9/17/2010. Phidippides. Phidippides. Sudden Death in the Young Athlete. What is the extent of the problem? Can we prevent it?

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1 Phidippides Phidippides Sudden Death in the Young Athlete What is the extent of the problem? Can we prevent it? 1

2 Number of cardiovascular (CV), trauma-related, and other sudden death events in 1866 young competitive athletes, tabulated by year ( ) Maron, B. J. et al. Circulation 2009;119: Approximately 1/200,000 in USA Reported rate increasing 6%/year Copyright 2009 American Heart Association 65% < 18 years old, 59% high school sports 89% male Basketball(33%) and football(25%) most common (soccer outside U.S.A.) 80% during or just after sports or physical activities, 20% not associated with activity Incidence 0.61/100,000 person years (2.3/100,000 person years in Italian lit.) Incidence and relative risk (RR) of sudden death (SD) among athletes (solid columns) and non-athletes (open columns) from cardiovascular and non-cardiovascular causes Does exercise increase the risk of sudden death in athletes? Corrado, D. et al. J Am Coll Cardiol 2003;42: Copyright 2003 American College of Cardiology Foundation. Restrictions may apply. 2

3 Causes of Sudden Death in 387 Young Athletes. Maron BJ. N Engl J Med 2003;349: Distribution of cardiovascular causes of sudden death in 1435 young competitive athletes Maron, B. J. et al. Circulation 2007;115: Copyright 2007 American Heart Association National Registry of Sudden Death in the Athlete The athlete participated in organized team or individual sports that required regular competition against others as a central component, placed a high premium on excellence and achievement, and required vigorous, systematic training. Sudden death or survived cardiac arrest at < 40 years old. Sudden death was defined as an unexpected collapse with or without physical exertion with no previous history 3

4 Flow diagram summarizing causes of death in 1866 young competitive athletes Maron, B. J. et al. Circulation 2009;119: Copyright 2009 American Heart Association Ray Chapman Shortstop, Cleveland Indians August 17 th 1920 Cardiovascular deaths according to race, with respect to the number of white and nonwhite athletes with each disease Deaths due to CV disease 64% of non-whites 51% of whites Maron, B. J. et al. Circulation 2009;119: Copyright 2009 American Heart Association 4

5 Classification of sports Recommendations 1A Mitchell, J. H. et al. J Am Coll Cardiol 2005;45: Copyright 2005 American College of Cardiology Foundation. Restrictions may apply. Hank Gathers LMU NCAA Leader in scoring and rebounding Hypertrophic Cardiomyopathy Inappropriate LV Hypertrophy (without cause) without dilatation With hyperdynamic systolic function 1/500 of US population 5

6 Hypertrophic Cardiomyopathy Synonyms Idiopathic Hypertrophic Subaortic Stenosis (IHSS) Hypertrophic Obstructive Cardiomyopathy (HOCM) Asymmetric Hypertrophic Cardiomyopathy (ASH) Genetic Abnormality in HCM Inherited trait is usually autosomal dominant. HCM is frequently a hereditary disorder with transmission to 1 st degree relatives in 50% of cases. Genetically and phenotypically y HCM is extremely heterogeneous disease. Mutations in genes for cardiac sarcomeric proteins are linked to HCM. (12 identified) Largest number of mutations have been identified in ß-myosin heavy chain gene on chromosome 14. Diagnosis of HCM Variants of HCM Asymmetric hypertrophy of LV, predominantly septum and anterior wall (70%); basal septal hypertrophy (15-20%); concentric left ventricular hypertrophy (8-10%); apical and lateral wall (<2%). Extent of hypertrophy inversely related to age* 6

7 Hypertrophic Cardiomyopathy Obstructive (25%) vs Non-Obstructive Hypertrophic Cardiomyopathy Hypertrophic Cardiomyopathy Increased Myocardial O2 Demand Decreased diastolic function 7

8 D S Hypertrophic Obstructive Cardiomopathy Dynamic Outflow Obstruction Hypertrophic Obstructive Cardiomyopathy Hypertrophic Cardiomyopathy Degree of Obstruction Directly related to degree of contractility Inversely related to degree of vascular resistance (afterload) Inversely related to degree of left ventricular filling (preload) Consider relationship to the physical activity 8

9 Pathophysiology of HCM Dynamic left ventricular outflow tract (LVOT) obstruction (25%). Diastolic dysfunction (Impaired Relaxation and Compliance) Arrhythmias PVC s, VT, Atrial fibrillation Myocardial ischemia (usually with normal coronary arteries). Mitral regurgitation (MR). Symptoms of HCM 1. Fatigueability 2. Angina Pectoris 3. Dyspnea 4. Palpitations 5. Syncope, sudden death Most patients are asymptomatic Incidental finding on exam, EKG, Echo 9

10 Hypertrophic Cardiomyopathy EKG Left ventricular hypertrophy Q Waves ST Segment and T Wave Abnormalities Arrhythmias Left bundle branch block EKG is frequently normal or non-diagnostic Systolic anterior motion SAM Prognosis of HOCM in Unselected Populations 2.0 Annual Cardiovascular Mortality 1.5 %/yr Study Doffland Spirito Cecchi Cannan Maron Pt (no.)

11 Survival in a Regional U.S. Cohort Age at Diagnosis >20 Years Age at Diagnosis <20 Years N = 234 N=43 Maron: JAMA, 1999 Survival According to Clinical Variables Maron et al. JAMA 1999;287: Highest Levels of Risk in HOCM Prophylactic use of AICD Previous cardiac arrest Family history of premature HCM-related death in (or multiple) relatives DNA documentation of certain high-risk mutant genes Syncope Multiple bursts of Non-sustained VT LVH >30 mm wall thickness Hypotensive BP response to exercise EPS response is an unresolved issue and has been largely abandoned. after Maron 11

12 Recommendations for HCM Class IA Competitive sports are contra-indicated regardless of age, gender, phenotypic appearance, symptoms, LVOT obstruction, or prior treatment with medical therapy or interventions such as AICD, surgical myectomy or ETOH septal ablation. Myocarditis Inflammatory disease of the myocardium, usually due to a viral infection Evolves through an acute inflammatory process followed by heterogeneous necrosis and healing with interstitial edema, focal necrosis, and replacement fibrosis. (unstable electrical substrate) May progress to dilated cardiomyopathy Myocarditis Antecedent viral illness Chest pain, DOE, fatigability, palpitations, syncope EKG: conduction abnormalities, supraventricular and ventricular arrhythmias ECHO: Global or regional abnormalities, dilatation, pericardial effusion CHF, Cardiogenic shock 12

13 Myocarditis Diagnosis (Extremely difficult) Clinical evaluation EKG, echocardiogram Myocardial biopsy Cardiac MRI Myocarditis Recommendations Competitive sports are contraindicated for 6 months after onset of symptons Resolution of EKG and ECHO abnormalities at rest (including arrhythmias) No inducible EKG or ECHO abnormalities with exercise testing Resolution of inflammatory markers Pericarditis Recommendations Competitive sports contra-indicated until complete resolution of clinical course as well as EKG, ECHO and biomarker abnormalities 13

14 Flo Hyman Olympic Volleyball 1980 and 1984 Marfan Syndrome Hereditary autosomal disorder (<1:5000) >400 Mutations Progressive aortic root dilatation of ascending or descending aorta predisposing to dissection or rupture Mitral valve prolapse with associated significant regurgitation and/or left ventricular dysfunction may be present Marfan Syndrome Diagnosis History (Family history) Physical Aortic or mitral regurgitation, other unique Marfan features Chest X-Ray Echocardiogram CT Angiography / MRA 14

15 Marfan Syndrome Recommendations Competitive sports contra-indicated if: a) aortic root dilatation b) moderate to severe regurgitation c) family history of dissection d) family history of sudden death (Marfan) Low to moderate sports only without above Avoid sports involving body collision Regardless of medical or prior surgical therapy Pistol Pete NBA Guard Pete Maravich Anomalous Coronary Artery 15

16 Single Coronary Artery May have exertional symptoms May have abnormal exercise test A 15-year-old male Italian soccer player with a history of exertional syncope one year before death who died suddenly while running during the second half of a game Basso, C. et al. J Am Coll Cardiol 2000;35: Copyright 2000 American College of Cardiology Foundation. Restrictions may apply. Congenital Coronary Anomalies Coronary arteries arising from the wrong coronary sinus Competitive sports is contra-indicated if the coronary artery traverses between the Aorta and Pulmonary Artery Can resume sports 3 months after successful intervention if no evidence of residual inducible ischemia, arrhythmias or myocardial damage 16

17 Arrhythmogenic Right Ventricular Dysplasia (ARVD) Replacement of RV myocardium with fatty and fibrotic tissue and thinning causing electrical instablility Autosomal dominant disorder Common in Italian literature (6/10,000) Difficult to diagnose clinically Difficult to diagnose non-invasively Syncope/ family history important Competitive sports contra-indicated Congenital Heart Disease Atrial septal defect Ventricular septal defect Aortic valve disease Mitral valve disease Coarctation of aorta Patent ducus arteriosus Congenital Heart Disease-untreated Recommendations Competitive sports are contra-indicated if the lesion is hemodynamically significant Extensive non-invasive evaluation is critical and frequently involves exercise testing as well Physical examination with careful cardiac auscultation is critical in the screening process 17

18 Primary cardiac arrhythmias Clinical Evaluation Frequently asymptomatic Symptoms such as palpitations are frequently benign Syncope requires extensive evaluation Family history of sudden death requires further evaluation Primary cardiac arrhythmias Sinus nodal dysfunction AV nodal dysfunction Atrial arrhythmias Ventricular arrhythmias Accessory bypass tracts (WPW) Ion channel disorders Brugada Sinus and AV Nodal Dysfunction in the Trained Athlete Very common due to high vagal tone Resolution should occur with exercise testing. If not, further evaluation may be needed to clear for sports Symptoms of ALOC, fatigue, etc., should warrant further evaluation Congenital, narrow QRS Complete AV Nodal Block with no symptoms, structurally normal heart, and appropriate response to exercise testing are cleared for sports 18

19 Athletes with Pacemakers Sports with high likelihood of bodily collision with potential direct blow to generator are contra-indicated ( Football, rugby, boxing, lacrosse, martial arts and hockey) Protective padding for the generator is recommended for other sports with possible bodily collision ( Baseball, softball, soccer, and basketball) WPW (Pre-excitation) Syndrome Accessory Bypass Tract = Beta pathway AV Node = Alpha Pathway Early, but Abnormal ventricular depolarization Accessory bypass tracts Prone to atrial fibrillation Can result in very rapid ventricular response and sudden death 19

20 Accessory Bypass Tracts (WPW) Symptoms or EKG needed to identify in advance If > 20 years old, asymptomatic, no evidence of structural heart disease and no inducible arrhythmias - can participate in competitive sports. Further evaluation, possible invasive, in those < 20 years old Symptoms or inducible arrhythmias requires further evaluation Successful ablation allows full participation in sports without restriction Ventricular Ectopy PVC s as well as ventricular tachyarrhythmias are common in the trained athlete. Usually no associated structural heart disease and do not appear to be at increased risk. Frequently disappear with deconditioning. H&P, EKG, ECHO and exercise testing are usually needed for evaluation Hereditary Arrhythmias Long QT Syndrome (QTc > 470ms) Brugada Syndrome Competitive sports usually contra- indicated QTc = QT/ R-R R Interval 20

21 Demographics and Natural History of Congenital LQTS 1:10,000 is gene carrier Estimated incidence of sudden death 3-4,000/year Untreated patients incidence of sudden cardiac death is 10%/year 30% of deaths or aborted deaths occur as first event LQTS: Phenotype-Genotype Considerations 6 genotypes; ~200 different mutations Clinical differences among LQT1, LQT2, & LQT3 genotypes Clinical variability within a genotype Clinical variability among members of a family with the same gene mutation suggests presence of modifier genes If asymptomatic, restricted to 1A activities Perce ent 40 Occurrence of Gene-Specific Triggers 70 Exercise 60 Emotional Stress Rest LQT1 LQT2 LQT3 Circ 2001;103:

22 Chromosome 3 II avf V 5 Event-Free Survival in 580 Patients According to the Genetic Locus No. at Risk LQT LQT LQT Priori et al., NEJM 2003;348: Event-Free Survival Among 580 Patients According to Quartile QTc 446 msec or less msec msec >498 msec No. at Risk 1 quartile quartile quartile quartile Priori et al., NEJM 2003;348:

23 Brugada Syndrome The syndrome is a clinical- electrocardiographic diagnosis based on the occurrence of syncopal or sudden death episodes in patients without demonstrable structural heart disease and a characteristic ECG pattern of apparent right bundle branch block and ST segment elevation in leads V1 to V3. Two Types of ST Segment Elevation From Patients with Brugada-Type LCG in leads V 1 to V 3 Coved Type Saddle- back Type Coved Type + Pilsicainide V 1 V 2 V 3 Ikeda ANE 2002;7: Risk Stratification in Brugada Common abnormality? (Italian lit.) SCD tends to occur around age 40 Males > Females (9:1) FH common but not predictive of fatal events Highest risk in pts with resuscitated SCD or syncope with spontaneous pattern No effective drug therapy only ICD Restricted to 1A sports activities only 23

24 Preparticipation Screening History and Physical Examination Formalized routine screening including EKG s and Echocardiograms? DNA Testing? * million athletes eligible for evaluation *0.3% incidence of cardiac diseases capable of causing sudden death Only 3% of screened trained athletes who died suddenly of heart disease were suspected of possible CV disease and none were disqualified from competition History Screening Symptoms: exertional chest pain, excessive exertional dyspnea, inappropriate fatigue, palpitations, altered loss of consciousness, syncope History of heart murmur Family history: Premature heart disease and/or unexpected sudden death < 50 years old Family history of cardiomyopathy, ion channelopathy, Marfan Syndrome Drug history (Cocaine abuse, etc.,) Co-morbid conditions AHA Consensus Panel Recommendations 2005 Bethesda Conference Physical Examination Elevated blood pressure, heart rate Peripheral pulses (Coarctation of aorta) Stigmata of Marfan syndrome Cardiac auscultation a) Significant irregularities b) Heart murmur: DYNAMIC auscultation JACC Vol.45. No.8,

25 Pre-participation Screening High School/ College 41 states have adequate questionnaires (> 9 of 12 questions as recommended by AHA) 4 states inadequate with < 4 questions No national mandate, governed state by state with 64% increase in non-physicians i performing evaluation in past decade, 18 states allow screening by chiropractors and naturopaths NCAA schools now routinely use mandated screening measures including 10 of 12 questions (excluding Marfan and fatigueability) Gaines Adams Defensive End Chicago Bears Thomas Herrion Offensive guard SF 49 s Athlete s Heart Physiological adaptations and cardiac structural remodeling in trained athletes can result in cardiac arrhythmias and morphological abnormalities that can closely resemble serious cardiovascular diseases such as hypertrophic, dilated, and arrhythmogenic right ventricular cardiomyopathies as well as sinus and complex ventricular dysrhythmias that may result in inappropriate disqualification of competitive sports. 25

26 Gray Area of Overlap between Athlete's Heart and Cardiomyopathies, Including Myocarditis, Hypertrophic Cardiomyopathy, and Arrhythmogenic Right Ventricular Cardiomyopathy. Maron BJ. N Engl J Med 2003;349: Sudden death in the competitive athlete is fortunately a rare event. The death of an athlete stirs public interest and arouses emotions about a young life, especially someone who should be in better health than the average person, needlessly lost. The approach to prevent these events is controversial. 26