Sudden Cardiac Death. Michael Liske, MD, FACC, FASE East Tennessee Pediatric Cardiology Knoxville, TN www.etpc-hearts.com. “Pistol” Pete Maravich. All time leading division I NCAA scorer with 44 PPG for LSU (would have been 57 PPG with 3 point rule)
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Sudden Cardiac Death Michael Liske, MD, FACC, FASE East Tennessee Pediatric Cardiology Knoxville, TN www.etpc-hearts.com
“Pistol” Pete Maravich • All time leading division I NCAA scorer with 44 PPG for LSU (would have been 57 PPG with 3 point rule) • "I don't want to play 10 years in the NBA and then die of a heart attack at 40.“ (age 25) • Died at age 40 while playing a pick up game of basketball with James Dobson, 1988 • Dx?
“Pistol” Pete Maravich • Single right coronary artery
Flo Hyman • 6 ft at 12 years, final height 6 ft, 5 in. • In 1984, led the US volleyball team to Olympic silver • 1986: collapsed on the sidelines watching her team play in Japan • Dx?
Flo Hyman • Aortic dissection from Marfan Syndrome • “Apart from her height, near-sightedness, very long arms and large hands, she showed few other physical symptoms. Hyman's sneaker size was a USA size 12.” (Wikipedia)
Jake Logue • Sullivan South (Kingsport) Rebels co-captain and linebacker • Died on the field while playing West High School, Knoxville, August 2009 • “Logue had been trailing the play, when he dropped to the ground without being touched. He lay on the field motionless. A fellow South player tried to help him, but Logue was unresponsive.” (News Sentinel) • Dx?
Jake Logue • “A heart problem with his bicuspid aortic valve.”(News Sentinel)
Emmanuel Negedu • Sept 2009: “Collapsed on the practice field before UT trainer Chad Newman and director of sports medicine Jason McVeigh heroically brought Negedu back to life with CPR and an automatic external defibrillator.” (ESPN) • Dr. Stuart Bresee, UT cardiologist, said the initial diagnosis was hypertrophic cardiomyopy, however, “once Negedu quit training, his heart was back to normal, . . the genetic testing was all negative.” (ESPN) • He received an ICD and transferred to New Mexico to continue competitive basketball. • Anyone know what happened to him next?
Emmanuel Negedu • Played 10 games for the U of NM Lobos, and led the team in three games for rebounding • While playing The Citadel in a pre-Christmas game, his defibrillator “produced a reading that led doctors to keep him from finishing the game. He never returned to competition.” SportingNews NCAAB, Apr 2011
Overview • Scope and demographics of the problem • Differential diagnoses, including new, in-vogue conditions • Pre-participation screening • Practical EKG: normal or abnormal? • Rational public health approach • Please refer to the 10 questions of your hand-out!
Scope of the Problem • Maron, registry data of multiple sources (2009): • News, LexisNexis, Internet, Center of Catastrophic Sports Injury, NHLBI pathology archives, direct submissions to registry, etc. • 66 sudden cardiovascular deaths per year in USA in athletes age < 39 years, on average. • 0.6/100,000 participant-years (vs. 100/100,000 in older adults) • Harmon, sources as above, plus NCAA database (2011): • 2.3/100,000 participant-years (NCAA athletes only)
Scope of the Problem, con’t • In a patient with an unrecognized cardiac condition, the risk of SCD is 2.5 times higher if he is an athlete than a non-athlete, • Yet, more non-athletes die per year of SCD because there are more of them. • (eg., more non-athletes with HCM die, yet if you have HCM, your individual chance of death increases substantially if you are an athlete) • These are highly publicized events • NOT the most common cause of death in children
Scope of the Problem National Vital Statistic Report 2005; Maron-Harmon data
Demographics of SCD(Maron, Circ, 2009) • Males represent 89% of cases. • White = 55%, African American = 36%, Hispanic = 3%, Asian = 1% • >50% of HCM deaths are in African Americans, however, they are much less likely to be identified before they die or have an arrest: • Diminished access related to socioeconomic status? • 80% occurred during or immediately after exertion, 20% at rest.
Demographics of SCD What is the most dangerous sport?
Hypertrophic Cardiomyopathy • #1 cause of SCD in competitive athletes • General mortality: 1%/yr (higher with some types) • Mutations of 11 genes affect cardiac muscle proteins (sarcomeric proteins) • Some forms are inherited AD. • Potential Sxs: exertional syncope, palps, CP • The murmur is HCM may be soft, but tends to increase with standing or Valsalva • In what other pathologic murmur does this occur? • The EKG is abnormal in 95% of cases: ST depression and T inversion in inferior and lateral precordial leads; prominent q’s, LVH • (EKG normal in 5% of patients)
Hypertrophic Cardiomyopathy • The degree of septal hypertrophy doesn’t always correlate with the risk of sudden death • Doppler Tissue Imaging may be particularly helpful in borderline cases. • Genetic testing is helpful in some cases. • Rx: • Exercise restrictions • Meds: if symptoms: blockers and verapamil • Septal myectomy: surgical or alcohol • ICD in high risk cases (the other two haven’t been shown to decrease the risk of SCD.)
Congenital coronary anomalies • 2nd most common cause of SCD in athletes • Left coronary arising from right sinus or coronary and coursing between aortic and PA roots is the most dangerous (in 0.15% of pop) • Slit-like orifice and acute angle take off are mechanisms leading to ischemia/arrhythmias.
Congenital coronary anomalies • No prodrome in 50% • SCD more common in the young • EKG and ETT are almost always normal • Difficult echo diagnosis in 140 kg linebacker • CT/MRI are accurate • Rx: • Exercise restrictions? • Prophylactic surgery is indicated in some cases.
Arrhythmogenic Right Ventricular Cardiomyopathy • #2 cause of athlete SCD in Italy, #4 cause in USA • Mutation of desmosomes (adhesion proteins) between ventricular myocytes • Secondary fibrosis and fatty replacement of myocardium • LV is also involved late • Most variants are AD • Pathologic and EKG changes increase with age
Arrhythmogenic Right Ventricular Cardiomyopathy • EKG clues (difficult diagnosis!): • Localized prolonged QRS • Epsilon waves in V1 • T inversion, especially V2 and V3
Arrhythmogenic Right Ventricular Cardiomyopathy • Most deaths occur in adulthood (< 15% prior to 18 years) • Difficult diagnosis in childhood; MRI can help • Some gene testing is available • Rx: • Exercise restrictions • Medical suppression of arrhythmias • ICD
Long QT Syndrome • Most famous “channelopathy” • Most LQTS types are due to Na channel “gain of function” or K channel “loss of function.”
Long QT Syndrome • QTc = QT/sq rt of the preceding RR • Top normal = 440 msec in males, up to 450-460 in teen females • EKG normal in 5% of LQTS patients
Long QT Syndrome • Channelopathies as a whole cause only 3% of SCD in athletes • But, . . . , 10% of SIDS • Males particularly die early
Long QT Syndrome • Multiple types, each with different genetics (12 so far), presentations, EKG’s and some with varying treatments. • Romano Ward S: heterozygote LQT 1, 2, 3 (>90% of all cases) • Jervell Lange-Nielsen S: homozygote LQT 1, 5; associated with severe sensorineural deafness • Timothy S: LQT8; syndactyly • Andersen-Tawil S: also periodic paralysis, dysmorphisms,
Long QT Syndrome • Torsades de Pointe • Prevention and Treatment: • Exercise restrictions • Swimming precautions • Beta blockers, Na channel blockers • Pacemaker if associated with AV block • Defibrillation, isoproteronol if bradycardia related • ICD
Long QT Syndrome • Rx, con’t • Avoid QT prolonging drugs • www.sads.org • What about all the fuss over Zofran? • “Avoid Zofran in patients with congenital long QT syndrome. ECG monitoring is recommended in patients with electrolyte abnormalities (e.g., hypokalemia or hypomagnesemia), congestive heart failure, bradyarrhythmias or patients taking other medicinal products that lead to QT prolongation.” FDA
Short QT Syndrome • Another channelopathy • Gain of function of K channels (early repol) • Peaked T’s and shortened QTc, typically < 300 msec • Familial syncope, atrial and ventricular fibrillation • “Impressively lethal”
Short QT Syndrome • ICD’s problematic: with sinus tachycardia, the device interprets peaked T wave as QRS complexes and gives an inappropriate shock • Quinidine has been used
Brugada Syndrome • Another channelopathy • Loss of function of the Na channel that causes LTQ3 • EKG (V1 findings) • J point elevation • ST elevation with a coved, downward convex pattern. • AD inheritance
Brugada Syndrome • Male:Female = 8:1 • Asians • Sudden death in sleep and with bradycardia • “Lai Tai” = sleep death, in Thailand • Causes some SIDS • Arrhythmias precipitated by fever in children • Rx: • Exercise restrictions (hyperthermia concern) • Quinidine • ICD if symptomatic
Others cardiac causes of SCD that might be identifiable • WPW • CPVT: Catecholaminergic Polymorphic Ventricular Tachycardia • Idiopathic Ventricular Fibrillation • Myocarditis • Dilated cardiomyopathy • Myocardial bridge over a coronary artery • Coronary artery disease • Aortic rupture • Aortic stenosis • MVP – on the list; real disease?
CommotioCordis • 3% of all deaths in athletes • Def: sudden death due to low energy trauma to the chest wall • Impact to heart at a critical time of the cardiac cycle • No pre-existing heart disease or genetic condition, no subsequent rib fractures, homothoraax, or significant myocardial contusion • Primarily age 5-15 years. • Pig model, anesthetized animals:
CommotioCordis • Wooden ball, 30 mph, 16 msec before T wave peak • Rhythm?
CommotioCordis • Wooden ball, 30 mph, on QRS • Rhythm?
CommotioCordis Implications for our Little Leaguers?
CommotioCordis • 13 year old hit on chest by inside pitch • Immediate bystander CPR • Passing police car “fortuitous-ly” carried an AED • Normal neurologic outcome • Implications?
Pre-participation Screening • It begins with the PCP! • History, history, history! (and physical exam) • EXERTIONAL SYMPTOMS ARE BAD • American Heart Association with American College of Cardiology joint recommendations contain 12 elements (Circ, 2007) • Any positive element should result in a cardiology evaluation. • Tennessee Secondary School Athletic Association form has all 12 elements! (only 7.5 in 2010) – Wonderful!!
Pre-participation Screening • What about ancillary testing? • What one test will offer more insight at a lower cost than any other?