ARVC (Arrhythmogenic Right Ventricular Cardiomyopathy)
Definition
Cardiomyopathy in which right ventricular myocardium is progressively replaced by fibrofatty tissue, predisposing to right ventricular arrhythmia and sudden death. Leading cause of SCD in young athletes in Italy (Corrado series). Easily missed at autopsy unless the right ventricle is specifically examined.
- Full name
- Arrhythmogenic right ventricular cardiomyopathy
- Pathology
- Right ventricular myocardium replaced by fibrofatty tissue
- Risk
- Right ventricular arrhythmia, sudden death
- At-risk group
- Young athletes
- Reference data
- Corrado series (Italy)
Common questions
Why is ARVC easy to miss at a standard autopsy?+
The fibrofatty replacement is often localised to the right ventricular free wall, which is thin and not always specifically examined during a routine cardiac dissection focused on the left ventricle and coronary arteries.
What should a pathologist do differently when ARVC is suspected as a cause of sudden death?+
The right ventricle needs dedicated sectioning and, where available, histology targeting the free wall, and a family history of sudden cardiac death in young relatives should prompt genetic counselling and testing of surviving family members.
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