Congenital long QT syndrome guidelines ‘too restrictive’

By Piriya Mahendra

Athletes who choose to continue competing after being diagnosed with long QT syndrome (LQTS) have a low rate of LQTS-triggered cardiac events, US researchers report.

Of the 60 (46%) athletes with LQTS included in the study who continued to participate in sports contrary to European and Bethesda guideline recommendations, only one experienced sporting-related LQTS events: a 9-year-old boy with LQT1, extreme QT prolongation (QT interval [QTc] >550 ms), and a history of aborted cardiac arrest.

As reported in JAMA, he received two appropriate ventricular fibrillation-terminating implantable cardioverter-defibrillator (ICD) shocks while warming up before games. Each episode occurred in the setting of admitted beta-blocker nonadherence, emphasize Jonathan Johnson and Michael Ackerman, both from the Mayo Clinic in Rochester, Minnesota.

Of the 157 patients included in the study who were athletes at the time of their evaluation for LQTS at the Mayo Clinic, 27 (17%) chose to disqualify themselves from competitive sports after being diagnosed with the syndrome. This finding "debunks the myth" that the families of athletes are reluctant for them to stop competing, remarked Ackerman in a press statement.

The overall rate of events per athlete-year was 0.003, corresponding to one event in 331 athlete-years.

The 130 athletes who continued competing after being diagnosed with LQTS participated in a variety of sports and 49 (38%) participated in more than one sport. Overall, 32 (25%) athletes competed in high school, and eight (6%) competed at college, university, or a professional level.

The Bethesda guidelines recommend that patients with either symptoms, a corrected QTc greater than 470 ms (in men) and 480 ms (in women), or an ICD should not participate in most sports. The European guidelines are more stringent and state that athletes must be disqualified from all sports based on a QTc cutoff of more than 440 ms in men and 460 ms in women.

All participants received a 2-3-day clinical and genetic evaluation, including a 1-2-hour consultation with Ackerman. Patients who chose to continue competing after their diagnosis received counseling on athletic participation guidelines.

The decision to continue sports after counseling had to be agreed by the physician, patients, and both parents, depending on the patient's age. In addition to medical therapy, each athlete was provided with an automated external defibrillator and their school officials and coaches were notified.

"About 8 years ago after I started to see some of these lives ruined by the recommendation to discontinue sports, we decided to challenge the status quo," explained Ackerman.

"We adopted a philosophy that empowered patients and their families with the right to make an informed and difficult decision about continuing in competitive sports, a possible LQTS risk-taking behavior."

Although the findings suggest that the Bethesda and European guidelines may be too restrictive, Ackerman cautions that LQTS patients who want to remain athletes should seek treatment at a center of excellence.

"The patient must be evaluated, risk-stratified, treated and counseled carefully so that the athletes and their families can make an informed decision," he stresses.

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