[322] The Biventricular Form of Arrhythmogenic Cardiomyopathy Is the Most frequent Morphologic Substrate of Juvenile Sudden Cardiac Death in Sports

Massimiliano Mancini, Cira RT di Gioia, Rocco Ierino, Carmela Preziuso, Cristian D'Ovidio, Pietro Gallo, Vincenzo Petrozza, Carla Giordano, Giulia d'Amati. Sapienza, University of Rome, Rome, Italy; "G. D'Annunzio" University, Chieti, Italy

Background: Juvenile cardiac sudden death (SD) can occur during sport activity, either recreational or competitive. Physical effort triggers sudden death in athletes affected by cardiac disease presisposing to life-threatening arrhythmias. Thus, a more specific understanding of the cardiac substrates of SD is helpful to guide prevention programs. Our aim was to investigate the morphologic substrates of exertional juvenile cardiac SD.
Design: We reviewed 266 consecutive cases of juvenile sudden cardiac death (≥1 yr.≤40 yrs.) referred to our Department from January 2000 to May 2012. Accurate gross description was available for each heart; the diagnosis was based on macroscopic features and systematic microscopic analysis of the coronary arteries, valves and myocardium based on a detailed study protocol. According to the circumstances, sudden deaths were divided into: i) events occurring during or immediately after sports; ii) events at rest or during normal daily activities.
Results: Thirty-seven (14%) deaths occurred during sports (6 competitive and 31 non competitive athletes). There were 33 males and 4 females, with a mean age of 24.6 yrs. (range 10-38 yrs.). The sport most frequently involved was soccer (51% of cases). Toxicology was available in 24/37 cases, and was positive in one (cannabinoids). The most frequent cause of death was arrhythmogenic cardiomyopathy (ACM, 7/37,19%). All ACM cases showed a biventricular pattern, often with prevalent involvement of the left ventricle (2/7, 29%). Other morphologic substrates were atherosclerotic coronary artery disease (CAD, 4/37, 11%), congenital valvular disease (4/37,11%), congenital anomalies of coronary arteries (3/37, 8%), hypertrophic cardiomyopathy (HCM, 3/37, 8%), conduction tissue disease (2/37, 5%), myocarditis (2/37 5%), coronary artery vasculitis (1/37,3%), cardiac amartoma (1/37 3%). In 10/37 cases (27%) the findings were non-specific left ventricular hypertrophy (11%) or structurally normal heart (16%).
Conclusions: According to our results, the biventricular form of ACM is the most frequent morphologic substrate of juvenile sudden cardiac death in sports. In one third of cases the fibro-fatty myocardial replacement is limited to mild changes almost exclusively affecting the left ventricle.
Category: Cardiovascular

Wednesday, March 6, 2013 9:30 AM

Poster Session V # 51, Wednesday Morning

 

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