title: Sudden death with circumferential subepicardial fibrofatty replacement: left-sided arrhythmogenic ventricular cardiomyopathy.
abstract: We report 5 cases of sudden cardiac death, with similar cardiac findings. All 5 cases had circumferential left ventricular subepicardial fibrofatty replacement of the myocardium, similar to the histologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC). In these cases, the findings were predominantly in the left ventricle with minimal or no involvement of the right ventricle. Four of the 5 cases had siblings with either sudden death or cardiac symptoms. This report highlights 5 cases of sudden death in the young with histologic findings similar to ARVC, with predominant left ventricular involvement and questions whether the cases represent a larger spectrum of the cardiomyopathy known as ARVC, which perhaps should be more correctly termed as "arrhythmogenic cardiomyopathy" or represent a separate, potentially inheritable cardiomyopathic entity. We report these cases to familiarize forensic pathologists with this uncommon and potentially inheritable condition.
authors.affiliation: Jesse E Edwards Registry of Cardiovascular Disease, John Nasseff Heart Hospital, Saint Paul, MN 55102, USA.
authors.forename: Shannon M
authors.initials: SM
authors.lastname: Mackey-Bojack
authors.forename: Susan J
authors.initials: SJ
authors.lastname: Roe
authors.forename: Jack L
authors.initials: JL
authors.lastname: Titus
mesh_terms: D001145:Arrhythmias, Cardiac / Q000209:etiology; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000145:classification / Q000175:diagnosis* / Q000235:genetics; D003645:Death, Sudden / Q000209:etiology; D005355:Fibrosis; D049429:Forensic Pathology; D006327:Heart Block / Q000209:etiology; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D009154:Mutation; D009206:Myocardium / Q000473:pathology*