Grant: Travel Awards - Institute Community Support

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable structural heart disease that predisposes individuals to sudden cardiac arrest (SCA), and has been recognized as an important cause of sport-related SCA. Exercise can worsen the disease, thus identifying individuals (through preparticipation screening) has important implications for the prevention of SCA. Guidelines suggest the electrocardiogram (ECG) is 80% sensitive for detecting ARVC; however, this sensitivity has not been evaluated in young populations. We examined the sensitivity and specificity of the ECG in detecting ARVC in adolescents with known ARVC versus healthy aged-matched controls. Methods: Two expert reviewers reviewed a total of 71 de-identified ECGs of ARVC patients (all diagnoses were confirmed using 2010 Task Force Criteria; n=49), and controls (confirmed negative ARVC patients, most were relatives of ARVC positive patients; n=22), in patients aged 7-18. ECG's were recorded as either normal (using 2016 international criteria for athletes) or abnormal (abnormalities were documented if present). Results: There were 49 patients with either confirmed positive/borderline ARVC, 32 males and 17 females, with a mean age of 15.5+/-2.5 years. Among 21 patients with ARVC and symptoms, 6 (29%) had abnormal ECG findings consistent with ARVC; among 28 patients with ARVC and no prior symptoms, 7 (25%) had findings consistent with ARVC. Among patients with symptoms, 15 had fainting, 5 had ventricular tachycardia (a fast heart rhythm) and 1 had palpitations. Of patients without symptoms, 21 were referred for positive family history versus 7 for incidental findings on physical exam or ECG. Of 14 patients that were gene positive, 5 had an abnormal ECG. All control patients had normal ECGs. Conclusions: Using the 12-lead ECG to detect ARVC in adolescents is less sensitive than previously thought. The ECG in pre-participation screening of young athletes for ARVC may be unreliable.