A perfect mimic: the role of multimodality imaging in right ventricular cardiomyopathy-a case report

Distinguishing right ventricular cardiac sarcoidosis (RVCS) from arrhythmogenic right ventricular cardiomyopathy (ARVC) is often a challenging task particularly when imaging findings are limited to the right ventricle and when electrocardiographic and arrhythmic findings are typical for ARVC. Here, we discuss the challenges of diagnosis and management in a patient whose initial work-up strongly suggested ARVC and was later diagnosed with cardiac sarcoidosis (CS), highlighting the role of multimodality imaging.

On presentation, this patient displayed electrocardiographic abnormalities, arrhythmia morphology, and cardiac magnetic resonance imaging findings consistent with the criteria for a definite diagnosis of ARVC. However, through the use of multimodal imaging, a final diagnosis of CS was made. Subsequent immunosuppressive treatment effectively managed the inflammation, and as a primary prevention measure, an implantable cardioverter-defibrillator was implanted. Almost 2 years following the initial presentation, the patient received an appropriate shock from the device, attributed to rapid ventricular tachycardia arising from myocardial scarring.

Right ventricular cardiac sarcoidosis can closely resemble ARVC, making it challenging to distinguish between the two. Multimodality imaging is pivotal for accurate diagnosis and risk assessment, as well as for adjusting immunosuppressive therapy and monitoring response to treatment in CS.