Key takeaways

  • All children should be evaluated for conditions predisposing them to sudden cardiac arrest (SCA) in the course of routine health care.

  • A thorough and detailed history, family history, and physical examination are necessary to begin assessing SCA risk.

  • The ECG (electrocardiogram) should be the first test ordered when there is a concern for SCA risk. The ECG should be interpreted by a physician trained in recognizing electrical heart disease (i.e., a pediatric cardiologist or pediatric electrophysiologist).

What are cardiomyopathies?

  • Dilated cardiomyopathy: Enlarged, dilated left and/or right ventricle with or without decreased systolic function.

  • Hypertrophic cardiomyopathy (HCM): Abnormally thickened ventricular myocardium without cause (e.g., hypertension, coarctation, aortic stenosis, etc). HCM is reported as the most common cause of SCA and sudden cardiac death (SCD) in young athletes.

  • Restrictive cardiomyopathy: Normal to thickened ventricular walls and normal ventricular size with impaired diastolic function and often with dilated atria.

  • Arrhythmogenic cardiomyopathy (includes arrhythmogenic right ventricular cardiomyopathy [ACM]): Enlarged, dilated right ventricle with or without decreased systolic function often associated with frequent arrhythmias (can be seen in the left ventricle as well).

  • Unclassified cardiomyopathies: This includes left ventricular noncompaction (the left ventricular myocardium is abnormal with hypertrabeculation and crypt formation of the left ventricular wall with thickened, normal, or thinned and dilated myocardium with or without impaired systolic function).

What are channelopathies?

  • Long QT syndrome (LQTS): Prolongation of the corrected QT interval (QTc) with abnormalities in T-wave morphology, some of which are associated with specific genotypes. Sudden infant death syndrome (SIDS) may be attributable to LQTS in approximately 10% of cases.

  • Short QT syndrome: Extremely rare condition with abnormal shortening of the QTc with prominent and peaked T waves.

  • Brugada syndrome (BrS): Associated with a coved and elevated ST elevation in ECG leads V1 and V2. Specific Brugada T-wave morphologies may indicate an elevated risk of cardiac arrest.

  • Catecholaminergic polymorphic ventricular tachycardia (CPVT): Resting ECGs in these patients are almost always normal. CPVT is mostly identified with exercise testing that results in increased ventricular ectopy and even polymorphic ventricular tachycardia (VT).

  • Idiopathic ventricular fibrillation (IVF): Patients presenting with ventricular fibrillation (VF) in whom known cardiac, respiratory, metabolic, and toxicological etiologies have been excluded through clinical evaluation. The term IVF is used when cardiac arrest remains unexplained despite this investigation.

Common channelopathies 

What are some of the questions asked during a heart screening?

  • Have you ever fainted, passed out or had an unexplained seizure suddenly and without warning, especially during exercise or in response to sudden loud noises such as doorbells, alarm clocks and ringing telephones?

  • Have you ever had exercise-related chest pain or shortness of breath?

  • Has anyone in your immediate family (parents, grandparents, siblings) or other more distant relatives (aunts, uncles, cousins) died of heart problems or had an unexpected sudden death before age 50? This would include unexpected drownings, unexplained car accidents in which the relative was driving or sudden infant death syndrome.

  • Are you related to anyone with hypertrophic cardiomyopathy or hypertrophic obstructive cardiomyopathy, Marfan syndrome, arrhythmogenic right ventricular cardiomyopathy, long QT syndrome, short QT syndrome, Brugada syndrome or catecholaminergic polymorphic ventricular tachycardia, or anyone younger than 50 years with a pacemaker or implantable defibrillator?

SOURCEPediatrics (2021) 148 (1): e2021052044.
See also: Blog by Pediatric Cardiology Expert, Victoria Vetter, MD, FAAP, FACC, Children's Hospital of Philadelphia


Further reading 

Read more about Cardiac Emergency Response Planning for Schools in the September 2016 issue of National Association of School Nurses (NASN):

School implementation resources


Evaluation materials