An Updated Review on the Clinicopathologic Aspects of Arrhythmogenic Right Ventricular Cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC) is a disease that affects young adults and perhaps is the most common cause of sudden death in this age group. Many of these cases are faced by forensic pathologists, without a prior history, and unless suspected by the pathologist at the time of autopsy, the diagnosis is likely to be missed. Since some of these cases are hereditary, consequences of missing the diagnosis have a medicolegal implication that extends to involve other family members of the deceased. ARVC raises many controversial issues with regard to its existence, defining and diagnostic criteria, pathologic spectrum of changes, and pathogenesis. We reviewed the recent literature and included our experience to dictate a novel approach to the inherent problems faced by pathologists. In this review, we identified the most characteristic and distinct histopathologic features that are diagnostic or highly suggestive of ARVC, even in the absence of clinical history. We also highlighted the new insights on the disease pathogenesis. Hence, this review provides a better understanding of the disease and sheds light on many controversial issues regarding ARVC.