Why the study?
Distinguishing right ventricular cardiac sarcoidosis from arrhythmogenic right ventricular cardiomyopathy is challenging when imaging findings are limited to the right ventricle and ECG/arrhythmic features mimic ARVC.
Multimodality imaging is crucial for distinguishing right ventricular cardiac sarcoidosis from ARVC, ensuring appropriate immunosuppressive and device therapy.
RV-predominant sarcoidosis may mimic ARVC; leaves open need for biopsy or multimodality imaging in ambiguous cases.
Background: 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. Case summary: 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. Discussion: 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.
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Viskin et al. (2024) studied this question.
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