Cardiac MRI is currently the noninvasive modality of choice for evaluating patients with suspected Arrhythmogenic Right Ventricular Dysplasia (ARVD) or Dysplasia/Cardiomyopathy (ARVD/C).^reference Aetna Policy 0520 (CMR) cites “assessment of ventricular dysplasia” as an indication in which CMR is considered medically necessary.

ARVD is a significant cause of sudden death in adolescents and otherwise healthy, athletic adults. Direct relatives of ARVD patients are at  significantly increased risk of also having the disease. A positive diagnosis in a family member increases the probability of disease to 1:2 for an individual from 1 in 1000-5000 for the general population.^reference

MRI is an effective screening tool for such persons.  Since ARVD patients are frequently young and require a number of repeated examinations, CMR is particularly suitable.^reference

Complete evaluation of cardiac structure and function

MR imaging in right ventricular cardiomyopathy recognizes these important aspects of the disease process:

1. Global and regional right ventricular morphology,
2. Global and regional right ventricular dysfunction, and
3. Fibrofatty infiltration of the right ventricle.

Cardiac MR results have been shown to correlate well with RV angiography, endomyocardial biopsy, and echocardiography, and can complement these and other investigations in the diagnosis, work-up and follow-up in ARVD patients.^reference

Role of MR in ARVD Diagnosis

Diagnosing ARVD /C relies on demonstrated structural, functional, and electrophysiological abnormalities caused by (or reflecting) underlying histological changes. MRI can image the right ventricle with reproducible measurements of volume and systolic function. These measurements permit classification of severity and differentiation from normal heart characteristics. ^reference

References:

Marcus F, et al. Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Proposed Modification of the Task Force Criteria. Circulation. 2010.

Razmi R, Meduri A. MRI of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. Journal of Cardiovascular Magnetic Resonance. 2004.

Jain A, Tandri H, Calkins H, Bluemke DA. Role of cardiovascular magnetic resonance in the diagnosis of arrhythmogenic right ventricular cardiomyopathy/ dysplasia with left ventricular involvement. Journal of Cardiovascular Magnetic Resonance. 2008.