The safety of sports in children with inherited arrhythmia substrates

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TL;DR

This narrative review summarizes current evidence and guidelines on sports participation for children with inherited arrhythmia substrates, including long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), arrhythmogenic cardiomyopathy (ACM), and hypertrophic cardiomyopathy (HCM). The authors argue that exercise restriction can negatively impact quality of life and that, with appropriate risk assessment, shared decision-making, and safety planning, most affected children can safely participate in sports—with the notable exception of ACM, where intense exercise may accelerate disease progression. The review emphasizes a multidisciplinary approach including medication compliance, genetic counseling, and community education on CPR and automated external defibrillator (AED) use.

Full summary

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**Background:** Sudden cardiac death (SCD) in young athletes is rare (1–3 cases/100,000) but devastating, often caused by inherited channelopathies (LQTS, CPVT) and cardiomyopathies (HCM, ACM). Historically, guidelines recommended strict exercise restriction for affected children, but this approach may reduce quality of life, promote obesity, and cause physical deconditioning. The field is shifting toward shared decision-making that balances risks with the benefits of physical activity.

**Methods:** This is a narrative review summarizing current guidelines (ESC, AHA/ACC, HRS/EHRA/APHRS) and observational studies on sports participation in pediatric patients with inherited arrhythmia substrates. The authors discuss evidence for each condition separately, drawing on registry data, retrospective cohort studies, and expert consensus statements. Key studies cited include the ICD Sports Safety Registry, Johnson & Ackerman (2013), Aziz et al. (2015), Chambers et al., Tobert et al., and Ostby et al.

**Key Results:** - **LQTS:** Johnson & Ackerman reported no LQT-triggered cardiac events among 130 competitive athletes (including 20 with ICDs) over >650 patient-years. Aziz et al. found no cardiac events in 26 competitive and 77 recreational pediatric LQTS patients on β-blocker therapy. Tobert et al. reported no exercise-related deaths in 494 LQTS patients over 2056 patient-years, with only 3 (0.6%) experiencing a sports-related cardiac event. - **CPVT:** Ostby et al. found that among 21 CPVT patients who continued sports, only 3 (14%) had breakthrough events (2 during recreational exercise, 1 due to non-compliance). Six of 43 non-athletes (14%) also had events, underscoring medication compliance as critical. - **ACM:** Exercise accelerates disease progression. Lie et al. reported ventricular arrhythmias in 74% of adult ACM patients doing high-intensity and 65% doing long-duration exercise. Wang et al. found a 9-fold increase in appropriate ICD discharges in TMEM43-positive patients doing high-intensity exercise. Smith et al. noted that desmoplakin-associated cardiomyopathy showed no exercise-arrhythmia correlation, suggesting genotype-specific risk. - **HCM:** Pelliccia et al. found no difference in SCA/SCD risk over 7 years between low-risk HCM athletes who continued sports and those who stopped. Malhotra et al. screened 11,168 adolescent soccer players (mean age 16), identifying 5 with HCM; 2 who continued playing died, while 3 who stopped survived. - **ICDs:** The multinational ICD Sports Safety Registry (328 athletes, 10–60 years) reported no deaths, resuscitated arrhythmias, or shock-related injuries during sports. Saarel et al. (129 athletes, 10–21 years, median 3.5 years follow-up) found no ICD failure or injury, though appropriate and inappropriate shocks occurred.

**Clinical Implications:** The authors advocate for a multidisciplinary approach including shared decision-making, regular expert follow-up, medication compliance, genotype-specific risk assessment, and emergency action plans with CPR-trained personnel and accessible AEDs. ACM remains the exception where high-intensity exercise should be restricted due to disease acceleration. For most other conditions, the evidence supports safe sports participation with appropriate precautions. The review highlights that current guidelines are largely based on expert consensus (LOE C) and that more pediatric-specific data are needed.