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Sudden Cardiac Death (Scd) in Young Athletes

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Sudden Cardiac Death (Scd) in Young Athletes SUDDEN CARDIAC DEATH (SCD) IN YOUNG ATHLETES Mehrdad Salamat, MD, FAAP, FACC Clinical Associate Professor of Pediatrics Texas A & M University; Health Science Center SCD in Young Athletes I have no relevant financial relationship(s) to disclose SCD in Young Athletes - 2021 History/Myth The earliest documented case of SCD occurred in 490 BC. Pheidippides (fīdĬp´Ĭdēz), a Greek soldier, conditioned runner, ran from Marathon to Athens to announce military victory over Persia, only to deliver his message, “Rejoice! We conquer!”, then to collapse and die. Rich BS. Sudden death screening. Med Clin North Am 1994;78(2):267-88 SCD in Young Athletes - 2021 Definition Sudden cardiac death (SCD) / arrest (SCA) – Non-traumatic and unexpected sudden cardiac arrest that occurs within 1 hour of a previously normal state of health Competitive athlete – Someone who participates in an organized team or individual sport that requires regular competitions against others as a central component, places a high premium on excellence and achievement, and requires vigorous and intense training in a systematic fashion SCD in Young Athletes - 2021 Objectives To know the incidence of SCD To learn the mechanism of SCD To understand the conditions that may lead to SCD To recognize the warning signs SCD in Young Athletes - 2021 Incidence Documentation? – No nationwide registry – Different sources National Collegiate Athletic Association (NCAA) Media database Insurance companies – During sleep or at rest – SCD vs SCA (emergence of AED) SCD in Young Athletes - 2021 Incidence Overall incidence of SCD – Not clear – Estimates 1:900,000 to 3,000 (AY) – More realistic 1:80,000 to 40,000 (AY) *(AY) = Athlete-year SCD in Young Athletes - 2021 Incidence Annual incidence of Annual Death Rate SCD/SCA in athletes MVA 14,700 – Higher in males (3:1) – Higher in blacks (3:1) Suicide 5,000 – Higher in basketball players Abuse 1,600 Drowning 1,500 Accidents by guns 500 Abuse (Texas) 150 SCD in HS & college 25-50 students (US) SCD in Young Athletes - 2021 Incidence Annual incidence of Annual Death Rate SCD/SCA in athletes MVA 14,700 – Higher in males (3:1) – Higher in blacks (3:1) Suicide 5,000 – Higher in basketball players Abuse 1,600 Drowning 1,500 Accidents by guns 500 Abuse (Texas) 150 Tragic event with multiple spectators and family SCD in HS & college 25-50 members present students (US) SCD in Young Athletes - 2021 Deaths in High-Level Athletes M (15) Soccer playing soccer with his dad M (16) Soccer during a game M (18) Tennis after a coaching session F (13) Swimming warming up at a swimming gala M (17) Soccer after sledging in snow with friends M (17) Rowing watching TV M (16) Soccer while resting SCD in Young Athletes - 2021 www.c-r-y.org.uk Sudden Cardiac Death - Pathophysiology V-fib as the final event Susceptibility to V-fib may be enhanced by – Increased ventricular mass – Relative bradycardia – Non-uniformity of refractoriness – Acidosis – Increased sympathetic nervous system activity 10x rise in norepinephrine, 3x rise in epinephrine during exercise – Dehydration, electrolyte imbalance SCD in young athletes (< 35 y) is rarely secondary to atherosclerotic coronary artery disease SCD in Young Athletes - 2021 Sudden Death in Young Competitive Athletes: Clinical, Demographic and Pathologic Profiles Maron BJ, et al. JAMA 1996;276:199-208 Lesion No. (%) of Athletes HCM + Possible HCM 48 (36) + 14 (10) CA abnormalities 31 (24) Ruptured aortic aneurysm 6 (5) Aortic valve stenosis 5 (4) Myocarditis 4 (3) Idiopathic myocardial scarring 4 (3) Idiopathic DCM 4 (3) ARVD 4 (3) MVP 3 (2) Atherosclerotic CA disease 3 (2) Other CHD 2 (1.5) Long QT syndrome 1 (0.5) Sarcoidosis 1 (0.5) Sickle cell trait 1 (0.5) “Normal” heart 3 (2) SCD in Young Athletes - 2021 Incidence, Etiology, and Comparative Frequency of Sudden Cardiac Death in NCAA Athletes: A Decade in Review Harmon KG, et al. Circulation 2015, 132: 10/2/2019 Forensically confirmed SCD in National Collegiate Athletic Association (NCAA) 2003-2013 ~ 4,200,000 (AY) 514 deaths – SCD 79 (15%) Most common cause – Structurally normal heart *SUD = Sudden unexplained death *SCT = Sickle Cell Trait SCD in Young Athletes - 2021 Sudden Cardiac Death Genetic/ Electrical abnormalities structural abnormalities – LQTS – Cardiomyopathies – SQTS HCM – Brugada syndrome DCM – CPVT ARVD/C – WPW Non-compaction Acquired abnormalities – Congenital coronary artery (CA) abnormalities – Infection > myocarditis > DCM LCA from right sinus of Valsalva – Acquired CA disease – Aortopathies Kawasaki Marfan syndrome – Drug abuse Familial TAAD – Trauma (commotio cordis) – AS, MVP SCD in Young Athletes - 2021 Sudden Cardiac Death Cardiomyopathy – Hypertrophic cardiomyopathy (HCM) Hypertrophic obstructive cardiomyopathy (HOCM) – Dilated cardiomyopathy (DCM) – Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) – Non-compaction cardiomyopathy SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy Hank Gathers Loyola Marymont University basketball star – SCD while playing basketball – Symptomatic months before his death – FHx HCM SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy Historically “most common cause of SCD in US adolescent” Most commonly autosomal dominant (AD) inheritance – Wide variability of expression – Mitochondrial transmission Obstructive or non-obstructive SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy SCD in Young Athletes - 2021 www.nlm.nih.gov Hypertrophic Cardiomyopathy Disarray and disorganization of myocardial fibers, showing abnormal branching, overlapping and hypertrophy, with interstitial plexiform fibrosis. members.aol.com/drhasegawa/hcm.htm SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy History (symptoms mostly with exercise) – Syncope – Near-syncope – Chest pain – Palpitations Significant FHx – Early SCD – Unexplained sudden death – Known FHx of HCM SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy Physical exam (“dynamic” auscultation) – ± SEM, depending on LVOTO – ± SRM, depending on MR ECG – LAD, LVH, ST-abnormalities – Prolonged QTc Echo – Diagnostic SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy SCD in Young Athletes - 2021 Hypertrophic Cardiomyopathy SCD in Young Athletes - 2021 Arrhythmogenic “Right Ventricular” Dysplasia Apoptosis, inflammation, fatty replacement of myocardium – Triangular of dysplasia - RV RV apex, outflow tract, posterior wall – LV could be involved as well Ventricular tachycardia – LBBB pattern – Originating from RV outflow tract Syncope or SCD Accounting for up to 22% of SCD among Italian athletes – Corrado D, et al. NEJM 1998 SCD in Young Athletes - 2021 Arrhythmogenic “Right Ventricular” Dysplasia Physical examination unrevealing 30-50% positive FHx – Major cause of SCD in athletes in the Veneto Region of Northeastern Italy – Mostly autosomal dominant (AD) – Different mutations on multiple chromosome Desmosomal proteins ECG, Holter, EST, echo, MRI, “biopsy” SCD in Young Athletes - 2021 Arrhythmogenic “Right Ventricular” Dysplasia Inverted T waves (V1-V3; 87% of pts) • > 14 years • Absence of CRBBB ε-waves (V1-V3; 33% of pts) • Late potentials; post excitation • Slow depolarization • Signal averaged ECG SCD in Young Athletes - 2021 Arrhythmogenic “Right Ventricular” Dysplasia Patchy replacement of myocardium by fatty or fibro-fatty tissue Hein W. et al. Diagnosis of Arrhythmogenic Right Ventricular Dysplasia: A Review Radiographics. 2002;22:639-648 SCD in Young Athletes - 2021 Arrhythmogenic “Right Ventricular” Dysplasia www.ipej.org/0303/indik.htm SCD in Young Athletes - 2021 Left Ventricular Non-Compaction Failure of myocardial development during embryogenesis – Trabeculated myocardium > myocardial compaction – Failure of compaction of the myocardium > LVNC 8 to 12 per 1,000,000 (underestimated) Autosomal dominant – Mutations in the MYH7 (14q11.2) and MYBPC3 (11011.2) genes have been estimated to cause up to 30% of cases ghr.nlm.nih.gov/condition/left-ventricular-noncompaction#genes SCD in Young Athletes - 2021 Left Ventricular Non-Compaction Clinical presentation – Asymptomatic, CHF, arrhythmias, SCD – Association with Barth syndrome, sickle cell disease Imaging – Comparison of non-compacted to compacted layer of myocardium (NC/C >2) – “Spongy” heart SCD in Young Athletes - 2021 Left Ventricular Non-Compaction SCD in Young Athletes - 2021 Left Ventricular Non-Compaction SCD in Young Athletes - 2021 Sudden Cardiac Death Congenital anomalies of the coronary artery – Left coronary artery (LCA) arising from the right sinus of Valsalva – Single coronary artery – Intramural coronary artery SCD in Young Athletes - 2021 Congenital Coronary Artery Abnormality Pete Maravich “Pistol Pete” Famous LSU and N.O. Jazz 70’s basketball star – Died at 40 years of age while playing a 3 on 3 game in a California gym – Autopsy showed single right coronary artery SCD in Young Athletes - 2021 LCA off the Right Sinus of Valsalva AAOC: ANOMALOUS AORTIC ORIGIN of CORONARY ARTERY https://thoracickey.com/wp- content/uploads/2016/06/B97814377 0637600057X_f1.jpg SCD in Young Athletes - 2021 LCA off the Right Sinus of Valsalva AAOC: ANOMALOUS AORTIC ORIGIN of CORONARY https://thoracickey.com/wp- content/uploads/2016/06/B97814377 0637600057X_f1.jpg SCD in Young Athletes - 2021 Congenital Coronary Artery Abnormality Ostial anomalies – Intramural coronary artery SCD in Young Athletes - 2021 Congenital Coronary Artery Abnormality Presentation (with exertion) – SCD – Syncope – Pre-syncope – Chest pain Diagnosis –
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