Background/Purpose: Sarcoidosis, characterized by non-necrotizing granulomatous inflammation, is a multisystem disease with an unpredictable disease spectrum. An estimated 5% of sarcoidosis patients have clinically evident cardiac involvement while 25% may have asymptomatic silent disease leading to challenging diagnosis. We aim to study the clinical characteristics in a cohort of cardiac sarcoidosis patients to identify patterns of cardiac involvement and clinical correlations.

Methods: We conducted a single center retrospective study of patients with cardiac sarcoidosis at Mayo Clinic Florida. The study was approved by the Mayo Clinic Institutional Review Board (IRB). Inclusion criteria was patients older than 18 years of age, with diagnosis of sarcoid myocarditis/cardiac sarcoidosis/sarcoid heart muscle disease specified ICD-10 code D86.85 with established visits using specified CPT codes between 02/25/2018 till 08/24/2023. A list of 99 patients was generated and 52 patients met above criteria. Subsequently data from each patient was collected that included baseline demographics and disease characteristics.

Results: Table 1 provides patient demographics. 23 (55.8%) patients were male and 29 (44.2 %) were female with 42 (81%) Caucasian and 8 (15%) African American. There were 5 deaths (9.6% mortality) with 1 from cardiogenic shock, 1 cardiopulmonary arrest and 1 attributed to side effects of immunosuppressive therapy. 8 (15%) patients had a positive exposure history to chemicals and toxins.  Only 4 (19%) patients had an abnormal angiotensin converting enzyme (ACE) level.

Table 2 outlines cardiac evaluation and outcomes. Non-necrotizing granulomas noted on pathology in 18 (34.6%) patients and 31 (59.6%) had negative biopsy. Tissue sampled included lung 13 (25%), lymph node 9 (17.3%) and cardiac 5 (9.6%). 30 (58%) patients had cardiomyopathy with dilated being the most common and heart failure reduced ejection fraction (EF) the predominant type (N=18[40%]). 26 (55.3%) patients had abnormal electrocardiogram (EKG) with premature ventricular contractions (PVCs), atrial fibrillation and atrioventricular (AV) blocks the most frequent rhythm abnormalities.

Conclusion: Development of a standardized diagnostic algorithm and classification criteria for cardiac sarcoidosis is imperative. Our cohort recorded a high mortality rate and surveillance for cardiac involvement must be undertaken in all patients with sarcoidosis. Certain occupational exposures were identified and prompts raising awareness for environmental safety.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/cardiac-sarcoidosis-is-associated-with-a-high-mortality-retrospective-analysis-of-a-cohort-at-mayo-clinic-florida/