Key result
Autopsy of two adult cases of sudden unexpected death revealed right ventricular noncompaction, suggesting pathological similarities with arrhythmogenic right ventricular cardiomyopathies.
Case Report (n=2)
Autopsy findings of isolated right ventricular noncompaction cardiomyopathy suggest pathological similarities or coexistence with arrhythmogenic right ventricular cardiomyopathy.
May prompt autopsy consideration of right ventricular noncompaction in unexplained sudden death; leaves open its distinction from ARVC and need for validation.
A predominantly right ventricular variant of isolated noncompaction cardiomyopathy is a potentially lethal disease entity, which only recently has become recognized in the clinical and cardiac imaging literature. There are currently few established morphologic criteria for the diagnosis other than right ventricular dilation and presence of excessive regional trabeculation. To date, there have been no autopsy reports of cases following either clinical diagnosis or sudden death. We report 2 adult cases of sudden unexpected death in which unexplained right ventricular dilation and prominent apical hypertrabeculation were the principal findings. The gross and microscopic results suggest pathological similarities between, or coexistence of, right ventricular noncompaction and arrhythmogenic right ventricular cardiomyopathies.
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Ilyas et al. (2013) conducted a case report in Isolated right ventricular noncompaction cardiomyopathy (n=2). Isolated right ventricular noncompaction cardiomyopathy was evaluated on Autopsy findings. Autopsy of two adult cases of sudden unexpected death revealed right ventricular noncompaction, suggesting pathological similarities with arrhythmogenic right ventricular cardiomyopathies.
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