In patient athletes with genetic heart diseases including long QT syndrome, risk of sudden cardiac death may be managed through a return-to-play shared decision-making process, according to a study presented July 27 during the Heart Rhythm Society's annual meeting and simultaneously published in the Journal of the American College of Cardiology.

Kathryn E. Tobert, BA, et al., conducted a retrospective review of electronic medical records of 672 patient athletes with genetic heart diseases that predisposed them to disease-triggered sudden cardiac arrest. All patients were treated at the Mayo Clinic between July 2000 and July 2020 and were given return-to-play approval. There were 495 athletes with long QT syndrome and 58 had an ICD.

Results showed that over the 20-year study, there was no genetic heart disease-sports associated mortality. In addition, 29 patients had one or more breakthrough cardiac events associated with their long QT syndrome. Fifteen of these were athletes at the time, and three had a sports-related cardiac event. In the 177 patients with other genetic heart diseases, including hypertrophic cardiomyopathy and catecholaminergic polymorphic ventricular tachycardia, 14 nonlethal cardiac events happened during the return-to-play time. Overall, the event rate was 1.16 nonlethal events per 100 athlete-years of follow-up.

The authors explain that shared decision-making for a return to play has important steps that "are critical to the success of the process." This begins with thorough testing, a customized treatment plan, discussion of risks and a review of current guidelines for sports participation. If the athlete and parents are unanimous in their decision – when age-appropriate – return to play is allowed. Then coaches and school officials are informed and safety measures put in place, including having a personal automatic electronic defibrillator, avoiding QT-prolonging drugs in the case of long QT syndrome, maintaining proper hydration and electrolyte balance, and having annual follow-up consultations with their genetic cardiologist for risk reevaluation and treatment review.

"The results of implementing shared decision-making have been incredibly satisfying and in fact have reshaped the global conversation for athletes with a wide variety of genetic heart diseases," says Michael J. Ackerman, MD, PhD, FACC, author of the study. "Young people with genetic heart disease can grow up and dream big. Such athletes will be able to reach for the highest level of Olympic and professional sports."

However, Ackerman cautions that this approach is not a universal solution. For some athletes, the shared decision-making process culminates in a recommendation to stop their sport of choice.

"It has been a 'long and winding road' for those of us who care for patients with genetic heart disease including those with ICDs, and we have come far in the understanding and management of these conditions," explain Susan P. Etheridge, MD, FACC, and Elizabeth V. Saarel, MD, FACC, in a related editorial comment. "Data like those provided by Tobert et al., help us understand that once evaluated, appropriately monitored, and treated, most can return to play and hopefully live long and happy lives."

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

Keywords: Decision Making, Tachycardia, Ventricular, Death, Sudden, Cardiac, Sports, Athletes, Long QT Syndrome, Cardiomyopathy, Hypertrophic, Defibrillators

< Back to Listings