Clinicopathological Study on Fatal Myocardial Sarcoidosis*

Abstract

Presented here was an outline of fatal myocardial sarcoidosis in Japan based on 42 autopsy cases. The incidence, clinical features, pathological findings (particularly the distribution of sarcoid lesions in various organs, and gross as well as histological patterns have been reviewed. The ratio of fatal myocardial sarcoidosis to the total number of sarcoidosis deaths is higher than that of other countries and it appears characteristic of Japanese sarcoidosis that the disease occurs here predominantly after the fourth decade in females. Only 12% of myocardial sarcoidosis cases are diagnosed exactly. Conduction disturbances and dysrhythmias due to myocardial damage are its most common manifestations. The modes of presentation or cardiac death are sudden death, 16 cases (41%); congestive heart failure, 9 cases (23.1%); Adams-Stokes syndrome, except sudden death, 7 cases (17.9%); death due to dysrhythmia, 6 cases (15.4%); and 2 pacemaker deaths among the application of pacemakers, 7 cases. As for the ECG findings, A-V block and bundle-branch block was observed in most cases and ectopic beats were also frequently observed. The difficulty in exact diagnosis of myocardial sarcoidosis appears to be due to the frequency of lack of manifestation of systemic sarcoidosis as well as insidious cardiac involvement. The gross findings on myocardial lesions showed 3 patterns and each of the localized patterns corresponded to the histological extension. The conglomerate-band-like pattern corresponded to expansive invasion, the dendrite pattern to interstitial extension. The histological findings on the myocardial lesions were classified into four types: 1) exudative type, 2) granuloma type, 3) combined type of granuloma and fibrosis, 4) fibrotic type. Two extreme cases showed the exudative and fibrotic type, respectively, and were discussed.

Joint SNMMI–ASNC expert consensus document on the role of 18F-FDG PET/CT in cardiac sarcoid detection and therapy monitoring

“…17,18 Depending on the type and extent of involvement, CS can present as conduction abnormalities, ventricular arrhythmias, sudden cardiac death, systolic and diastolic heart failure, or valvular disorders, which may be related to papillary muscle involvement. [19][20][21][22][23][24] Sarcoidosis of the pericardium can present as a pericardial effusion with or without hemodynamic instability. 25,26 Myocardial ischemia and infarction due to coronary artery involvement have also been described but are rare, 27 and myocardial fibrosis due to CS itself typically occurs in a nonvascular distribution and in some cases is the only evidence of prior active CS.…”

Postpartum Complete Atrioventricular Block Due to Cardiac Sarcoidosis Steroid Therapy Without Permanent Pacemaker

“…Cardiac sarcoidosis has been reported to occur most frequently in middleaged or older women in Japan, 3) although its incidence in Western countries was shown to have no gender difference and to be higher in the younger generation. 4,5) This paper has reported a very rare case with cardiac sarcoidosis occurring in a young Japanese postpartum female.…”

Deterioration of Cardiac Function during the Progression of Cardiac Sarcoidosis: Diagnosis and Treatment

“…The most frequent cardiac manifestations of cardiac sarcoidosis (CS) include conduction disturbances and arrhythmias, sudden death and congestive heart failure (CHF), the latter being reported in up to 30% of affected patients (3)(4)(5)(6)(7)(8)(9). Increased awareness, early medical treatment, the use of pacemakers and implantable cardioverter-defibrillators (ICDs) and cardiac resynchronization therapy with ICD (CRTD) are changing the causes of death in CS patients, with CHF becoming the most common cause.…”