Exercise guidelines in athletes with isolated repolarisation abnormalities and structurally normal heart.

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Exercise guidelines in athletes with isolated repolarisation abnormalities and structurally normal heart. Hanne Rasmusen Consultant cardiologist, PhD Dept. of Cardiology Bispebjerg University Hospital Copenhagen, Denmark Conflicts of interest: None

Circulation 2011;124:746-57

Exercise guidelines Competitive sports vs. leisure-time sports (incl. treatment) To be avoided: Burst exertion Extremely adverse environmental conditions Excessive participation Specific exercise in certain diseases Maron et al, Circulation 109:2807-16, 2004 Mitchell et al, JACC 45 (8) 1364-7, 2005.

Exercise guidelines with ICD No competitive sports unless Leisure-time sports: Avoid bodily contact Avoid high risk sports Extreme ipsilateral arm movement Tailor ICD settings Avoid inappropriate shocks Heidbrüchel et al, Eur J Cardiovasc Prev Rehabil, 13:676, 2006 Follow-up every 6 months Mitchell et al, JACC 45 (8) 1364-7, 2005.

ECG changes might precede phenotypic signs of structural heart disease 24 yrs canoer 26 yrs soccer player 29 yrs soccer player No signs of disease Died suddenly within 1 yr Autopsy: ARVC No signs of disease After 7 yrs develops phenotypic evidence of HCM No signs of disease After 12 yrs still phenotypic healthy Pellicia et al, NEJM, 2008

Classification of abnormalities of the athletes ECG Group 1: Sinus bradycardia First-degree AV block Incomplete RBBB Early repolarization Isolated QRS voltage criteria for left ventricular hypertrophy Group 2: T-wave inversion ST-segment depression Pathological Q-waves Left atrial enlargement Left axis deviation/left posterior hemiblock Right axis deviation/left posterior hemiblock Right ventricular hypertrophy Ventricular pre-excitation Complete LBBB or RBBB Long- or short QT interval Brugada-like early repolarization Corrado et al, European Heart Journal 2010

Repolarization incl. J-point Reopening of potassium channels PLUS closing of calcium channels are responsible for the repolarization phase.

Repolarization abnormalities in structually normal heart Group 1: Early repolarization Group 2: T-wave inversion ST-segment depression Long- or short QT interval Brugada-like early repolarization Corrado et al, European Heart Journal 2010

Early repolarization Elevation of the J-point of at least 0,1 mv from baseline, associated with nothing or slurring of the terminal QRS complex which may vary in location, morphology, and degree Corrado et al, Eur Heart J 2010

ST-segment elevation Normal Early repolarization Normal variant Wang et al, NEJM 2003

Haïssaguerre et al, NEJM 2008 Case-control study: 206 subjects with ideopathic ventricular fibrillation (IVF) All cases received ICD ER inferior/lateral leads odds ratio x10 in case subjects In case subjects with ER hazard ratio of recurrence within 5 yrs was 2 Few cases of athletes or blacks among cases

Circulation 2011

Brugada syndrome (BS) Characterized by an ST-segment elevation in the right precordial leads (type 1) and a high incidence of sudden cardiac death in patients with structually normal hearts Antzelevitch et al, Circulation 2005

Brugada like ECG changes STJ ST80 Brugada: STJ / ST80 > 1 Corrado et al, Eur Heart J 2010

Diagnostic criteria of BS Ajmaline Type 1 ST segment elevation 2 mm in > 1 right precordial lead (V1-3) spontaniously or after sodium-blocker exposure AND One of the following: Documented VF (Self-terminating) polymorphic ventricular tachycardia Inducible ventricular arrythmias with EPS Family history of SCD <45 yrs Precense of a coved-type ECG in family embers Syncope Nocturnal agonal respiration Other factors accounting for the ECG abnormality should be ruled out Documented Ventricular arrhythmias Family history Arrhythmia-related symptoms Antzelevitch et al, Circulation 2005

Clinical manifestation and prognosis of BS Symptom occurence: Mean age of onset: 40 yrs Phenotypic more prevalent in males (x10) Typically occur during sleep (like Type 3 LQTS) or fever N=547 patients with BS Mean follow-up 24 months 8 % developed cardiac event Brugada et al, Circulation 108:3092-96, 2003 Benito et al, Prog in Cardiovasc Dis 51(1):1-22, 2008

Treatment of BS Benito et al, Progress in Cardiovasc Dis 2008

Exercise recommendations for Competitive sports: BS Manifest syndrome + ICD or high risk type Ia sports Silent gene carriers or Brugada like ECG changes Moderate cardiovascular demand (no high risk sports, avoid hyperthermia) close follow-up Leisure-time sports (+/- ICD): Manifest syndrome moderate demand No high risk sports for BS No extremely adverse environmental conditions/excessive participation Silent gene carriers or Brugada like ECG changes No restrictions close follow-up

Long QT syndrome (LQTS) An inherited cardiac arrythmic disorder due to mutations affecting the cardiac ion channels resulting in delayed ventricular repolarization and a prolonged QT interval on ECG. The assiciated arrythmias may manifest as syncope, aborted cardiac arrest or SCD Liu & Moss JACC 2011 Short QT syndrome (SQTS) Channelopathy characterized by abnormally short repolarization: QT/QTc < 325 ms

Long QT syndrome QTc measured by Bazett s formula Athletes ESC Corrado et al, 2010 >440 ms >460 ms AHA Zipes et al, 2005 470 ms 480 ms Further evaluation

Diagnostic criteria for LQTS Scoring: 1 point low probability 2-3 points intermediate 4 points high Schwartz et al, Circulation 1993

Proposed sheme for risk stratification among patients with LQTS Genotype Gender QTc Priori et al, Circulation 2003

Investigation of athletes with long QTc Exclude accuired long QTc: Drugs Antibiotics Anti-histaminic drugs Antifungals Tricyclic antidepressant www.qtdrugs.org Organic cardiac disease Sinus node disease or AV block Non-cardiac causes Hypokalaemia Anorexia nervosa Hypothyroidism

Investigation of athletes with long QTc QTc > 500 ms and/or symptoms Referred to a specialist for further evaluation and restricted from sports Grey zone (460/470 500 ms) and asymptomatic Deconditionering Repeated ECGs Holter Exercise testing + Genetic testing 1. Degree relatives ECG Schwartz et al, Circulation 1993

Exercise recommendations for Competitive sports: LQTS Phenotypically overt No competitive sports Silent gene carriers No competitive sports Leisure-time sports (on beta-blockers +/- ICD): No high risk sports for LQTS Swimming (LQTS 1) Diving (LQTS 1) Abrupt loud noises (race starters pistol) (LQTS 2) No burst exertion Basketball Soccer Tennis No extremely adverse environmental conditions/excessive participation

Investigation of athletes with short QTc Exclude accuired short QTc: Organic cardiac disease Tachycardia Non-cardiac causes Hyperkalaemia Hypercalcaemia Hyperthermia Acidosis

Exercise recommendations for Competitive sports: SQTS Phenotypically overt Low static/dynamic demand Silent gene carriers Same Leisure-time sports (+/- ICD): No burst exertion No extremely adverse environmental conditions/excessive participation No high risk sports (motorbike/car driving, diving, swimming)

Conclusion Interpretation of the ECG depends on the context We need more large scale data on repolarization abnormalities in athletes to give evidence-based guidelines on exercise