The Screening Debate. Robert M. Campbell, MD Children s Healthcare of Atlanta Emory University School of Medicine

Transcription

1 The Screening Debate Robert M. Campbell, MD Children s Healthcare of Atlanta Emory University School of Medicine

2 No Disclosures

3 Screening screen ing ˈskrēniNG/ noun noun: screening; plural noun: screenings 1. a showing of a movie, video, or television program. 2. the evaluation or investigation of something as part of a methodical survey, to assess suitability for a particular role or purpose.

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7 Children s Healthcare of Atlanta Emory University School of Medicine

8 What are we trying to find? Children s Healthcare of Atlanta Emory University School of Medicine

9 SCA Differential Diagnosis - Genetic Structural/Functional HCM Other CM (DCM, RCM, ARVC, LVNC) Coronary Artery Anomalies Aortic Rupture/Marfan Myocarditis Left Ventricular Outflow Tract Obstruction Mitral Valve Prolapse (MVP) Coronary Artery Atherosclerotic Disease Postoperative Congenital Heart Disease Electrical Long QT Syndrome (LQTS) Wolff-Parkinson-White Syndrome (WPW) Brugada Syndrome Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Short QT Syndrome Complete Heart Block (CHB) Other Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

10 What about clinical screening?

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13 Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest J Pediatr Aug 5. pii: S (16) doi: /j.jpeds [Epub ahead of print] Dalal A, Czosek RJ, Kovach J, von Alvensleben JC, Valdes S, Etheridge SP, Ackerman MJ, Auld D, Huckaby J, McCracken C, Campbell R. Abstract OBJECTIVES: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R). STUDY DESIGN: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to RESULTS: A total of 450 patients (257 male/193 female; median age 10.1 years [ years, 25th-75th percentiles]) were enrolled. Patient age was 13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT). CONCLUSION: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest.

14 What about ECG screening?

15 WHAT IF THIS NUMBER WERE NOT CORRECT? Trends in sudden cardiovascular death in young competitive athletes after implementation of a preparticipation screening program. Corrado D, Basso C, Pavei A, Michiel P, Shiavon M, and Thiene G. JAMA, 2006 Oct 4 296(13):

16 An Electrocardiogram Should Not Be Included in routine Preparticipation Screening of Young Athletes Bernard R. Chaitman Circulation 2007;116; Electrocardiograms Should Be Included in Preparticipation Screening of Athletes Robert J. Myerburg and Victoria L. Vetter Circulation 2007;116;

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21 AHA/ACCF Scientific Statement Assessment of the 12-Lead Electrocardiogram as a Screening Test for Detection of Cardiovascular Disease in General Healthy Populations of Young People (12-22 Years of Age) Barry J. Maron, MD, Chair; Richard A. Friedman, MD, Vice-Chair; Paul Kligfield, MD; Benjamin D. Levine, MD; Sami Viskin, MD; Bernard R. Chaitman, MD; Peter M. Okin, MD; J. Philip Saul, MD; Lisa Salberg; George F. Van Hare, MD; Elsayed Z. Soliman, MD; Jersey Chen, MD, MPH; Paul Matherne, MD; Steven F. Bolling, MD; Matthew J. Mitten, JD; Arthur Caplan, PhD; Gary J. Balady, MD; and Paul D.

22 SCA Differential Diagnosis Expected ECG Findings Structural/Functional HCM Other CM (DCM, RCM, ARVC, LVNC) Coronary Artery Anomalies Aortic Rupture/Marfan Myocarditis Left Ventricular Outflow Tract Obstruction Mitral Valve Prolapse (MVP) Coronary Artery Atherosclerotic Disease Postoperative Congenital Heart Disease Electrical Long QT Syndrome (LQTS) Wolff-Parkinson-White Syndrome (WPW) Brugada Syndrome Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Short QT Syndrome Complete Heart Block (CHB) Other Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

23 SCA Differential Diagnosis Expected ECG Findings Possible ECG Findings Structural/Functional HCM Other CM (DCM, RCM, ARVC, LVNC) Coronary Artery Anomalies Aortic Rupture/Marfan Myocarditis Left Ventricular Outflow Tract Obstruction Mitral Valve Prolapse (MVP) Coronary Artery Atherosclerotic Disease Postoperative Congenital Heart Disease Electrical Long QT Syndrome (LQTS) Wolff-Parkinson-White Syndrome (WPW) Brugada Syndrome Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Short QT Syndrome Complete Heart Block (CHB) Other Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

24 SCA Differential Diagnosis Expected ECG Findings Possible ECG Findings Structural/Functional HCM Other CM (DCM, RCM, ARVC, LVNC) Coronary Artery Anomalies Aortic Rupture/Marfan Myocarditis Left Ventricular Outflow Tract Obstruction Mitral Valve Prolapse (MVP) Coronary Artery Atherosclerotic Disease Postoperative Congenital Heart Disease Electrical Long QT Syndrome (LQTS) Wolff-Parkinson-White Syndrome (WPW) Brugada Syndrome Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Short QT Syndrome Complete Heart Block (CHB) Other Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

25 10 issues to be addressed before ECG screening. 1. Who to screen? 2. When to screen? 3. Who performs screen and interpretation? 4. Where to screen? 5. What are the validated normal ECG values? 6. Overread? vs Underread? 7. Follow-up after positive ECG screen? 8. Database for ECG screen outcomes? 9. Is ECG screening suggested or mandated? 10. Costs?

26 What about echocadiography screening?

27 Echocardiogram

28 Sudden Cardiac Death (SCD): Differential Diagnosis Expected Echo Findings Structural/Functional 1) Hypertrophic Cardiomyopathy (HCM) 2) Coronary Artery Anomalies 3) Aortic Rupture/Marfan 4) Dilated Cardiomyopathy (DCM) 5) Myocarditis 6) Left Ventricular Outflow Tract Obstruction 7) Mitral Valve Prolapse (MVP) 8) Coronary Artery Atherosclerotic Disease 9) Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) 10) Post-operative Congenital Heart Disease Electrical 11) Long QT Syndrome (LQTS) 12) Wolff-Parkinson-White Syndrome (WPW) 13) Brugada Syndrome 14) Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) 15) Short QT Syndrome 16) CHB Complete Heart Block Other 17) Drugs and Stimulants 18) Primary Pulmonary Hypertension (PPH) 19) Commotio Cordis Children s Healthcare of Atlanta Emory University School of Medicine

29 Summary We all want to prevent SCD First step is to screen to identify patients and families at risk for SCA No screening technique is currently perfect Children s Healthcare of Atlanta Emory University School of Medicine

30 Primary Prevention Diagnose Treat Secondary Prevention EAP, CPR/AED Chain of Survival Non viable SCD SCA (witnessed, unwitnessed) SCD Viable ( healthy dead ) Hospital discharge Neuro intact Return to work

31 We all want the same thing but see from different perspectives

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