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

No Disclosures

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.

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

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

What are we trying to find?

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)

SCA Differential Diagnosis - Genetic

Other

Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

What about clinical screening?

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.

Clinical Presentation of Pediatric Patients at Risk for Sudden Cardiac Arrest

What about ECG screening?

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.

WHAT IF THIS NUMBER WERE NOT CORRECT?

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.

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)

SCA Differential Diagnosis

Other

Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

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)

SCA Differential Diagnosis

Other

Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

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)

SCA Differential Diagnosis

Other

Drugs and Stimulants Primary Pulmonary Hypertension (PPH) Commotio Cordis

Expected ECG Findings Possible ECG Findings

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

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

Sudden Cardiac Death (SCD): Differential Diagnosis

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

Expected Echo Findings

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

Primary Prevention

Diagnose Treat

Secondary Prevention

EAP, CPR/AED Chain of Survival

SCA (witnessed, unwitnessed) Non viable

SCD

Viable (“healthy dead”) Hospital discharge Neuro intact Return to work

SCD

We all want the same thing but see from different perspectives