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Sudden Cardiac Death in Pediatrics: Screening and Prevention

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Sudden Cardiac Death in Pediatrics: Screening and Prevention Sudden Cardiac Death in Pediatrics: Screening and Prevention Lou Bezold, MD Joint Pediatric Heart Care Program Sudden Cardiac Death A 9-year-old Ohio boy died during football practice Monday after he was found unconscious by coaches. He was practicing with the Big Bend Youth Football League when he was found unresponsive by a coach after the team took a break from running sprints. Authorities were called and coaches performed CPR on the third grader until medics arrived. He died later that night. Sudden Cardiac Death Nontraumatic, nonviolent, unexpected event resulting from sudden cardiac arrest within 6 hours of a previously witnessed state of normal health Sudden Cardiac Death • Sudden cardiac death (SCD) in childhood and adolescence is associated with 4 principal forms of cardiovascular disease – Cardiomyopathies – Congenital heart disease • Coronary artery anomalies – Myocarditis – Channelopathies and arrhythmia syndromes How big is the problem? Incidence • Adults – Leading cause of death in the United States – Approximately 500,000 annual deaths – Estimated that 10-30% of adults die suddenly and unexpectedly – Majority due to myocardial ischemia secondary to coronary atherosclerosis with reduced ejection fraction Incidence • Minnesota high school and college athletes (1985-2007)1 – 0.93 deaths per 100,000 person years • Review of NCAA database, media reports, insurance claims (2004-2008)2 – 2.28 per 100,00 person years 1Maron BJ, AJC 2009;104:276-80 2Harmon et al Circ 2011;123:1594-600 Number of Sudden Death Events in 1866 Young Competitive Athletes, by Year Barry J. Maron et al. Circulation. 2009;119:1085-1092 Copyright © American Heart Association, Inc. All rights reserved. Comparative frequencies of death attributable to all causes in young individuals aged <25 yrs. Barry J. Maron et al. Circ. 2014;130:1303-1334 Sudden Cardiac Death • Most athletes who die of sudden death DO NOT have a known history of cardiac abnormality • Most patients with cardiac disease who die suddenly DO NOT do so during exercise – Garson (1985) • 23,402 pts followed for up to 25 yrs (1958-1983) • 0.004% incidence of sudden death • 22% of sudden death related to exercise/activity Alexander Dale Oen, Norway's top Olympic swimmer Died from Cardiac Arrest - He was 26 He died after a day of light training and a game of golf. Cardiovascular Causes of Sudden Death in 1435 Young Competitive Athletes. Barry J. Maron et al. Circulation. 2007;115:1643-1655 Major Causes of SCD • Hypertrophic cardiomyopathy – Most common cause of SCD in athletes – Incidence: – 0.2% in general population – 0.07% in athletes – May not present until adolescence – Common in start-stop sports, ie basketball and football – Myocardial disarray and hypertrophy • Predisposes to ischemia • Predisposes to arrhythmias – Not all have abnormal ECGs – Red flags: FHx, exertional Sx, murmur Hypertrophic RV Cardiomyopathy LV Ao LA RV Ao LV Major Cause of LA Sudden Cardiac Death Major Causes of SCD • Coronary Artery Abnormalities – 12 – 33% of sudden deaths in athletes – Most commonly implicated are left coronary from right sinus and right coronary from left sinus – Exertional chest pain occurs in up to 40% Major Causes of SCD • Myocarditis –Accounts for 5 to 20% of sudden cardiac deaths in the young –Viral etiology most common • Previously healthy heart • Typically viral syndrome 2-3 weeks prior • Ventricles enlarged with poor function –Red flags: new exertional Sx, murmur Durante et. al. Cardiology in the Young. 2015;25:408-423 Long QT Syndrome • Disorder of cardiac ion channels that control contractions • Congenital LQTS occurs in 1:5K-10K people • Incidence of mutations is at least 1 per 2K persons1 –2.5x as common as childhood leukemia –Half as common as cystic fibrosis • 12 different genes identified currently –>70% due to LQT1, LQT2, LQT3 –Genetic test commercially available 1Schwartz, JCE 2003 Long QT Syndrome • Most pts affected with LQTS have prolonged QTc intervals on ECG – 10-15% can have QTc intervals within the normal range • Predisposed to Torsades de Pointes form of VT • Recurrent syncope, especially during exercise, is a significant risk factor for SCD • SCD is estimated to occur at a rate of 1-2% per year in patients with definitively diagnosed LQTS – Accounts for 2% of SCD in athletes – Females are at higher risk – Incidence appears to peak in adolescence and young adulthood – LQT1 most commonly associated with SCD during exercise, particularly swimming and diving Wolff-Parkinson-White Syndrome • Incidence of SCD between 0 and 0.4% per year2 • Mechanism: – Atrial fibrillation conducts rapidly through accessory pathway leading to VT/VF1 • 50% with sudden death had no prior arrhythmia3 1 Broomberg. J AM Coll Cardiol 27:690-695,1996 2 Fitzsimmons P. Am Heart J 142:530-536, 2001 3 Deal. PACE 18:815,1995 Atrial Fibrillation in WPW Other Causes of SCD • Catecholamine-sensitive polymorphic ventricular tachycardia (CPVT) – Normal resting ECG and exam – Increasing ventricular ectopy with exercise – Leads to ventricular fibrillation • Arrhythmogenic right ventricular cardiomyopathy (ARVC) – Fatty infiltrates of right ventricle – More common in Italy Commotio Cordis • Cardiac Concussion – Baseball, fist, etc. to chest – Induced ventricular tachycardia/fibrillation – May be difficult to cardiovert/defibrillate – Arrhythmias may occur up to 24 hours after impact – No increased lifetime risk if survive – Animal models demonstrate a single blow over precordium 10 to 30 msec before the T- wave can induce VF Maron BJ et al. Circulation 1996;94:850-856 SCD in Young Athletes • Sudden cardiac arrest/death may be the first sign of any of these problems • Vigorous exercise may act a trigger for sudden death episodes • Risk of sudden death higher in athletes, but does occur in the general population Maron BJ. NEJM 2003;349:1064-1075 Sudden Cardiac Death Does Not Only Occur in Athletes Maron et al. Am J Cardiol. 2016;117:1339-41 How do you not miss patients at risk for SCD? SCD in Athletes and Children • Who is at risk? – Elementary school • Negligible – Junior high •Extremely low – High school/college • Highest risk category Sudden Death in 1866 Young Athletes in US (1980-2006) • Mean age: – 18 years + 5 years – 84% <25 yrs • 89% male overall – Proportion of women has increased to 12% during 2000-2006 • 55% white, 36% African American • 56% cardiovascular cause Maron BJ et al. Circulation. 2009; 119:1085-1092 Sudden Death Events in 1866 Young Competitive Athletes Barry J. Maron et al. Circulation. 2009;119:1085-1092 Copyright © American Heart Association, Inc. All rights reserved. Sudden Cardiac Deaths – 1049 pts • 64% nonwhite • HCM and coronary anomalies were higher in nonwhites (predominantly African-American) • Ion channelopathies were higher in whites • 80% occurred during or just after physical exertion i.e. practice sessions or organized competition • 20% died suddenly during routine daily activities or while sedentary or sleeping Maron BJ et al. Circulation. 2009; 119:1085-1092 Sudden Cardiac Deaths – 1049 pts All Sports Are Not Equal • Football - 30% • Basketball - 22 % • Soccer - 6% • Baseball - 6% • Track and Field - 5% SCD in Athletes and Children • Why do teenagers have a higher risk of sudden death? – Fight through the pain/push themselves – High level of physical activity – Physiologic changes • Hypertrophic cardiomyopathy may not present until teenage years • Many children who have SCD have had prior symptoms or warning signs – These warning signs may not be communicated by a teenager Prevention of Sudden Cardiac Event • American Heart Association Recommendations for Preparticipation Physical Evaluation – Screening...should be mandatory for all athletes and should be performed before participation in organized high school and college sports. – For high school athletes, screening should occur every two years, with an interim history in intervening years Prevention of Sudden Cardiac Event • American Heart Association Recommendations – “A complete and careful personal and family history and physical examination designed to identify...cardiovascular lesions known to cause sudden death...is the best available and most practical approach to screening” – “The examination is to be performed by a health care worker who has the requisite training, medical skills and background to reliably obtain a detailed cardiovascular history, perform a physical examination and recognize heart disease.” – 14 Element AHA Preparticipation Screening Maron et al. Circulation. 2014;130:1303-1334 Prevention of Sudden Cardiac Event Screening by H&P alone is not sufficient to detect many critical cardiovascular abnormalities Prevention of Sudden Cardiac Event • Electrocardiogram (ECG) – Advantages • Inexpensive • Non-invasive • Frequently abnormal in many lethal cardiovascular diseases – Disadvantages • Wide spectrum of normal ECG variants, particularly in athletes • May miss significant structural or electrical abnormalities Prevention of Sudden Cardiac Event • ECG abnormalities in the athlete’s heart1 – 1005 elite athletes in 38 sports – 40% with abnormal ECG • Only 5% with structural heart disease (echo) • ECG abnormalities in hypertrophic cardiomyopathy – Most common cause of SCD in athletes – 25% with normal ECG2 1Pelliccia A, Circ 2000; 102(3): 278-84 2Texter, Cannon. AHA 2005 Prevention of Sudden Cardiac Event • Most common ECG finding in hypertrophic cardiomyopathy: left ventricular hypertrophy (LVH) • When LVH present on ECG, actual pathology is found ~30%1 • Many false positives – inappropriate
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