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July 1, 1998Journal of Forensic Sciences

Pathology of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia—An Autopsy Study of 20 Forensic Cases

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Key result

In 20 forensic cases of sudden death due to ARVC, the disease was previously unrecognized in all cases, with biventricular involvement in 40% and inflammatory infiltrates in 60%.

Population

20 people who died suddenly of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia, mean age 41 years…

Design

Case_series

Authors

PFPaul FornésInsermSRSébastien RatelUniversity of GloucestershireDLDominique LecomteCentre National de la Recherche Scientifique

Discussion

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Implication

ARVC autopsy series underscores sudden death risk in the young; leaves open optimal prevention and screening strategies.

Key Points

  • To evaluate clinical characteristics, postmortem pathological features, and potential disease mechanisms in individuals who experienced sudden death from arrhythmogenic right ventricular cardiomyopathy (ARVC).
  • Analyzed autopsy and histological records across a 7-year period for 20 forensic cases (9 men, 11 women; mean age 41 years, range 17–80) meeting ARVC diagnostic criteria.
  • Evaluated circumstances surrounding sudden death, macroscopic heart weights, ventricular involvement, and microscopic presence of fat infiltration, fibrosis, lymphocytic inflammation, and myocyte necrosis.
  • ARVC was undiagnosed prior to death in all 20 individuals; 14 died at rest and 6 during effort, with acute emotional stress triggering death in 45% (9/20) of cases.
  • Postmortem analysis identified a mean heart weight of 380 g (range 280–520 g), biventricular involvement in 40% (8/20), lymphocytic inflammatory infiltrates in 60% (12/20), and myocyte necrosis in 5% (1/20).

Study Design

Type

Observational (n=20)

Structured PICO

P
Population
20 individuals (mean age 41 years, 55% female) who died suddenly of previously undiagnosed arrhythmogenic right ventricular cardiomyopathy.
O
Outcome
Clinical characteristics and pathological patterns in the hearts

Autopsy findings of sudden death victims with ARVC show frequent biventricular involvement and inflammatory infiltrates, suggesting a degenerative process involving genetic, viral/autoimmune, and apoptotic factors.

Cite This Study

Fornés et al. (1998) conducted an observational in Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) (n=20). Arrhythmogenic right ventricular cardiomyopathy (ARVC) was evaluated on Clinical characteristics and pathological patterns. In 20 forensic cases of sudden death due to ARVC, the disease was previously unrecognized in all cases, with biventricular involvement in 40% and inflammatory infiltrates in 60%.

synapsesocial.com/papers/6a6d1ffcf44fa9f079db8a17https://doi.org/10.1520/jfs14306j
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Arrhythmogenic right ventricular dysplasia: a generalized cardiomyopathy?1983 · 142 citations
  2. 2Chronic (or healed) myocarditis mimicking arrhythmogenic right ventricular dysplasia1993 · 39 citations
  3. 3Right Ventricular Cardiomyopathy: Another Cause of Sudden Death in the Young1988 · 54 citations
  4. 4Dysplastic conditions of the right ventricular myocardium: Uhl's anomaly vs arrhythmogenic right ventricular dysplasia.1993 · 125 citations
  5. 5Arrhythmogenic right ventricular disease, dysplasia and cardiomyopathy1996 · 5 citations