Morphological Re-Evaluation of Cardiac Sarcoidosis - A Case-Based Analysis of 14 Post-Mortem Japanese Hearts.

BACKGROUND

Cardiac sarcoidosis is a major cause of secondary cardiomyopathy leading to lethal arrhythmias and heart failure; however, identifying specific imaging findings of cardiac sarcoidosis remains a major issue. We re-examined the macroscopic morphology of post-mortem hearts to identify and reconfirm crucial findings for the clinical diagnosis of cardiac sarcoidosis.

METHODS AND RESULTS

We examined 14 autopsied hearts from patients who were diagnosed with cardiac sarcoidosis from 2002 to 2019 at several medical institutions in Japan. The cases were classified into 2 groups: ante-mortem diagnosis (Group A, n=8), and post-mortem diagnosis (Group B, n=6). Of the 14 hearts, steroids were only administered in Group A. Seven macroscopic features were re-evaluated in all hearts: (1) epicardial irregularity (n=5); (2) fibrosis in the posterior interventricular junction (n=11); (3) ventricular septum wall thinning (n=9); (4) diffuse left ventricular wall thinning (n=4); (5) ventricular aneurysms (n=3); (6) papillary muscle deformities (n=12); and (7) white colored fibrosis (n=8).

CONCLUSIONS

This study highlights the underreported macroscopic findings of cardiac sarcoidosis. The 7 features highlight the uniqueness of cardiac sarcoidosis, which warrants recognition of this disease as a distinct pathological entity rather than merely a dilated cardiomyopathy-like secondary cardiomyopathy.