title: Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/or dysplasia.
abstract: BACKGROUND
  Sudden death is a possible consequence of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Prevalence of ARVC/D in unexpected sudden cardiac death (USCD), however, remains imprecise, as do circumstances of death and ARVC/D-associated gross and microscopic findings, especially His bundle anomalies.
  
  
  RESULTS
  We reviewed 14 000 forensic autopsies required by judicial authorities from January 1980 to January 1999 in a 2 000 000-resident area. Age, gender, and circumstances of death were recorded. Hearts were examined macroscopically and microscopically. In this series, the ARVC/D group accounted for 200 consecutive cases (10.4%) of USCD, including 108 males and 92 females (average age 32.5 and 34.5 years, respectively). Nearly one third of deaths occurred during the fourth decade of life. Circumstances of death were various, but 75.6% occurred during everyday life events (at home, 63.1%; in the street, 6.6%; or at work, 6.1%); only 7 cases (3.5%) occurred during sports activity. Nineteen cases (9.5%) happened during the perioperative period. Adipose infiltration of the right ventricle was either isolated (20%) or associated with fibrosis (74.5%) and lymphocytes (5.5%). A total of 14.5% of cases had cardiac hypertrophy, assessed by an increase in heart weight and/or left ventricular wall thickness. In most cases, the His bundle and its branches were abnormal either because of infiltration of adipose tissue (8.1%), fibrosis (54.3%), or both (5.6%).
  
  
  CONCLUSIONS
  In ARVC/D, both sexes are equally affected, and there is a peak of risk during the fourth decade. Death most frequently occurs during sedentary activity. His abnormalities and left ventricular hypertrophy may be associated with ARVC/D.
authors.affiliation: Service d'Anatomie-Pathologie, Hôpital Louis Pradel, Hospices Civils de Lyon, France.
authors.forename: A
authors.initials: A
authors.lastname: Tabib
authors.forename: R
authors.initials: R
authors.lastname: Loire
authors.forename: L
authors.initials: L
authors.lastname: Chalabreysse
authors.forename: D
authors.initials: D
authors.lastname: Meyronnet
authors.forename: A
authors.initials: A
authors.lastname: Miras
authors.forename: D
authors.initials: D
authors.lastname: Malicier
authors.forename: F
authors.initials: F
authors.lastname: Thivolet
authors.forename: P
authors.initials: P
authors.lastname: Chevalier
authors.forename: P
authors.initials: P
authors.lastname: Bouvagnet
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000401:mortality* / Q000473:pathology*; D002036:Bundle of His / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D016757:Death, Sudden, Cardiac / Q000453:epidemiology / Q000473:pathology*; D005260:Female; D006352:Heart Ventricles / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D009929:Organ Size; D015995:Prevalence; D012189:Retrospective Studies