P. Fornes et al., PATHOLOGY OF ARRHYTHMOGENIC RIGHT-VENTRICULAR CARDIOMYOPATHY DYSPLASIA - AN AUTOPSY STUDY OF 20 FORENSIC CASES/, Journal of forensic sciences, 43(4), 1998, pp. 777-783
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is ch
aracterized histologically by massive infiltration of the right ventri
cular walt by fat tissue, with surviving strands of cardiomyocytes bor
dered by or embedded in fibrosis. ARVC has been recognized as a cause
of sudden death, especially in the young. The purpose of our autopsy s
tudy was to examine the clinical characteristics and the pathological
patterns in the hearts of 20 people who died suddenly of ARVC. In view
of our findings and the literature, we discussed the possible causes
and pathogenesis of ARVC, as well as the mechanisms by which sudden de
ath occurs in this disease. During the 7-year study period, 20 hearts
from 9 men and 11 women fulfilled the criteria for ARVC. The mean age
was 41 years (range, 17 to 80). The disease was unknown prior to death
in all cases. Fourteen persons died at rest, and six on effort. In 9
of the 20 cases, the trigger of sudden death was an acute emotional st
ress, sometimes associated with a moderate physical activity. The mean
heart weight was 380 g (range, 280 to 520). Both ventricles were invo
lved in 40% of the cases. Inflammatory infiltrates consisting of lymph
ocytes were present in 60% of the cases, but myocyte necrosis was foun
d in only one case. ARVC is more likely to result from a degenerative
process than a congenital disorder. Genetic factors, viral or autoimmu
ne inflammation or both, and apoptosis are also involved in the degene
rative disorder.