SUDDEN CARDIAC DEATH IN CHILDREN & ADOLESCENTS JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN

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1 JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN CARDIOMYOPATHIES: HYPERTROPHIC, DILATED, OR JUST AN ATHLETE? A NTHONY C. CHANG, MD, MBA, MPH D IRECTOR, HEART I NSTITUTE, CHOC Causes HCM most common in U.S. but not in Italy Maron BJ and Zipes DP (2005). 36 th Bethesda Conference. JACC 45(8): January 14-15, 2011 SCA Conference 1

2 Causes C A B Which activity leads to the largest % increase in LV wall thickness? E D January 14-15, 2011 SCA Conference 2

3 Hypertrophic Cardiomyopathy/ Genetics Be vigilent for apical type HCM Hypertrophic Cardiomyopathy/ Echo Maximal LV wall Maximal LV wall thickness 13-15mm January 14-15, 2011 SCA Conference 3

4 Hypertrophic Cardiomyopathy/ EKG ST changes and inverted T waves Hypertrophic Cardiomyopathy/ Types Be vigilent for apical type HCM January 14-15, 2011 SCA Conference 4

5 Hypertrophic Cardiomyopathy/ Apical Type Deep inverted T waves Hypertrophic Cardiomyopathy/ vs. Athlete Late gadolinium enhancement MRI (LGE-MRI) can be helpful. January 14-15, 2011 SCA Conference 5

6 A Which does not belong to the others? C B Arrhythmogenic RV Cardiomyopathy/ History Personal communication with FI Marcus, January January 14-15, 2011 SCA Conference 6

7 Arrhythmogenic RV Cardiomyopathy/ Genetic Arrhythmogenic RV Cardiomyopathy/Anatomy Marcus FI et al. Arrhythmogenic RV Dysplasia. Springer, New York, January 14-15, 2011 SCA Conference 7

8 Arrhythmogenic RV Cardiomyopathy/ Cell Marcus FI et al. Arrhythmogenic RV Dysplasia. Springer, New York, Arrhythmogenic RV Cardiomyopathy/ Criteria 1994 vs 2010 criteria Marcus FI. Circulation 2010; 121: January 14-15, 2011 SCA Conference 8

9 Arrhythmogenic RV Cardiomyopathy/ EKG Inverted T waves V1-3,4 and epsilon waves Arrhythmogenic RV Cardiomyopathy/ EKG LBBB pattern tachycardia Marcus FI et al. Arrhythmogenic RV Dysplasia. Springer, New York, January 14-15, 2011 SCA Conference 9

10 Arrhythmogenic RV Cardiomyopathy/Imaging Marcus FI et al. Arrhythmogenic RV Dysplasia. Springer, New York, Cardiomyopathies/ Summary Type Incidence %Sudden Diagnosisi Therapy Death Hypertrophic 2.0: 1,000 ARVC/D : 1,000 ~25-35 ECG/Echo/ MRI/CPX Β Blockade and AICD ~5-17 ECG/MRI Β Blockade and AICD Dilated 0.4-1: <5 Eh Echo ACEi and 1,000 diuretics Restrictive Rare NA ECG/Echo Transplant Minneapolis Heart Institute Foundation January 14-15, 2011 SCA Conference 10

11 Conclusion -The myriad of cardiomyopathies can all manifest as sudden cardiac death in the young athlete, but the most common is hypertrophic cardiomyopathy, which at times can be difficult to distinguish from remodeling in athlete s heart. - Among differentiating aspects for athlete s heart are: higher max VO2 with exercise testing, no gadolinium delayed enhancement on CMR, and decrease in LV mass with deconditioning. Conclusion -Arrhythmogenic A h h i RV cardiomyopathy/ dysplasia (ARVC/D) is a very under-appreciated clinical entity in young athletes and can be very elusive as a diagnosis (especially in its early form). - The revised 2010 criteria by the Task Force includes quantitative criteria and accommodates emerging diagnostic modalities and advances in genetic analysis. January 14-15, 2011 SCA Conference 11

12 JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN CARDIOMYOPATHIES: HYPERTROPHIC, DILATED, OR JUST AN ATHLETE? A NTHONY C. CHANG, MD, MBA, MPH D IRECTOR, HEART I NSTITUTE, CHOC JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN CARDIOMYOPATHIES: HYPERTROPHIC, ARVC/D, OR JUST AN ATHLETE? A NTHONY C. CHANG, MD, MBA, MPH D IRECTOR, HEART I NSTITUTE, CHOC January 14-15, 2011 SCA Conference 12

13 January 14-15, 2011 SCA Conference 13

SUDDEN CARDIAC DEATH IN CHILDREN & ADOLESCENTS JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN

SUDDEN CARDIAC DEATH IN CHILDREN & ADOLESCENTS JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN JANUARY 14-15, 2011 DISNEY S GRAND CALIFORNIAN ECG SCREENING SHOULD BE MANDATORY IN ALL SCHOOL AGED CHILDREN- PRO A NTHONY C. CHANG, MD, MBA, MPH D IRECTOR, HEART I NSTITUTE, CHOC St. Camillus Best Child

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