J Pathol Transl Med.  2020 Sep;54(5):396-410. 10.4132/jptm.2020.06.10.

Indirect pathological indicators for cardiac sarcoidosis on endomyocardial biopsy

Affiliations
  • 1Division of Cardiology, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
  • 2Department of Pathology, Seoul National University College of Medicine, Seoul, Korea
  • 3Department of Internal Medicine and Interdisciplinary Program for Bioengineering, Seoul National University College of Medicine, Seoul, Korea
  • 4Cardiology Division, Cardiovascular Center, and Cardiac Electrophysiology Lab, Seoul National University Hospital, Seoul, Korea
  • 5Division of Cardiovascular Imaging, Department of Radiology, Seoul National University Hospital, Seoul, Korea
  • 6Department of Forensic Medicine, School of Medicine, Kyungpook National University, Daegu, Korea
  • 7Department of Pathology, Kyungpook National University Hospital, Daegu, Korea
  • 8Department of Radiology, Seoul Metropolitan Government - Seoul National University Boramae Medical Center, Seoul, Korea
  • 9Department of Pathology, Sejong Hospital, Bucheon, Korea

Abstract

Background
The definitive pathologic diagnosis of cardiac sarcoidosis requires observation of a granuloma in the myocardial tissue. It is common, however, to receive a “negative” report for a clinically probable case. We would like to advise pathologists and clinicians on how to interpret “negative” biopsies.
Methods
Our study samples were 27 endomyocardial biopsies from 25 patients, three cardiac transplantation and an autopsied heart with suspected cardiac sarcoidosis. Pathologic, radiologic, and clinical features were compared.
Results
The presence of micro-granulomas or increased histiocytic infiltration was always (6/6 or 100%) associated with fatty infiltration and confluent fibrosis, and they showed radiological features of sarcoidosis. Three of five cases (60%) with fatty change and confluent fibrosis were probable for cardiac sarcoidosis on radiology. When either confluent fibrosis or fatty change was present, one-third (3/9) were radiologically probable for cardiac sarcoidosis. We interpreted cases with micro-granuloma as positive for cardiac sarcoidosis (five of 25, 20%). Cases with both confluent fibrosis and fatty change were interpreted as probable for cardiac sarcoidosis (seven of 25, 28%). Another 13 cases, including eight cases with either confluent fibrosis or fatty change, were interpreted as low probability based on endomyocardial biopsy.
Conclusions
The presence of micro-granuloma could be an evidence for positive diagnosis of cardiac sarcoidosis. Presence of both confluent fibrosis and fatty change is necessary for probable cardiac sarcoidosis in the absence of granuloma. Either of confluent fibrosis or fatty change may be an indirect pathological evidence but they are interpreted as nonspecific findings.

Keyword

Myocarditis; Arrhythmogenic right ventricular dysplasia; Tachycardia, ventricular; Sarcoidosis; Cardiac muscle
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