1. Coarctation of the Aorta F postoperative hypertension noted beyond the 10th postoperative yr: -- alive and well and normotensive -- alive and well and normotensive 70% at 10 yrs 70% at 10 yrs 65% at 15 yrs 65% at 15 yrs 20% at 25 yrs. 20% at 25 yrs. F arm leg gradient with exercise average is 80 mm Hg.

2. SUDDEN DEATH in YOUNG ATHLETES Maron, et al, Circ 1980

3. Clinical Findings F asymptomatic 21/29 F syncope 3/29 F presyncope 1/29 F chest pain 2/29 F mild fatigue 2/29

4. Circumstances of Death F death during or after severe exertion: 22/29 F death occurred during mild exertion: 2/29 F death occurred during sedentary activity: 5/29

5. Causes of Sudden Death 2929 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 3ALCAPA 14 HOCM**

6. Magnitude of the Problem F excluding trauma, cardiac death is the most frequent cause of sports related death. F 5/100,000 have a condition which predisposes them to sudden death. F 1/200,000 athletes per yr have sudden death F ~12 high school ath. die/yr in U.S.

7. Types of Sports F basketball 33% F football 20% F running 16% F swimming 4.8% F wrestling 3.8% F volleyball 2.9% F tennis 2.9% F baseball 2.9% GOLF<1%

8. Hypertrophic Cardiomyopathy and Sudden Death F Annual mortality rate 2-4% F Mechanism probably acute dysrhythmia(v.tach, v.fib., asystole) F Sudden death most common 10-25 yrs. F Peak age is 14 yrs. F Approx. 40% occur during ahtletics F If there is documented v. tach on holter,death rate 8%.

9. HCM and Sudden Death Increased risk of sudden death ass. with: documented v. tach, family hx. of sudden death, young age of onset of symptoms. Sudden death not related to presence or degree of outflow gradient. NO INTERVENTION(SURG,MEDICAL) HAS BEEN SHOWN TO DECREASE RISK OF SUDDEN DEATH.

10. Abnormal Origin of CA’s and Sudden Death F Left CA from right cusp is the most common cause of sudden death. F 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. F 97% die at < 22 yrs of age F Rule out in pat with exercise chest pain or syncope. Tx. surgical

11. OTHER CAUSES of SUDDEN DEATH in ATHLETES F Marfan Syndrome: related to aortic rupture. F Myocarditis: may be associated with acute inflammation and chronic multifocal scarring-- arrthymias F Drugs: anabolic steroids predispose to thrombotic MI, CVA, and cardiomyopathy. COCAINE

12. Other Causes, F Primary dysrhythmias: a. sudden death reported with SVT,long QT, SSS. a. sudden death reported with SVT,long QT, SSS. b. exercise syncope most common presentation. b. exercise syncope most common presentation.

13. SCREENING? F Scale: to identify 1000 atheletes at risk, 200,000 would have to be screened to prevent 1 death. F Routine screening by ECHO impractical F Routine EKG’s on all athletes probably impractical.

14. SCREENING? F SMA 1: history and PE focused hx of syncope, chest pain, or seizures in patient- always ask about sudden death in family members focused hx of syncope, chest pain, or seizures in patient- always ask about sudden death in family members focused PE looking for path. murmur, gallop, or S4, obvious ectopy focused PE looking for path. murmur, gallop, or S4, obvious ectopy

15. LONG TERM EXPERIENCE AFTER CARDIAC SURGERY F 60% of important CHD: VSD VSD ASD ASD PS PS PDA PDA CoA CoA

16. Long Term Experience, F Surgery for uncommon lesions- has been available for 25 yrs. TGA TGA TA TA Single ventricle Single ventricle F These patients are now showing up in adult clinics.

17. RESIDUAE & SEQUELAE of CONGENITAL HEART SURGERY It ain’t over, til it’s over

18. Surgical Residuae & Sequelae F Obstructive lesions F Hypertension F Shunts F pulm. artery hypertension/ distortion F valve regurg

19. Surgical Residuae & Sequelae F Arrhythmias F Systemic right ventricle- TGA Mustard or Senning Mustard or Senning F Fontan physiology- physiologic correction with single ventricle chamber

20. Coarction of the Aorta F 50-85% incidence of bicuspid Ao valve. -- Late developement of stenosis/insuf- -- Late developement of stenosis/insuf- ficiency. ficiency. Associated with calcific changes Associated with calcific changes midlife event midlife event -- infective endocarditis -- infective endocarditis F >50% have mitral abnormalities

21. Coarctation of the Aorta Associated abnormalities: -- intracranial aneurysms -- intracranial aneurysms -- late aortic dissection -- late aortic dissection -- intramural coronary artery disease -- intramural coronary artery disease

22. Coarctation of the Aorta Aortic aneurysms F With dacron onlay patches -- 38% incidence of aneurysms -- 38% incidence of aneurysms F Aortic balloon angioplasty -- incidence of aneurysms unknown -- incidence of aneurysms unknown native vs recoarc. For recoarctation, native vs recoarc. For recoarctation, balloon is procedure of choice balloon is procedure of choice

23. Coarctation of the Aorta F Surgical results; aim for gradient < 10 30-40% have recurrent gradient when surgery done at less than 1yr. 30-40% have recurrent gradient when surgery done at less than 1yr. F Significant late mortality- --10-20% have resting hypertension --10-20% have resting hypertension This is directly related to age at surgery.Exercise testing will provock gradient. This is directly related to age at surgery.Exercise testing will provock gradient.