Sarcoid heart disease.

Abstract

Sarcoid of the heart has not been commonly reported in the United Kingdom. The author's personal experience suggested that it was not rare, and an inquiry among colleagues revealed a total of 50 cases, 20 of which were fatal and had necropsy confirmation. The diagnosis hadfrequently not been made in life.The clinical features of these cases are analysed and it is suggested that sarcoid infiltration of the myocardium should be considered in any unusualform of heart disease, particularly where disturbance of rhythm is prominent. Treatment with steroids should be tried in the active phase, but the degree of success will depend on the degree of the myocardial involvement. Treatment of rhythm disturbances should be carried out as necessary.

Sarcoidosis

“…Longcope and Freiman (1952) noted the frequency with which the heart had been found to be infiltrated by sarcoid in the recorded necropsies on patients with uveoparotid fever (Souter, 1929;Garland and Thompson, 1933;Granström et al, 1946;Bates and Walsh, 1948;van Rijssel, 1947, Ca se 1; Adickes et al, 1951;Yesner and Silver, 1951;Longcope and Freiman, 1952, Case 16). On the other hand, others (Fleming, 1974;Roberi:s et al, 1977;Virmani et al, 1980) have commented on the rarity of c1inically evident cardiac involvement in patients with syrnptomatic sarcoidosis of other organs. This variable relationship to changes in other organs is not peculiar to the heart.…”

“…Many published reports are of single or a few cases presenting in one of these remarkable ways. Reports of larger numbers of personally studied cases, with reviews, have been published by Fleming (1974), Roberts et al (1977) andSilverman et al (1978).…”

“…This is not only because of the incidental finding of granulomas in the heart in patients dying from other manifestations of sarcoidosis, but also because of the inclusion of cases in which sudden death was the first evidence of disease of any sort, or of cardiac involvement in patients with sarcoidosis. Longcope and Freiman (1952) found the myocardium involved in four of their 30 fatal cases, and in 18 of 92 necropsies in cases of sarcoidosis reported by orhers, Of 50 cases of cardiac sarcoidosis occurring in the United Kingdom, reviewed by Fleming (1974), 20 were fatal; in 14 of these the diagnosis of sarcoidosis had not been made in life. Matsui et al (1976) studied material The Heart 331 from 72 cases reported as sarcoid or giant-cell myocarditis in Japan, and finally diagnosed 42 as myocardial sarcoidosis; in only five of these had the diagnosis of sarcoidosis been made during life, and death had been sudden in 16.…”

Sarcoidosis: histopathological definition and clinical diagnosis.

“…Histology showed non-caseating granulomas, chronic intrahepatic cholestases, increased copper in hepatocytes, progressive diminution in number of interlobular bile ducts, periportal fibrosis, and the eventual development of a micronodular 'biliary' cirrhosis; but the characteristic nonsuppurative destructive cholangitis of PBC was not present and mitochondrial antibodies were not found. HEART A high proportion of the reported cases of sarcoidosis affecting the heart have been found at necropsy after sudden death, eg, 12 of the 50 cases in the United Kingdom summarised by Fleming (1974). The diagnosis is unlikely to be made during life unless there is evidence of involvement of other organs, and, although in some cases this is obvious, in others it is difficult to find.…”

“…Both the extent and the location of cardiac involvement are very variable, ranging from scattered granulomas which produce evidence of their presence only if they are so placed that they affect the conducting tissues of the heart or produce dysrhythmias, to extensive replacement of myocardium. Even at necropsy the diagnosis may be overlooked unless sections are prepared from the ventricular septum which is most frequently infiltrated microscopically in minor involvement of the heart by sarcoidosis (Bashour et al, 1968;Fleming, 1974). In view of the rhythmic contractions of the myocardium, it is perhaps not surprising that the granulomatous infiltration observed in it tends to be less uniform in its appearance than is usual in other organs.…”