Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia

Aditya Jain, Harikrishna Tandri, Hugh Calkins, David A Bluemke, Aditya Jain, Harikrishna Tandri, Hugh Calkins, David A Bluemke

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic cardiomyopathy characterized clinically by ventricular arrhythmias and progressive right ventricular (RV) dysfunction. The histopathologic hallmark is fibro-fatty replacement of RV myocardium. It is inherited in an autosomal pattern with variable penetrance. ARVD is unique in that it most commonly presents in young, otherwise healthy and highly athletic individuals. The cause of ARVD is not well-known but recent evidence suggests strongly that it is a disease of desmosomal dysfunction. The disease involvement is not limited only to the RV as left ventricle (LV) has also been reportedly affected. Diagnosis of ARVD is challenging and is currently based upon a multi-disciplinary work-up of the patient as defined by the Task Force. Currently, implanted cardioverter defibrillators (ICD) are routinely used to prevent sudden death in patients with ARVD. Cardiovascular MR is an important non-invasive diagnostic modality that allows both qualitative and quantitative evaluation of RV. This article reviews the genetics of ARVD, current status and role of CMR in the diagnosis of ARVD and LV involvement in ARVD.

Figures

Figure 1
Figure 1
A. Axial black-blood image from a patient with ARVD showing fat infiltration and thinning of the underlying myocardial wall (arrow).B. Axial fat-suppressed image from the same patient at the same level showing an irregular epicardial surface of the RV due to fat replacement of the RV (arrow).
Figure 2
Figure 2
Axial bright-blood image from a patient with ARVD showing a subtle 5 mm aneurysm near the moderator band (arrow).
Figure 3
Figure 3
Axial end-diastolic bright-blood image from a patient with ARVD showing RV dilatation. Note the diameter of RV is greater than LV at the mid-ventricular level.
Figure 4
Figure 4
Short-axial bright-blood image from a patient with ARVD showing increased trabeculation in the RV (arrows).
Figure 5
Figure 5
Axial bright-blood image from a patient with ARVD showing enlargement of RV outflow tract (large arrow). Note diameter of RV outflow tract is greater than that of the adjacent aorta (small arrow).
Figure 6
Figure 6
A. Axial end-diastolic bright-blood image from a patient with ARVD. The basal free wall (arrow) appears smooth and regular, with no aneurysms. B. Axial end-systolic bright-blood image from the same patient. A small aneurysm forms at the basal free wall due to dyssynchronous contraction of the RV (arrow).
Figure 7
Figure 7
Axial bright-blood image from a patient with ARVD showing a thinned lateral wall of the LV (arrow) due to fatty replacement.

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Source: PubMed

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