title: Sports Participation in Genotype Positive Children With Long QT Syndrome.
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:KCNQ1n = 60 (58%),KCNH2n = 36 (35%),SCN5An = 6 (6%),KCNE1n = 1 (1%), andKCNE2n = 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.
authors: Department of Pediatrics and Division of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland Clinic Lerner College of Medicine, Cleveland, Ohio.
authors: Peter F
authors: PF
authors: Aziz
authors: The Children's Hospital of Philadelphia, Division of Cardiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
authors: Tammy
authors: T
authors: Sweeten
authors: The Children's Hospital of Philadelphia, Division of Cardiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
authors: Ramon L
authors: RL
authors: Vogel
authors: The Children's Hospital of Philadelphia, Division of Cardiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
authors: William J
authors: WJ
authors: Bonney
authors: The Children's Hospital of Philadelphia, Division of Cardiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
authors: Jacqueline
authors: J
authors: Henderson
authors: University of California San Francisco, Pediatric Heart Center, San Francisco, California.
authors: Akash R
authors: AR
authors: Patel
authors: The Children's Hospital of Philadelphia, Division of Cardiology, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania.
authors: Maully J
authors: MJ
authors: Shah
keywords: cardiac arrest; long QT syndrome; pediatrics; sports participation