The Screening Debate Robert M. Campbell, MD Childrens Healthcare of - PowerPoint PPT Presentation

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

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Screening screen·ing ˈ skr ē niNG/ 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.

Children’s Healthcare of Atlanta | Emory University School of Medicine

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

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

What about clinical screening?

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest J Pediatr. 2016 Aug 5. pii: S0022-3476(16)30518-2. doi: 10.1016/j.jpeds.2016.06.088. [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 2014. RESULTS: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 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.

What about ECG screening?

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):1593-601.

An Electrocardiogram Should Not Be Included in routine Preparticipation Screening of Young Athletes Bernard R. Chaitman Circulation 2007;116;2610-2615. Electrocardiograms Should Be Included in Preparticipation Screening of Athletes Robert J. Myerburg and Victoria L. Vetter Circulation 2007;116;2616-2626.

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.

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

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

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

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?

What about echocadiography screening?

Echocardiogram

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

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

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

We all want the same thing but see from different perspectives