November 29, 2012
- Accepted December 11, 2012
- Published online March 19, 2013.
- Juan Villafañe, MD⁎,⁎ (, )
- Joseph Atallah, MD, CM, SM†,
- Michael H. Gollob, MD‡,
- Philippe Maury, MD§,
- Christian Wolpert, MD∥,
- Roman Gebauer, MD¶,
- Hiroshi Watanabe, MD, PhD#,
- Minoru Horie, MD⁎⁎,
- Olli Anttonen, MD, PhD††,
- Prince Kannankeril, MD‡‡,
- Brett Faulknier, DO§§,
- Jorge Bleiz, MD∥∥,
- Takeru Makiyama, MD, PhD¶¶,
- Wataru Shimizu, MD, PhD##,
- Robert M. Hamilton, MD⁎⁎⁎ and
- Ming-Lon Young, MD, MPH†††
- ↵⁎Reprints requests and correspondence:
Dr. Juan Villafañe, Department of Pediatrics (Cardiology), University of Kentucky, 743 East Broadway, No. 300, Louisville, Kentucky 40202
Objectives The purpose of this study was to define the clinical characteristics and long-term follow-up of pediatric patients with short QT syndrome (SQTS).
Background SQTS is associated with sudden cardiac death. The clinical characteristics and long-term prognosis in young patients have not been reported.
Methods This was an international case series involving 15 centers. Patients were analyzed for electrocardiography characteristics, genotype, clinical events, Gollob score, and efficacy of medical or defibrillator (implantable cardioverter-defibrillator [ICD]) therapy. To assess the possible prognostic value of the Gollob score, we devised a modified Gollob score that excluded clinical events from the original score.
Results Twenty-five patients 21 years of age or younger (84% males, median age: 15 years, interquartile range: 9 to 18 years) were followed up for 5.9 years (interquartile range: 4 to 7.1 years). Median corrected QT interval for heart rate was 312 ms (range: 194 to 355 ms). Symptoms occurred in 14 (56%) of 25 patients and included aborted sudden cardiac death in 6 patients (24%) and syncope in 4 patients (16%). Arrhythmias were common and included atrial fibrillation (n = 4), ventricular fibrillation (n = 6), supraventricular tachycardia (n = 1), and polymorphic ventricular tachycardia (n = 1). Sixteen patients (84%) had a familial or personal history of cardiac arrest. A gene mutation associated with SQTS was identified in 5 (24%) of 21 probands. Symptomatic patients had a higher median modified Gollob score (excluding points for clinical events) compared with asymptomatic patients (5 vs. 4, p = 0.044). Ten patients received medical treatment, mainly with quinidine. Eleven of 25 index cases underwent ICD implantation. Two patients had appropriate ICD shocks. Inappropriate ICD shocks were observed in 64% of patients.
Conclusions SQTS is associated with aborted sudden cardiac death among the pediatric population. Asymptomatic patients with a Gollob score of <5 remained event free, except for an isolated episode of supraventricular tachycardia, over an average 6-year follow-up. A higher modified Gollob score of 5 or more was associated with the likelihood of clinical events. Young SQTS patients have a high rate of inappropriate ICD shocks.
Dr. Wolpert has received speaker honoraria from Medtronic, St. Jude Medical, Bard, Inc., and AstraZeneca; and serves on the advisory board for Sorin. Dr. Faulknier receives research support from Medtronic; serves on a steering committee for St. Jude Medical Research; and is a speaker for Cardionet. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received October 4, 2012.
- Revision received November 29, 2012.
- Accepted December 11, 2012.
- American College of Cardiology Foundation