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Avoiding sports-related sudden cardiac death in children with congenital channelopathy

Recommendations for sports activities

Vermeidung des sportbezogenen plötzlichen Herztods bei Kindern mit angeborenen Ionenkanalerkrankungen

Empfehlungen für sportliche Aktivitäten

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Abstract

For the past few years, children affected by an inherited channelopathy have been counseled to avoid (recreational) sports activities and all competitive sports so as to prevent exercise-induced arrhythmia and sudden cardiac death. An increased understanding of the pathophysiological mechanisms, better anti-arrhythmic strategies, and, in particular, more epidemiological data on exercise-induced arrhythmia in active athletes with channelopathies have changed the universal recommendation of “no sports,” leading to revised, less strict, and more differentiated guidelines (published by the American Heart Association/American College of Cardiology in 2015). In this review, we outline the disease- and genotype-specific mechanisms of exercise-induced arrhythmia; give an overview of trigger-, symptom-, and genotype-dependent guidance in sports activities for children with long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), or short QT syndrome (SQTS); and highlight the novelties in the current guidelines compared with previous versions. While it is still recommended for patients with LQT1 and CPVT (even when asymptomatic) and all symptomatic LQTS patients (independent of genotype) to avoid any competitive and high-intensity sports, other LQTS patients successfully treated with anti-arrhythmic therapies and phenotype-negative genotype-positive patients may be allowed to perform sports at different activity levels – provided they undergo regular, sophisticated evaluations to detect any changes in arrhythmogenic risk.

Zusammenfassung

Bis vor Kurzem wurde Kindern mit angeborenen Ionenkanalerkrankungen die Teilnahme am Freizeit- oder Wettkampfsport meist strikt untersagt, um sportinduzierte Arrhythmien und den plötzlichen Herztod zu vermeiden. Verbesserte pathophysiologische Erkenntnisse über die zugrunde liegenden Erkrankungen, Fortschritte in der antiarrhythmischen Therapie und insbesondere epidemiologische Erfahrungen mit Rhythmusereignissen bei aktiven Athleten mit Ionenkanalerkrankungen haben zu weniger strengen, individualisierten Empfehlungen in den neuen amerikanischen Leitlinien der American Heart Association/des American College of Cardiology von 2015 geführt. Für die Beratung von Kindern mit Long-QT-Syndrom (LQTS), Brugada-Syndrom (BrS), katecholaminerger polymorpher ventrikulärer Tachykardie (CPVT) oder Short-QT-Syndrom (SQTS) beschreiben die Autoren die krankheits- und genotypspezifischen Mechanismen sportinduzierter Arrhythmien, geben einen Überblick über die Neuerungen im Vergleich zu älteren Leitlinien und erörtern die differenzierteren, trigger-, symptom- bzw. mutationsabhängigen Empfehlungen zu den einzelnen Krankheitsentitäten. Neben dem fortbestehenden Sportverbot im kompetitiven und hochintensiven Bereich für alle CPVT- und LQT1-Patienten sowie generell für alle symptomatischen LQTS-Patienten gibt es vor allem beim LQTS Patientengruppen, deren Sportverbot unter bestimmten Voraussetzungen gelockert werden kann. So können z. B. erfolgreich antiarrhythmisch behandelte und darunter asymptomatische LQTS-Patienten sowie mutations-positive phänotyp-negative LQTS-Patienten, die unter regelmäßiger Reevaluation ihres Risikoprofils von Spezialisten begleitet werden, durchaus zu sportlichen Aktivitäten zurückkehren.

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Abbreviations

APD:

action potential duration

BrS:

Brugada syndrome

CPVT:

catecholaminergic polymorphic ventricular tachycardia

EAD:

early afterdepolarization

ICa,L :

depolarizing L‑type calcium current

ICD:

Implantable cardioverter defibrillator

IKs :

slowly activating delayed outward rectifying repolarizing potassium current

IK1 :

inward rectifying repolarizing potassium current

IKr :

rapidly activating delayed outward rectifying potassium current

LQTS:

long QT syndrome

SQTS:

short QT syndrome

RyR:

ryanodine receptor

SrSCD:

sports-related sudden cardiac death

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Authors and Affiliations

  1. Department of Cardiology and Angiology I, Heart Center University of Freiburg, Hugstetter Str. 55, 79106, Freiburg, Germany

    C. N. Lang, J. Steinfurt & K. E. Odening MD

  2. Faculty of Medicine, University of Freiburg, Freiburg, Germany

    C. N. Lang, J. Steinfurt & K. E. Odening MD

  3. Institute for Experimental Cardiovascular Medicine, Heart Center University of Freiburg, Freiburg, Germany

    K. E. Odening MD

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  1. C. N. Lang
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Correspondence to K. E. Odening MD.

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C. N. Lang, J. Steinfurt, and K. E. Odening declare that they have no competing interests.

This article does not contain any studies with human participants or animals performed by any of the authors.

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Lang, C.N., Steinfurt, J. & Odening, K.E. Avoiding sports-related sudden cardiac death in children with congenital channelopathy. Herz 42, 162–170 (2017). https://doi.org/10.1007/s00059-017-4549-2

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