The Many Faces of Cardiac Sarcoidosis

Virian D. Serei, MD, PhD; Billie Fyfe, MD


Am J Clin Pathol. 2020;153(3):294-302. 

In This Article

Abstract and Introduction


Objectives: The objective of this study was to review and illustrate the sometimes diagnostically challenging features of cardiac sarcoidosis. We emphasize variable phenotypes presented at explant and biopsy evaluation and review literature regarding ancillary clinical and pathologic studies to enhance diagnostic accuracy.

Methods: A literature review was performed and two cardiac sarcoidosis cases were illustrated.

Results: Our cases and literature review demonstrate the pathologic spectrum of cardiac sarcoidosis. Irregular left ventricular free wall involvement is most common, followed by the interventricular septum and right ventricle. Although granulomas are often composed of tight epithelioid macrophage aggregates, early granulomas comprise loosely associated macrophages with lymphocyte predominance. Chronic disease leads to fibrosis and end-stage heart failure. Sampling errors and variable histology cause low endomyocardial biopsy sensitivity.

Conclusions: Current guidelines use clinical, radiologic, and immunohistologic criteria for diagnosing cardiac sarcoidosis. Knowledge of these guidelines will assist pathologists in making accurate diagnosis of this disease.