Return-to-Play for Athletes With LQTS or Genetic Heart Diseases

Quick Takes

  • Genetic heart diseases (GHDs), including long QT syndrome, are common causes of sudden cardiac death in young athletes.
  • Historically, guidelines from major cardiology societies have recommended restriction from sports in most cases with GHDs.
  • The findings in this 20-year observational study support recent momentum for more shared decision making in return-to-play decisions.

Study Questions:

What are the prevalence and outcomes of athletes with sudden cardiac death–predisposing genetic heart diseases (GHDs), particularly long QT syndrome (LQTS), after their return to play (RTP)?

Methods:

This was a single-center (Mayo Clinic, Dr. Michael Ackerman), retrospective review of the electronic medical records of all treated athletes with GHD over a 20-year period. The RTP protocol and shared decision making (SDM) has been described previously and includes: 1) appropriate risk stratification and consultation appropriate for the GHD, 2) development of an optimized treatment plan, 3) evidence-based discussion of risks of RTP, 4) consensus decision on RTP by athlete (and parents if appropriate) and physician, 5) informing of stakeholders (e.g., coaches, school, team physicians) of condition and decision, 6) obtaining a personal automatic external defibrillator to be present at all times, and 7) plan for regular follow-up.

Results:

Out of 672 athletes with GHD overall, 494 athletes had LQTS (46.8% female); mean age at diagnosis was 14.8 ± 10.5 years; mean follow-up 4.2 ± 4.8 years; 79 of 494 (16%) athletes with LQTS were symptomatic before diagnosis, and 58 (11.7%) had an implantable cardioverter-defibrillator. In this cohort, encompassing 2,056 total follow-up years, there was no GHD–sports-associated mortality. LQTS-associated breakthrough cardiac events (BCE) occurred in 29 patients (5.9%); of those, 15 (3.0%) were athletes at the time of the BCE, and 3 (0.6%) experienced a sports-related BCE. The overall event rate was 1.16 nonlethal events per 100 years of follow-up.

Conclusions:

This single-center’s contemporary SDM approach to RTP resulted in a low risk of cardiac events in athletes with LQTS; the findings support continued challenge to the frequent disqualification for all athletes with LQTS.

Perspective:

The unique experience and expertise described in this extensive observational study will not be easily replicated; nevertheless, the results should encourage more permissive SDM-based approaches to RTP in patients with GHD. Indeed, in the last several years, the major cardiac society guidelines have increasingly recognized the importance of SDM in RTP discussions. The 2020 American Heart Association/American College of Cardiology guideline for the management of patients with hypertrophic cardiomyopathy is the most recent example. The care team should include cardiologists with sufficient expertise in caring for athletes and GHD.

Two related topics of interest but not discussed in this paper are patients who: 1) were not permitted to play by certain stakeholders (e.g., team, school) despite the thorough evaluation and planning described, and 2) were referred for GHD but found to be misclassified or misdiagnosed.

Clinical Topics: Arrhythmias and Clinical EP, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Prevention, Sports and Exercise Cardiology, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Sports and Exercise and Congenital Heart Disease and Pediatric Cardiology

Keywords: Arrhythmias, Cardiac, Athletes, Cardiomyopathy, Hypertrophic, Death, Sudden, Cardiac, Decision Making, Defibrillators, Implantable, Genetics, Long QT Syndrome, Risk Assessment, Secondary Prevention, Sports


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