title: Distribution of biventricular disease in arrhythmogenic cardiomyopathy: an autopsy study.
abstract: Arrhythmogenic cardiomyopathy is a rare cardiomyopathy characterized by fibrofatty replacement primarily of the right ventricular myocardium. It is a major cause of sudden death in the young and in athletes. There are few autopsy studies of the ventricular distribution of the disease. Fifty cases of sudden cardiac death with fibrofatty replacement in either ventricle from a single medical examiner's office were studied. Distribution of disease as determined grossly and microscopically was correlated with activity at time of death, race, and presence of inflammation. Extent of disease was right ventricular in 6 cases (12%; age, 25 ± 5 years), biventricular in 25 (50%; age, 36 ± 3 years), and left ventricular in 19 (38%; age, 37 ± 3 years) (P = .13). Inflammation was present in 44% of biventricular arrhythmogenic cardiomyopathy versus 74% of left ventricular arrhythmogenic cardiomyopathy and 83% of right ventricular arrhythmogenic cardiomyopathy (P = .06). Arrhythmogenic cardiomyopathy, when presenting with sudden death, is usually biventricular. There is a trend that univentricular involvement occurs at an earlier age and that right ventricular involvement shows more inflammation, suggesting different stages of disease.
authors.affiliation: Department of Pathology, Escola Paulista de Medicina, UNIFESP, Sao Paulo 04023-900, Brazil.
authors.forename: Fabio
authors.initials: F
authors.lastname: Tavora
authors.forename: Mingchang
authors.initials: M
authors.lastname: Zhang
authors.forename: Marcello
authors.initials: M
authors.lastname: Franco
authors.forename: Joao Bosco
authors.initials: JB
authors.lastname: Oliveira
authors.forename: Ling
authors.initials: L
authors.lastname: Li
authors.forename: David
authors.initials: D
authors.lastname: Fowler
authors.forename: Ziqin
authors.initials: Z
authors.lastname: Zhao
authors.forename: Nathaniel
authors.initials: N
authors.lastname: Cresswell
authors.forename: Allen
authors.initials: A
authors.lastname: Burke
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000401:mortality / Q000473:pathology*; D001344:Autopsy; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D005260:Female; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D012189:Retrospective Studies; D055815:Young Adult