Diagnosing arrhythmogenic right ventricular cardiomyopathy: Is an image always worth a thousand words?

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited genetic cardiomyopathy caused by mutations in the gene encoding proteins of desmosome complexes. To illustrate some challenges with ARVC diagnosis, we present the case of a young patient with family history of sudden cardiac death whose baseline cardiac MRI (CMR) results were normal. Upon development of additional arrhythmic abnormalities 3 years later, CMR was repeated and revealed abnormalities suggestive of ARVC. Since certain diagnoses, including ARVC, may have serious consequences if the timely diagnosis is not made, clinical and imaging clues need to be integrated taking the pre-test likelihood of the disease in question into consideration.