304 Cardiac Sarcoidosis; A Clinical Diagnostic Challenge in a 10-Year Retrospective Study from a Single Institution

Introduction/Objective The challenge of diagnosing cardiac sarcoidosis (CS) lies in the often nonspecific or subtle symptoms of cardiac involvement, which can easily be overlooked or misattributed to other conditions. It is essential to establish a definite diagnosis of CS to enable timely, appropriate treatment. Methods/Case Report We conducted a retrospective review of medical records and pathological analyses of cases with histologically confirmed CS at our institution from January 2013 to December 2023. This cohort was derived from a patient population enrolled in our Center for Advanced Cardiopulmonary Therapies and Transplantation, including approximately 600 patients seen during that time period. Tissue diagnosis of cardiac sarcoidosis was based on presence of well-defined non-necrotizing granulomas with or without multinucleated giant cells and negative special stains for mycobacterial and fungal microorganisms. The medical records, laboratory test results, imaging studies, and other pathological specimens from non-cardiac sites when available were reviewed to determine if patients had a prior diagnosis or suspicion of sarcoidosis. Isolated cardiac sarcoidosis was diagnosed when there was no evidence of extracardiac involvement. Results Sixteen patients were identified, with an age range of 43-70 years, comprising ten females and six males. The specimens included 11 explanted hearts, 3 left ventricular cores obtained during placement of left ventricular assist devices (LVADs), 1 autopsy case, and 1 endomyocardial biopsy (EMB). Isolated cardiac sarcoidosis was found in 8 of 16 patients (50%). Of these, in 7 of 8 patients (87%) the diagnosis was unexpected. These included 6 heart explants, 1 EMB, and 1 autopsy case. In the remaining 8 patients, there was a known history of systemic sarcoidosis. Conclusion In this series, the diagnosis of isolated cardiac sarcoidosis was made unexpectedly in 50% of patients emphasizing the importance of pathologic evaluation of endomyocardial biopsy and other tissue specimens with a high index of suspicion for the diagnosis of CS