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Journal of the American College of Cardiology: Clinical Electrophysiology, Dr Rajay Narain

Clinical Articles, Lead Article

Rethink on sports restrictions for children with arrhythmia

Sports participation may be safer than previously thought for children with the heart rhythm disorder long QT syndrome, and restrictions should be eased to allow appropriately treated children with the condition to participate more, according to a study published recently in JACC: Clinical Electrophysiology.1

The authors reviewed the records of 103 patients, ages 4 to 21, who were seen at Children’s Hospital of Philadelphia between 1998 and 2003 for symptoms of long QT syndrome that occurred during sports participation to determine the prevalence of cardiac events or deaths. All patients were treated with beta-blockers, though one patient quit taking the medication and another could not tolerate it.

26 patients in the study participated in competitive sports and 77 participated in recreational sports. The patients were followed for an average of seven years and no cardiac events or deaths were observed during sports participation in treated patients. Five appropriate implantable cardioverter defibrillator (ICD) shocks occurred in two patients, but none were related to sports participation.

Dr Peter Aziz (Cleveland Children’s Clinic)

Dr Peter Aziz (Cleveland Children’s Clinic)

“Re-examining participation rules is important because the physiologic benefits of exercise at all ages have been emphasized repeatedly and promoted as a national public health agenda,” said lead author Dr Peter Aziz (Cleveland Children’s Clinic, Cleveland). “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.”

“Given the changing diagnostic trends, it may be clinically beneficial to re-examine sports recommendations that were made based on data from an earlier era,” Dr 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.”

Researchers said their patient population skewed toward asymptomatic patients, but this better reflects the general population as many asymptomatic patients are diagnosed with long QT syndrome through liberal electrocardiogram (ECG) testing and genetic screening. Limitations include the study being limited to a single institution; majority of patients being engaged in recreational sports and less physically demanding competitive sports, which may reflect a lower level of exertion; and only including patients who were actively participating in sports, which may show some survival or selection bias.

UK clinician comment on congenital long QT syndrome

Dr Rajay Narain (St Georges University Hospital, London)

Dr Rajay Narain (St Georges University Hospital, London)

Speaking to BJC Arrhythmia Watch, Dr Rajay Narain (St Georges University, London) provides a cardiologist’s perspective on the study:

Congenital long QT syndrome (LOTS) is characterised by a heterogeneous group of ion channel diseases that have a propensity to polymorphic ventricular tachycardia or ventricular fibrillation during adrenergic surges. The overall risk of having a cardiac event in patients with LQTS is dependent on the specific genotype and mutation involved, the age and gender of the patient and degree of abnormal repolarisation.2 The adverse event range ranges from 0.2%–0.8% per annum and is highest among peripubertal male & adolescent/young adult females.

The diagnosis of LQTS in young athlete can be challenging due to slow heart rate, sinus arrhythmia and T U wave configuration which affects the interpretation of the QT interval. In general QT >470 in males and >480 in females is considered abnormal. However, the diagnosis of LQTS should not be based on the QT internal alone, although a QTc of more than 500 ms is highly suggestive of the diagnosis. Athletes with borderline QT lengthening should be evaluated with supine and standing ECG recordings, exercise testing and 24-h Holter monitoring. Given the genetic nature of the condition, familial evaluation is advised both for diagnostic and cascade screening purposes.

More recent publications have suggested that affected individuals with mild phenotype may be able to engage in competitive sports with a low risk of cardiac events. The study included a relatively low number of athletes and a small number of professional athletes competing at elite level. Until further data are available, taking into consideration the devastating impact of SCD in the athletic field, congenital long QT syndrome remains a contraindication for competitive sports, except those with low intensity (class 1A), even in athletes without documented major arrhythmic events.3 Competing in sports beyond class 1A may be considered, on an individual basis. They should, however remain under close periodic surveillance.4

For LQT1 patients, swimming should be specifically limited or performed under supervision. LQT2 patients should avoid exposure to acoustic stimuli especially during sleep (avoidance of telephone and alarm clock on the night stand). All patients with LQTS should avoid drugs known to prolong the QT interval and those that deplete potassium and magnesium.

The addition of β-blockers significantly reduces the risk of aborted cardiac arrest (ACA) and sudden cardiac death in children with LQTS, particularly in patients with a history of syncope.2


1. Aziz PF, Sweeten T, Vogel RL, Bonney WJ, Henderson J, Patel AR, Shah MJ. Sports participation in genotype positive children with long QT syndrome. JACCCEP 2015;1:62–70.

2. Liu JF, Jons C, Moss AJ, et al. Risk factors for recurrent syncope and subsequent fatal or near-fatal events in children and adolescents with long QT syndrome. J Am Coll Cardiol 2011;57:941–50.

3. Zipes DP, Camm J, Borggrefe M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death) developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Europace 2006;8:746–837.

4. Johnson JN, Ackerman MJ. Return to play? Athletes with congenital long QT syndrome. Br J Sports Med 2013;47:28–33.

Published on: June 26, 2015

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