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An updated review on the clinicopathologic aspects of arrhythmogenic right ventricular cardiomyopathy
Dina El Demellawy1, Ahmed Nasr, Salem Alowami
1Department of Pathology and Laboratory Medicine, Thunder Bay Regional Health, Sciences Centre, University Of Northern Ontario School of Medicine, Thunder Bay, Ontario, Canada. dina_eldemellawy@rogers.com
Insights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a key cause of sudden death in young adults. This review details diagnostic histopathologic features for forensic pathologists to identify ARVC, even without clinical history.
Area of Science:
- Cardiology
- Pathology
- Forensic Medicine
Background:
- Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary cause of sudden cardiac death in young adults.
- Diagnosis is often missed in forensic pathology due to lack of clinical history.
- Undiagnosed ARVC has hereditary implications for family members and medicolegal consequences.
Purpose of the Study:
- To review current literature and provide a novel approach to diagnosing ARVC in forensic pathology.
- To identify characteristic histopathologic features for ARVC diagnosis.
- To elucidate new insights into ARVC pathogenesis and address controversial issues.
Main Methods:
- Comprehensive review of recent scientific literature on ARVC.
- Inclusion of expert experience in forensic pathology cases.
- Focus on identifying distinct histopathologic findings.
Main Results:
- Identified key histopathologic features diagnostic or highly suggestive of ARVC.
- Highlighted new insights into the pathogenesis of ARVC.
- Provided a framework for improved diagnosis in cases lacking clinical history.
Conclusions:
- Histopathologic examination is crucial for diagnosing ARVC in forensic settings.
- Understanding characteristic features aids in identifying hereditary cases and preventing missed diagnoses.
- This review clarifies controversial aspects and enhances understanding of ARVC.
Abstract:
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.
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