Some Young Athletes With Genetic Heart Disease Can Stay in the Game, With Appropriate Care

By Megan Brooks

July 30, 2021

NEW YORK (Reuters Health) - New data from the Mayo Clinic challenges the automatic benching of all athletes found to have a genetic heart disease (GHD) predisposing to sudden cardiac death, such as long QT syndrome (LQTS), in favor of the more contemporary shared decision-making approach, the researchers say.

In more than 2,000 person-years of follow-up, no sudden cardiac deaths related to sports happened in 494 young athletes with LQTS who were evaluated, risk stratified and approved for return to play at Mayo Clinic's Windland Smith Rice Genetic Heart Rhythm Clinic.

In email to Reuters Health, Dr. Michael Ackerman, genetic cardiologist and director of the clinic, said, "This study group represents the largest cohort of athletes ever followed at a single center with genetic heart diseases which would have resulted - in the past and still today - with a response of 'sorry kid, you are done.'"

At the Mayo Clinic, he said, "they have been met with an attitude of 'let's be on the same team and see if we can make this work.'"

The study findings were published in Journal of the American College of Cardiology to coincide with a presentation the Heart Rhythm Society annual meeting.

Seventy-nine of the 494 athletes with LQTS (16.0%) were symptomatic before diagnosis, and 58 (11.7%) had an implantable cardioverter-defibrillator (ICD).

The vast majority of the athletes (94.1%) have not experienced any LQTS-related breakthrough cardiac events (BCE).

Twenty-nine athletes (5.9%) had one or more BCE during follow-up. Of those, 15 (3.0%) experienced a BCE during their return to play period, with three (0.6%) having an event during exercise and 12 (2.4%) during non-sports-related activities.

Overall, athletes in the cohort had just over a 1% chance of having a nonlethal episode during sports each year, the researchers found.

Based in large part by Dr. Ackerman's team at the Mayo Clinic, cardiac society guidelines have begun to acknowledge shared decision making for the athlete with (GHDs), such LQTS, and the possibility for that athlete's return to play.

The return to play (RTP) protocol they developed offers an approach for cardiologists to risk stratify athletes with LQTS.

But Dr. Ackerman said, "Doctors must ask themselves if they are the physician best suited to work with these athletes, as these patients must be carefully evaluated and risk stratified so that a patient-specific therapy can be configured. Then and only then, a careful discussion can follow. This is not a quick 5-10 minute office visit, but instead can and does take hours," he noted.

The authors of an editorial say the Mayo Clinic data "reassure us that optimally treated athletes with a comprehensive 'return-to-play' plan can compete with a low rate of adverse events."

"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," write Dr. Susan Etheridge, with University of Utah in Salt Lake City and Dr. Elizabeth Saarel, with Cleveland Clinic Lerner College of Medicine in Ohio.

"Full competitive sports participation may not be the desire of every genetic heart disease patient and his or her family, and in some it may not be possible. Patients at risk for therapy noncompliance and other high-risk behaviors remain a concern, and restrictions may be needed. We continue to have trepidation about competitive swimming and diving in certain types of LQTS as well as catecholaminergic polymorphic ventricular tachycardia," the editorial writers say.

"But we are moving toward a more lenient and less paternalistic approach. Patients live long lives despite their genetic arrhythmia diagnoses. Data like those provided by (the Mayo Clinic) help us understand that once evaluated, appropriately monitored, and treated, most can return to play and hopefully live long and happy lives," they conclude.

This work was supported by the Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program and Mayo Clinic Center for Clinical and Translational Science through a grant from the National Center for Advancing Translational Sciences. Disclosures for authors are listed with the original article.

SOURCE: Journal of the American College of Cardiology, online July 27, 2021.