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Should sports participation be restricted in LQTS? L. Környei Hungarian Pediatric Heart Center 5th February 2017 14:00 – 15:15 Inherited Arrhythmia Syndromes VI: Long QT Syndrome: Complicated Cases Topics 1. Early data 2. Recent data 3. Recent recommendation 4. The “real” question to be answered Early data… • increased frequency of cardiac events during exercise in patients with LQT1 – (swimming 62% vs sleeping 3%) Schwartz, Circulation 2001 • In untreated patients, LQTS-related cardiac events occurred in 55% Shimizu, JACC 2001 • Thus, guidelines have historically advocated for an aggressive restriction from competitive sports, with the exception of low-intensity sports Bethesda 36, ESC 2005 “historical decision making” • beta-blockade • avoidance of medications that prolong the QT interval • “you're not to play sports” Physiologic benefits of exercise sports participation brings to children and adolescents • enhanced self-confidence, • sense of psychological, physical and social wellbeing • improved overall quality of life …but the pendulum is now swinging away… LETTERS It started with .. ONLINE FIRST involved in sports (1 sports (27, 12%) fo patients (37%, 60 fe RESEARCH LETTER [46] milliseconds) r Competitive Sports Participation in Athletes including 20 with IC With Congenital Long QT Syndrome ences between the to the nonathletes wer Johnson, Ackerman, JAMA, August 8 To the Editor: Competitive sports participation for 22/29, ath- 2012—Vol competed308, in aNo. variet letes with long QT syndrome (LQTS) is guided by the and 49 of 130 (38%) 36th Bethesda Conference, which recommends that There were 32 athlet patients with either (1) symptoms, (2) a corrected QT and 8 (6%) competin interval (QTc) greater than 470 milliseconds (males) or fessional level. 480 milliseconds (females), or (3) an implantable Seventy athletes ( cardioverter-defibrillator (ICD) not participate in most European guideline sports.1 The European Society of Cardiology guidelines None had a sport-rela are more restrictive, disqualifying athletes from all sports (46%) continuing in based solely on a stringent QTc cutoff (!440 millisec- only 1 experienced s Research letter • 105 mutations • most common mutation was KCNQ1 (58%) – KCNH2 (35%),SCN5A (6%),KCNE2 (2%),KCNE1 (1%) • All patients were treated with beta-blockade – (1 noncompliant, 1 intolerant) • 26 pts competitive sports (15 girls; mean follow-up 6.9 years; mean QTc, 461 ms • 77 pts 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 • 5 appropriate ICD shocks in 2 pts, but non were related to sports …but the pendulum is now swinging away… It is accelerated by .. JACC: CLINICAL ELECTROPHYSIOLOGY VOL. 1, NO. 1-2, 2015 ª 2015 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION PUBLISHED BY ELSEVIER INC. ISSN 2405-500X/$36.00 http://dx.doi.org/10.1016/j.jacep.2015.03.006 Sports Participation in Genotype Positive Children With Long QT Syndrome Peter F. Aziz, MD,* Tammy Sweeten, MS,y Ramon L. Vogel, MD,y William J. Bonney, MD,y Jacqueline Henderson, RN,y Akash R. Patel, MD,z Maully J. Shah, MBBSy ABSTRACT OBJECTIVES The study sought to examine the prevalence and outcomes of sports participation (both competitive and recreational) in our single-center LQTS genotype positive pediatric population. Pediatric data • • • • • • • • Children’s Hospital of Philadelphia from 1998 to 2013 212 patients with LQTS 103 participated in sports (53 girls) all were treated with BB—(nadolol for 97 pts) mean follow-up, 7.1 years mean QTc, 468 ms there were no cardiac events and no deaths Recent data Sports participation appears safer today for children with long QT syndrome ? Explanation ? Different patient population • Ten years ago before genotyping was mainstream, most patients were diagnosed as a result of an event • With the introduction of genetic testing and cascade familial testing, there are a lot more patients being identified with LQTS, without any event • the chance of a patient without symptoms presenting for the first time with sudden cardiac death/cardiac arrest is approximately 5% “shared decision” • if the restriction is devastating, and in some cases it might be, harming the child's psychological, physical, or social well-being • sports participation might be safe in certain circumstances JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. 66, NO. 21, 2015 ª 2015 BY THE AMERICAN HEART ASSOCIATION, INC. AND ISSN 0735-1097/$36.00 THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION http://dx.doi.org/10.1016/j.jacc.2015.09.042 PUBLISHED BY ELSEVIER INC. AHA/ACC SCIENTIFIC STATEMENT Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies A Scientific Statement From the American Heart Association and American College of Cardiology Michael J. Ackerman, MD, PHD, FACC, Chair* Douglas P. Zipes, MD, FAHA, MACC* Richard J. Kovacs, MD, FAHA, FACC* Barry J. Maron, MD, FACC* The cardiac channelopathies are a collection of primary, fibrillation. Approximately 1 in 1,000 people are genetically mediated heart rhythm disorders (also affected by a cardiac channelopathy, with LQTS referred to as the primary electrical disorders) that are being most common, involving an estimated 1 in 2,000 generally associated with a structurally normal heart people (1). and a propensity for syncope, seizures, or sudden car- Presently, these channelopathies should be viewed diac arrest precipitated by a channelopathy-mediated as potentially lethal but highly treatable conditions. episode of nonsustained or sustained polymorphic However, unlike the various bradyarrhythmias and AHA/ACC SCIENTIFIC STATEMENT Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies A Scientific Statement From the American Heart Association and American College of Cardiology Recommendations 1. For athletes with a suspected/diagnosed cardiac channelopathy, a comprehensive evaluation by a heart rhythm specialist or genetic cardiologist with sufficient experience and expertise with these disorders is recommended (Class I; Level of Evidence C). Michael J. Ackerman, MD, PHD, FACC, Chair* Douglas P. Zipes, MD, FAHA, MACC* Richard J. Kovacs, MD, FAHA, FACC* Barry J. Maron, MD, FACC* The cardiac channelopathies are a collection of primary, fibrillation. Approximately 1 in 1,000 people are genetically mediated heart rhythm disorders (also affected by a cardiac channelopathy, with LQTS referred to as the primary electrical disorders) that are being most common, involving an estimated 1 in 2,000 generally associated with a structurally normal heart people (1). and a propensity for syncope, seizures, or sudden car- Presently, these channelopathies should be viewed diac arrest precipitated by a channelopathy-mediated as potentially lethal but highly treatable conditions. episode of nonsustained or sustained polymorphic However, unlike the various bradyarrhythmias and ventricular tachycardia (torsade de pointes) or ven- tachyarrhythmias detailed in the Task Force 9 report tricular fibrillation. These cardiac channelopathies (2), there remains significant variability and hetero- include long-QT syndrome (LQTS), catecholaminergic geneity among pediatric and adult heart rhythm polymorphic ventricular tachycardia (CPVT), Brugada specialists in terms of their ability to diagnose, risk syndrome (BrS), early repolarization syndrome, short- stratify, and treat patients with these conditions. For QT syndrome, and potentially idiopathic ventricular example, in 1 study, 40% of the patients who received AHA/ACC SCIENTIFIC STATEMENT Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies A Scientific Statement From the American Heart Association and American College of Cardiology Recommendations 2. It is recommended that symptomatic athletes with any suspected or diagnosed cardiac channelopathy be restricted from all competitive sports until a comprehensive evaluation has been completed Michael J. Ackerman, MD, PHD, FACC, Chair* Douglas P. Zipes, MD, FAHA, MACC* Richard J. Kovacs, MD, FAHA, FACC* Barry J. Maron, MD, FACC* The cardiac channelopathies are a collection of primary, fibrillation. Approximately 1 in 1,000 people are genetically mediated heart rhythm disorders (also affected by a cardiac channelopathy, with LQTS referred to as the primary electrical disorders) that are being most common, involving an estimated 1 in 2,000 generally associated with a structurally normal heart people (1). and a propensity for syncope, seizures, or sudden car- Presently, these channelopathies should be viewed diac arrest precipitated by a channelopathy-mediated as potentially lethal but highly treatable conditions. episode of nonsustained or sustained polymorphic However, unlike the various bradyarrhythmias and ventricular tachycardia (torsade de pointes) or ven- tachyarrhythmias detailed in the Task Force 9 report tricular fibrillation. These cardiac channelopathies (2), there remains significant variability and hetero- include long-QT syndrome (LQTS), catecholaminergic geneity among pediatric and adult heart rhythm polymorphic ventricular tachycardia (CPVT), Brugada specialists in terms of their ability to diagnose, risk syndrome (BrS), early repolarization syndrome, short- stratify, and treat patients with these conditions. For QT syndrome, and potentially idiopathic ventricular example, in 1 study, 40% of the patients who received AHA/ACC SCIENTIFIC STATEMENT Eligibility and Disqualification Recommendations for Competitive Athletes Recommendations With Cardiovascular Abnormalities: It is reasonable for Force an asymptomatic athlete with genotype-positive/ Task 10: The Cardiac Channelopathies phenotype-negative LQTS A Scientific Statement From the American Heart Association and American College of Cardiology 1) avoidance of QT-prolonging drugs for athletes with LQTS Michael J. Ackerman, MD, P D, FACC, Chair* Douglas P. Zipes, MD, FAHA, MACC* (http://www.crediblemeds.org) Richard J. Kovacs, MD, FAHA, FACC* H Barry J. Maron, MD, FACC* 3) electrolyte/ hydration replenishment and avoidance of dehydration The cardiac channelopathies are a collection of primary, fibrillation. Approximately 1 in 1,000 people are 4) avoidance or treatment of hyperthermia from febrile illnesses or traininggenetically mediated heart rhythm disorders (also affected by a cardiac channelopathy, with LQTS related heatreferred exhaustion orelectrical heat disorders) strokethatfor with involving eitheranLQTS BrS to as the primary are athletes being most common, estimated 1or in 2,000 generally associated with a structurally normal heart people (1). and a propensity for syncope, seizures, or sudden car- Presently, these channelopathies should be viewed tricular fibrillation. These cardiac channelopathies (2), there remains significant variability and hetero- diacof arrest by a automatic channelopathy-mediated as potentially lethal but highly conditions. 5) acquisition a precipitated personal external defibrillator as treatable part of the episode of nonsustained or sustained polymorphic However, unlike the various bradyarrhythmias and athlete’s personal sports safety gear, and ventricular tachycardia (torsade de pointes) or ventachyarrhythmias detailed in the Task Force 9 report include long-QT syndrome (LQTS), catecholaminergic geneity among pediatric and adult heart rhythm 6) establishment of an emergency action plan with the appropriate school or polymorphic ventricular tachycardia (CPVT), Brugada specialists in terms of their ability to diagnose, risk syndrome (BrS), early shortstratify, team officials (Class IIa;repolarization Level ofsyndrome, Evidence C). and treat patients with these conditions. For QT syndrome, and potentially idiopathic ventricular example, in 1 study, 40% of the patients who received AHA/ACC SCIENTIFIC STATEMENT Eligibility and Disqualification Recommendations for Competitive Athletes With Cardiovascular Abnormalities: Task Force 10: The Cardiac Channelopathies A Scientific Statement From the American Heart Association and American College of Cardiology Recommendations Ackerman, MD, P D, FACC, Chair* Douglas P. Zipes, MD, FAHA, MACC* 5. ForMichael an J.athlete with either symptomatic LQTS or Richard J. Kovacs, MD, FAHA, FACC* Barry J. Maron, MD, FACC* electrocardiographically manifest LQTS (i.e., corrected QT interval >470 ms in males or >480 ms The cardiac channelopathies are a collection of primary, fibrillation. Approximately 1 in 1,000 people are in females), competitive sports participation (except genetically mediated heart rhythm disorders (also affected by a cardiac channelopathy, with LQTS referred to as the primary electrical disorders) being most common, involving an estimated 1 in 2,000 competitive swimming inthataarepreviously symptomatic generally associated with a structurally normal heart people (1). LQT1 host)formay considered aftertheseinstitution of be viewed and a propensity syncope, be seizures, or sudden carPresently, channelopathies should diac arrest precipitated by a channelopathy-mediated as potentially lethal but highly treatable conditions. treatment and appropriate precautionary measures episode of nonsustained or sustained polymorphic However, unlike the various bradyarrhythmias and ventricular tachycardia (torsade de pointes) or ventachyarrhythmias detailed in the Task Force 9 report assuming the athlete has been asymptomatic onand heterotricular fibrillation. These cardiac channelopathies (2), there remains significant variability include long-QT syndrome (LQTS), catecholaminergic geneity among pediatric and adult heart rhythm treatment for at least 3 months (Class polymorphic ventricular tachycardia (CPVT), Brugada specialists in termsIIb) of their ability to diagnose, risk H syndrome (BrS), early repolarization syndrome, short- stratify, and treat patients with these conditions. For QT syndrome, and potentially idiopathic ventricular example, in 1 study, 40% of the patients who received Practical recommendations • BB - 15%-20% reduction in peak HR during exercise • avoid routinely exceeding 65% of their target HR • emphasize the importance of – – – – compliance with medication maintaining hydration resting when experiencing symptoms rescue systems are in place • good relationship with the family – surveillance monitoring for efficacy of medical therapy (Holter,exercise testing) – access to advice and feedback regarding exercise Cheung et al, Canadian Journal of Cardiology 32 (2016) 452e458 doctor relationship (Fig. 3). and the Canad Making recommendations fromDisclosures Conclusions Recent evidence suggests a lower risk of exercise-related A.D.K. rec arrhythmias in treated patients than previously assumed. evaluation to participation Foundation of Stroke Founda Chair in Heart Charles Kerr D netics. S.S. is s of Canada (G conflicts of int References 1. Krahn AD, H tients with un Preserved Ejec 278-85. 2. Behr E, Wood degree relativ 2003;362:145 3. Tan HL, Hof Sudden unexp logical and g 2005;112:207 Figure 3. Making recommendations from evaluation to participation. al, Canadian Journal of Cardiology 32 AED,Cheung automatedetexternal defibrillator; ICD, implantable cardioverter 4. McGorrian C (2016) 452e458 screening in r Fact and question Fact: aerobic activity is a "potential risk-taking behavior" for patients with LQTS Question: Whether the increased risk can be "circumvented, neutralized, or minimized" rather than disqualifying the patient from sports entirely. M. Ackerman ugh of ins ing een no ost ntof odnes owver, cise ith arula- condition. Cartoon toofencourage safe and Increased awareness the patient, family, and community issupervised essential to managing the riskactivity of arrhythmic and SCD physical in children events, and we emphasize the patient’s role in creating a safe LQTS environment for sportswith participation (Fig. 1). Proficiency in Figure 1. et Cartoon to encourage and supervised physical activity Cheung al, Canadian Journalsafe of Cardiology 32 (2016) 452e458 in children.