Clinical and histopathological profile of sarcoidosis of the heart and acute idiopathic myocarditis. Concepts through a study employing endomyocardial biopsy. I. Sarcoidosis

Sekiguchi, M.; Numao, Y.; Imai, M.; Furuie, T.; Mikami, R.

Japanese Circulation Journal 44(4): 249-263

1980


ISSN/ISBN: 0047-1828
PMID: 7373823
Accession: 004965247

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Abstract
Of 96 autopsied cases of sarcoidosis, 55 were determined as being fatal myocardial sarcoidosis. Additionally, analysis of sarcoidosis patients who were clinically assessed as having cardiac dysfunction was made through the literature and in 17 cases a questionnaire review of the reported cases in Japan was made. In 72 cases there was a high incidence of cardiac sarcoidosis (fatal myocardial sarcoidosis + sarcoidosis with cardiac dysfunction) in 40- to 59-yr-old women. The modes of cardiac death in fatal myocardial sarcoidosis in 55 cases were: sudden death, 20 cases (36.4%); congestive heart failure, 10 cases (18.2%); arrhythmia, 9 cases (16.4%): Adams-Stokes Syndrome (except sudden death), 8 cases (14.5%); cardiogenic shock, 1 case (1.8%); pacemaker death, 1 case (1.8%); pulmonary infarction, 1 case (1.8%); and unknown, 5 cases (9.1%). Expectancy of a definite diagnosis of endomyocardial biopsy which was made by an imitation endomyocardial biopsy in 7 autopsied cases of fatal myocardial sarcoidosis was 62.8% from the right ventricle and 46.9% from the left ventricle. When such pathology as a distinct scar or acute inflammatory change is incorporated, the possibility of the usefulness of the biospy increases. Examples of 8 biopsied cases were shown. Ultrastructural findings of epithelioid cells and giant cells in a 42-yr-old female in whom endomyocardial biopsy was performed from the right ventricle were described. Steroid therapy in cases with fatal myocardial sarcoidosis and with cardiac dysfunction was effective in 5 of the 14 cases of the former group (35.7%), and 10 of the 14 cases of the latter group (71.4%). Pacemaker implantation, temporary and/or permanent, was made in 9 of the 55 cases (16.5%) of fatal myocardial sarcoidosis and in 6 of the 17 cases (35.5%) of sarcoidosis with cardiac dysfunction. In the former group, the pacemaker was effective for a certain period of time but death finally occurred. In the later group, pacemaking was effective.