Arrhythmogenic RV Cardiomyopathy

 

Image Library Home >>Cardiomyopathy>> Arrhythmogenic Right Ventricular Cardiomyopathy

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ARVC, sometimes also called ARVD (D for Dysplasia), is a rare condition where fibrofatty collagen tissue replaces the normal RV wall. Less commonly, this process involves the LV.
Classically the RV has focal wall motion abnormalities, as well as small aneurysms. This parasternal view shows a markedly enlarged RV and RVOT. Small aneurysms are present in the most anterior portion.

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In this apical 4 chamber view note how much of the RV is akinetic. Small aneurysms are again noted toward the apex.
Individuals with ARVC are at high risk for ventricular arrhythmias (hence the name). Note the dual chamber ICD present (also seen in the parasternal view above).

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Frequently because the RV is dilated there is tricuspid annular dilation along with with regurgitation as shown here.

A

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B

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C

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D

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Additional views are shown here including the parasternal RV inflow view (panel A), the same view with color Doppler (panel B) a parasternal short axis view (panel C) and a subcostal view (panel D).
Cardiac MRI is a complementary imaging modality that helps to make this diagnosis as echocardiographic findings are often not specific. This individual had both a cardiac MRI and endomyocardial biopsy suggestive of ARVC.