Cardiac imaging in right ventricular cardiomyopathy/dysplasia--how does cardiac imaging assist in understanding the morphologic, functional, and electrical changes of the heart in this disease?

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is a complex disease and a difficult diagnosis to make that requires a multifaceted approach. Genetic mutations in proteins that alter the cardiomyocyte gap junction formation, stabilization, and general function lead to electrical and structural disturbances. The electrocardiogram (ECG) remains an excellent barometer of severe disease, but there is a large variation in the electroanatomic relationship and the ECG is less sensitive for detection of concealed early disease. With added knowledge of the genotypic/phenotypic expressions of this disease, continued experience with and development of current imaging modalities, and greater use of quantitative, 3-dimensional interpretative techniques, it seems clear that imaging will continue to play a pivotal role in collaborating with the ECG findings for the screening, diagnostic, prognostic, and serial assessment of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.