Sudden Cardiac Death in Children

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Sudden Cardiac Death in Children

  1. 1. Coarctation of the Aorta <ul><li>postoperative hypertension noted beyond the 10th postoperative yr: </li></ul><ul><li>-- alive and well and normotensive </li></ul><ul><li>70% at 10 yrs </li></ul><ul><li>65% at 15 yrs </li></ul><ul><li>20% at 25 yrs. </li></ul><ul><li>arm leg gradient with exercise average is 80 mm Hg. </li></ul>
  2. 2. SUDDEN DEATH in YOUNG ATHLETES Maron, et al, Circ 1980
  3. 3. Clinical Findings <ul><li>asymptomatic 21/29 </li></ul><ul><li>syncope 3/29 </li></ul><ul><li>presyncope 1/29 </li></ul><ul><li>chest pain 2/29 </li></ul><ul><li>mild fatigue 2/29 </li></ul>
  4. 4. Circumstances of Death <ul><li>death during or after severe exertion: 22/29 </li></ul><ul><li>death occurred during mild exertion: 2/29 </li></ul><ul><li>death occurred during sedentary activity: 5/29 </li></ul>
  5. 5. Causes of Sudden Death 29 Probable CV Disease 6 5 1 Idiopathic Concentric hypertrophy (no fiber disarray) Hypoplastic coronaries 1 No CV disease 22 Unequivocal CV dis. 2 Ruptured aorta 3 Atherosclero. CA 3 ALCAPA 14 HOCM**
  6. 6. Magnitude of the Problem <ul><li>excluding trauma, cardiac death is the most frequent cause of sports related death. </li></ul><ul><li>5/100,000 have a condition which predisposes them to sudden death. </li></ul><ul><li>1/200,000 athletes per yr have sudden death </li></ul><ul><li>~12 high school ath. die/yr in U.S. </li></ul>
  7. 7. Types of Sports <ul><li>basketball 33% </li></ul><ul><li>football 20% </li></ul><ul><li>running 16% </li></ul><ul><li>swimming 4.8% </li></ul><ul><li>wrestling 3.8% </li></ul><ul><li>volleyball 2.9% </li></ul><ul><li>tennis 2.9% </li></ul><ul><li>baseball 2.9% </li></ul><ul><li>GOLF<1% </li></ul>
  8. 8. Hypertrophic Cardiomyopathy and Sudden Death <ul><li>Annual mortality rate 2-4% </li></ul><ul><li>Mechanism probably acute dysrhythmia(v.tach, v.fib., asystole) </li></ul><ul><li>Sudden death most common 10-25 yrs. </li></ul><ul><li>Peak age is 14 yrs. </li></ul><ul><li>Approx. 40% occur during ahtletics </li></ul><ul><li>If there is documented v. tach on holter,death rate 8%. </li></ul>
  9. 9. HCM and Sudden Death <ul><li>Increased risk of sudden death ass. with: documented v. tach, family hx. of sudden death, young age of onset of symptoms. </li></ul><ul><li>Sudden death not related to presence or degree of outflow gradient. </li></ul><ul><li>NO INTERVENTION(SURG,MEDICAL) </li></ul><ul><li>HAS BEEN SHOWN TO DECREASE RISK OF SUDDEN DEATH. </li></ul>
  10. 10. Abnormal Origin of CA’s and Sudden Death <ul><li>Left CA from right cusp is the most common cause of sudden death. </li></ul><ul><li>Potential mechanisms: coronary comes off tangentially from the aorta, ostium may be slit like,ostium may be partially covered by flap valve, initial few mm’m may be in wall of aorta. </li></ul><ul><li>97% die at < 22 yrs of age </li></ul><ul><li>Rule out in pat with exercise chest pain or syncope . Tx. surgical </li></ul>
  11. 11. OTHER CAUSES of SUDDEN DEATH in ATHLETES <ul><li>Marfan Syndrome: related to aortic rupture. </li></ul><ul><li>Myocarditis: may be associated with acute inflammation and chronic multifocal scarring-- arrthymias </li></ul><ul><li>Drugs: anabolic steroids predispose to thrombotic MI, CVA, and cardiomyopathy. COCAINE </li></ul>
  12. 12. Other Causes, <ul><li>Primary dysrhythmias: </li></ul><ul><li>a. sudden death reported with SVT,long QT, SSS. </li></ul><ul><li>b. exercise syncope most common presentation. </li></ul>
  13. 13. SCREENING? <ul><li>Scale: to identify 1000 atheletes at risk, 200,000 would have to be screened to prevent 1 death. </li></ul><ul><li>Routine screening by ECHO impractical </li></ul><ul><li>Routine EKG’s on all athletes probably impractical. </li></ul>
  14. 14. SCREENING? <ul><li>SMA 1: history and PE </li></ul><ul><li>focused hx of syncope, chest pain, or seizures in patient- always ask about sudden death in family members </li></ul><ul><li>focused PE looking for path. murmur, gallop, or S4, obvious ectopy </li></ul>
  15. 15. LONG TERM EXPERIENCE AFTER CARDIAC SURGERY <ul><li>60% of important CHD: </li></ul><ul><li>VSD </li></ul><ul><li>ASD </li></ul><ul><li>PS </li></ul><ul><li>PDA </li></ul><ul><li>CoA </li></ul>
  16. 16. Long Term Experience, <ul><li>Surgery for uncommon lesions- has been available for 25 yrs. </li></ul><ul><li>TGA </li></ul><ul><li>TA </li></ul><ul><li>Single ventricle </li></ul><ul><li>These patients are now showing up in adult clinics. </li></ul>
  17. 17. RESIDUAE & SEQUELAE of CONGENITAL HEART SURGERY It ain’t over, til it’s over
  18. 18. Surgical Residuae & Sequelae <ul><li>Obstructive lesions </li></ul><ul><li>Hypertension </li></ul><ul><li>Shunts </li></ul><ul><li>pulm. artery hypertension/ distortion </li></ul><ul><li>valve regurg </li></ul>
  19. 19. Surgical Residuae & Sequelae <ul><li>Arrhythmias </li></ul><ul><li>Systemic right ventricle- TGA </li></ul><ul><li>Mustard or Senning </li></ul><ul><li>Fontan physiology- physiologic correction with single ventricle chamber </li></ul>
  20. 20. Coarction of the Aorta <ul><li>50-85% incidence of bicuspid Ao valve. </li></ul><ul><li>-- Late developement of stenosis/insuf- </li></ul><ul><li>ficiency. </li></ul><ul><li>Associated with calcific changes </li></ul><ul><li>midlife event </li></ul><ul><li>-- infective endocarditis </li></ul><ul><li>>50% have mitral abnormalities </li></ul>
  21. 21. Coarctation of the Aorta <ul><li>Associated abnormalities: </li></ul><ul><li>-- intracranial aneurysms </li></ul><ul><li>-- late aortic dissection </li></ul><ul><li>-- intramural coronary artery disease </li></ul>
  22. 22. Coarctation of the Aorta Aortic aneurysms <ul><li>With dacron onlay patches </li></ul><ul><li>-- 38% incidence of aneurysms </li></ul><ul><li>Aortic balloon angioplasty </li></ul><ul><li>-- incidence of aneurysms unknown </li></ul><ul><li>native vs recoarc. For recoarctation, </li></ul><ul><li>balloon is procedure of choice </li></ul>
  23. 23. Coarctation of the Aorta <ul><li>Surgical results; aim for gradient < 10 </li></ul><ul><li>30-40% have recurrent gradient when surgery done at less than 1yr. </li></ul><ul><li>Significant late mortality- </li></ul><ul><li>--10-20% have resting hypertension </li></ul><ul><li>This is directly related to age at surgery.Exercise testing will provock gradient. </li></ul>

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