April 21, 2015
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Sports participation appears safer today for children with long QT syndrome

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A single-center review of children and adolescents with long QT syndrome yielded no deaths or cardiac events during sports participation.

The findings could mean that sports participation is safer for these children than previously thought, Peter Aziz, MD, and colleagues wrote in JACC: Clinical Electrophysiology.

Peter Aziz, MD

Peter Aziz

“Re-examining participation rules is important because of the physiologic benefits of exercise at all ages have been emphasized repeatedly and promoted as a national public health agenda,” Aziz, from the department of pediatrics and division of pediatric cardiology at Cleveland Clinic, said in a press release. “The enhanced self-confidence, sense of psychological, physical and social well-being, and improved overall quality of life that sports participation brings to children and adolescents is equally important.”

The researchers performed a retrospective review of genotype-positive patients referred for evaluation and management of long QT syndrome at The Children’s Hospital of Philadelphia from 1998 to 2013. The analysis included 212 patients, 103 of whom participated in sports (53 girls; mean follow-up, 7.1 years; mean QTc, 468 ms).

In total, the researchers identified 105 mutations known to cause long QT syndrome. The most common mutation was KCNQ1 (58%), followed by KCNH2 (35%), SCN5A (6%), KCNE2 (2%) and KCNE1 (1%). All patients were treated with beta-blockade. One patient was noncompliant and one was intolerant to beta-blockade.

Twenty-six patients participated in competitive sports (15 girls; mean follow-up 6.9 years; mean QTc, 461 ms) and 77 patients participated in recreational sports (35 girls; mean follow-up 7.3 years; mean QTc, 470 ms).

During 755 patient-years of follow-up, no patients had long QT syndrome symptoms during sports participation, according to the researchers. Five appropriate implantable cardioverter-defibrillator shocks occurred in two patients, but none were related to sports participation.

“Given the changing diagnostic trends, it may be clinically beneficial to re-examine sports recommendations that were based on data from an earlier era,” Aziz said. “In addition to taking medications regularly and openly communicating with doctors, we recommend long QT syndrome patients who do not have an ICD always have access to an automated external defibrillator. Many of our patients have purchased personal AEDs that they carry around with them.”

Michael J. Ackerman, MD, PhD

Michael J. Ackerman

In a related editorial, Michael J. Ackerman, MD, PhD, from Windland Smith Rice Cardiovascular Genomics Research Laboratory at Mayo Clinic, wrote that “aerobic activity is a potential risk-taking behavior for patients with [long QT syndrome] in general, type 1 [long QT syndrome] in particular. … The question is whether that increased risk can be circumvented, neutralized or minimized in other ways besides elimination of that specific risk-taking behavior by its removal (ie, sports disqualification). We have concluded that the answer is unequivocally yes, and so have Aziz et al. However, in tight linkage disequilibrium is the need for a robust diagnostic, prognostic and therapeutic program that is tailored exquisitely to each patient.” – by Erik Swain

Disclosures: One researcher reports receiving research grant support from Medtronic. Ackerman reports consulting for Boston Scientific, Gilead Sciences, Medtronic and St. Jude Medical and receiving royalties from Transgenomic.