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. 2015 Mar-Apr;1(1-2):62-70.
doi: 10.1016/j.jacep.2015.03.006.

Sports Participation in Genotype Positive Children With Long QT Syndrome

Peter F Aziz  1 , Tammy Sweeten  2 , Ramon L Vogel  2 , William J Bonney  2 , Jacqueline Henderson  2 , Akash R Patel  3 , Maully J Shah  2
Affiliations

Sports Participation in Genotype Positive Children With Long QT Syndrome

Peter F Aziz et al. JACC Clin Electrophysiol. 2015 Mar-Apr.

Abstract

Objective: The study sought to examine the prevalence and outcomes of sports participation (both competitive and recreational) in our single-center LQTS genotype positive pediatric population.

Background: The risks of sports participation in patients with long QT syndrome (LQTS) are not clearly elucidated.

Methods: A retrospective review was performed on genotype positive patients referred for the evaluation and management of LQTS between 1998 and 2013 at the Children's Hospital of Philadelphia. Pediatric patients participating in competitive or recreational sports were included in the analysis and their charts were reviewed for documented LQTS events during follow-up.

Results: The cohort of genotype-positive LQTS patients included 212 patients, and 103 patients (49%, female n = 53, average follow-up 7.1 ± 4.0 years, average QTc 468 ± 42 ms) participated in sports. A total of 105 LQTS disease-causing mutations were identified: KCNQ1 n = 60 (58%), KCNH2 n = 36 (35%), SCN5A n = 6 (6%), KCNE1 n = 1 (1%), and KCNE2 n = 2 (2%). All patients were treated with beta-blockade, with noncompliance in 1 patient and intolerance in 1 patient. Twenty-six patients participated in competitive sports (26%, female n = 15, average follow-up 6.9 ± 4.1 years, average QTc 461 ± 35 ms). Seventy-seven patients (75%, female n = 35, average follow-up 7.3 ± 3.9 years, average QTc 470 ± 43 ms) participated in recreational sports. No patients had LQTS symptoms during sports participation. Five appropriate implantable cardioverter-defibrillator shocks occurred in 2 patients, though none were related to sports participation.

Conclusions: In this series no cardiac events and no deaths were observed in treatment-compliant LQTS children while participating in sports in 755 patient-years of follow-up.

Keywords: cardiac arrest; long QT syndrome; pediatrics; sports participation.

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Figures

FIGURE 1
FIGURE 1. Athlete Presentation
Athlete presentation is categorized for all sports participants. The majority of patients were identified through familial electrocardiogram (ECG) screening. LQTS = long QT syndrome.
FIGURE 2
FIGURE 2. Competitive Sports Participation
A total of 39 sports are depicted by type in this pie chart. The most frequently participated sports were basketball, baseball, soccer, and field hockey.
FIGURE 3
FIGURE 3. Bethesda Classification (Competitive Athletes as Well as LQT1 Subtype)
(A) Sports classified in accordance with the Bethesda Guidelines. Increasing static component (I–III) is depicted on the y-axis and increasing dynamic component (A–C) is depicted on the x-axis. (B) Sports classified for long QT1 (LQT1) patients in accordance with Bethesda guidelines. This figure shows a proportionate distribution of LQT1 athletes compared to Figure 2.
FIGURE 4
FIGURE 4. Recreational Sports Participation
A total of 133 sports are depicted by type in this pie chart.
See this image and copyright information in PMC

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