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

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 sports restriction • Many false negatives

1Rivenes, AJC 2005 Prevention of Sudden Cardiac Event • Echocardiography – Advantages • Useful in detecting some forms of lethal cardiovascular disease – Disadvantages • False positive / false negative results • Echocardiogram may change over time • Difficult to see coronary arteries unless complete and detailed study • Rapid portable scan not adequate for determining large number of causes for SCD Prevention of Sudden Cardiac Event

Even with full cardiac screening, persons at risk for sudden death will still be missed

How do we reduce the incidence of sudden death in athletes and children? Rhythm Prior to SCD

• 157 patients who had a sudden cardiac arrest while being monitored: • – 62% VT/VF – 16% Bradycardia – 13% Polymorphic VT / Torsades – 8% Ventricular fibrillation

Bayes de Luna A, Am Heart J 1989 How Do We Prevent SCD in Athletes and Children? -Determine the final common pathway

Ventricular Fibrillation (VF) -Address the issue How Do We Prevent SCD in Athletes and Children?

Automated External Defibrillator (AED) Ventricular Fibrillation in Children?

• More common than you may think – 19-24% of pediatric out of hospital arrests from VF (SIDS excluded) • Cardiac dysrhythmias typically degenerate from bradyarrhythmias and VF to asystole over the course of a pediatric arrest

Hickey RW. Ann Emerg Med. 1995;25:484-491 Walsh DK. Am J Cardiol. 1983;51:557-561. SCD in Athletes and Children

• Survival – Victims of sudden cardiac arrest are more likely to survive than traumatic cardiac arrest (accident) –But…… – Only if they receive prompt support and treatment SCD in Athletes and Children

• Public Access Defibrillation (PAD) – For each minute the heart fibrillates, the chance of survival decreases by 10% – 5 minutes to defibrillation is critical time – Public access to AED’s reduces response and defibrillation time • Improved survival • Reduced brain damage AED’s in Casinos

• Prospective case series • 105 people w/ VF arrest (Nevada, Philadelphia, Mississippi) • Security officers trained to use AED's • Mean shock time 4.4 +/- 2.9 min • Mean time of arrival of paramedics 9.4 +/- 4.3 min • Survival to hospital discharge 59%

Valenzuela TD.. NEJM 2000; 343(17):1206-1209. • Sixth grade children trained in AED use • All children successfully placed pads and were clear of patient during shock • Mean time of children to shock was 90 +/- 14 sec • Mean time of paramedics to shock was 67 +/- 10 sec Accuracy of AED's in Pediatrics • Advising a shock for shockable rhythms – VT 90-95% • Not advising a shock for non-shockable rhythms – 98-100%

Cecchin F. Circulation. 2001;103:2483-2488. Atkinson E. Ann Emerg Med. 2003;42:185-196 The Problem

• The AEDs have to be: – Available • Not locked in nurses office, across the street, on another field – Maintained • Pads should not be expired • Should be part of the equipment taken onto the field by the coach • Someone has to know how to use them Resources

A Project ADAM Affiliate is an organization (e.g. a children’s hospital) that has committed itself to providing resources to schools and institutions wishing to implement public access defibrillation (PAD) programs in their buildings Lindsay’s Law • Legislation passed in 2017 in Ohio • Educational campaign and screening protocol – Primary and secondary prevention of SCA/SCD in young athletes – Broad application for athletes <19 years old • Organized sports of all types – Raise awareness in the general population • Parents, athletes, coaches • Improve recognition of risk factors • Improve appropriate screening • Improve access and application of AEDs Summary • Sudden cardiac death in young people is rare but devastating – Many causes, some without warning signs • Any screening will miss athletes at risk for SCD – Currently, best screening is careful H&P, per AHA recommendations • Rapid application of AED has the greatest chance of stopping a lethal heart rhythm • Educated population: – aims at improving awareness and optimizing primary and secondary prevention of SCD in young athletes • Thank You! • Appendix

Cardiovascular Response to Exercise • Increased myocardial oxygen consumption – 4-5X normal with strenuous exercise • Increased cardiac output – Increased stroke volume (25-50% above normal) – Increased heart rate – Decreased systemic vascular resistance – Static exercise  increased pulmonary vascular resistance 14 Element AHA Preparticipation Screening • Personal history – Exertional chest pain/discomfort – Unexplained syncope/near syncope • Particularly concerning when related to exertion – Excessive exertional and unexplained dyspnea/fatigue or palpitations associated with exercise – Prior recognition of a heart murmur – Elevated systemic blood pressure – Prior restriction from competitive sports – Prior testing of the heart ordered by a physician 14 Element AHA Preparticipation Screening • Family history – Premature death (sudden and unexplained ) before age 50 because of heart disease in 1 or more relative • Car accidents •Drowning – Disability from heart disease in a close relative younger than 50 – Specific knowledge of certain cardiac conditions in family members: HCM or dilated cardiomyopathy, long QT syndrome, other ion channelopathies, Marfan syndrome, or clinically important arrhythmias 14 Element AHA Preparticipation Screening • Physical examination – Heart murmur (auscultation should be performed in supine and standing positions [or with Valsalva maneuver]), specifically to identify murmurs of dynamic left ventricular outflow tract obstruction • Palpation -RV tap and displaced PMI – Femoral pulses to exclude aortic coarctation – Physical stigmata Marfan syndrome – Brachial artery blood pressure (sitting position) preferably taken in both arms