Small Cardiac Lesions Fibrosis of Papillary Muscles and Focal
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Small Cardiac Lesions Fibrosis of Papillary Muscles and Focal Cardiac Myocytolysis Arthur STEER, M.D.,1 Teruyuki NAKASHIMA, M.D.,2 Taketsugu KAWASHIMA, M.D.,1 Kelvin K. LEE, M.A.,3 Michael D. DANZIG, M.D.,4* Thomas L. ROBERTSON M.D.,4** and Donald S. DOCK, M.D.4 SUMMARY Three types of small cardiac lesions were described and illustrated: (1) focal type of papillary muscle fibrosis, evidently a healed infarct of the papillary muscle present in 13% of the autopsies, is a histologically characteristic lesion associated with coronary artery disease and healed myocardial infarction, (2) diffuse type of papillary muscle fibrosis, prob- ably an aging change present in almost half of the autopsies, is associated with sclerosis of the arteries in the papillary muscle, is identifiable his- tologically, and apparently is not associated with any cardiac abnormal- ity, and (3) focal cardiac myocytolysis, a unique histologic lesion, usually multifocal without predilection for any area of the heart, is associated with ischemic heart disease, death due to cancer complicated by non- bacterial thrombotic endocarditis and microthrombi in small cardiac arteries as well as with other diseases. Differentiation of the 2 types of papillary muscle fibrosis is important in the study of papillary muscle and mitral valve dysfunction. Focal cardiac myocytolysis may contribute to the fatal extension of myocardial infarcts. From the Radiation Effects Research Foundation Hiroshima and Nagasaki. A Cooperative Research Institute supported by funds from the U.S.A. National Academy of Sciences-National Research Council, Atomic Energy Commission and Environmental Protection Agency and from the Japanese National Institute of Health of the Ministry of Health and Welfare. 1 Department of Pathology, Radiation Effects Research Foundation, 5-2 Hijiyama Park, Hiro- shima City 730. 2 Department of Pathology , Kurume University School of Medicine, 67 Asahi-machi, Kurume City 830. 3 Department of Epidemiology and Statistics , Radiation Effects Research Foundation, 5-2 Hiji- yama Park, Hiroshima City 730. 4 Department of Medicine , Radiation Effects Research Foundation, 5-2 Hijiyama Park, Hiro- shima City 730. + Hiroshima Branch Laboratory , Japanese National Institute of Health, Ministry of Health and Welfare. * Surgeon and ** Senior Surgeon , U.S. Public Health Service. Address for reprint request to: Teruyuki Nakashima, M.D., Department of Pathology, Kurume University School of Medicine, 67 Asahi-machi, Kurume City 830, Japan. Received for publication May 27, 1977. 812 Vol.18 No.6 SMALL CADIAC LESIONS 813 Additional Indexing Words: Focal and diffuse papillary muscle fibrosis Healed papillary muscle infarct HERE is considerable discrepancy in the reported incidence of fibrosis of papillary muscles and even less agreement on pathogenesis. In an extensive review of changes after myocardial infarction, Schechter1) stated, under the heading of papillary muscle infarction, that fibrous scars were re- ported present in cardiac papillary muscles in 25% to 80% of random au- topsies. In 1969, Brand et a12) distinguished the papillary muscle scar of infarction, which he called focal papillary muscle fibrosis, from the more fre- quent diffuse type of papillary muscle scar. There has been little subsequent attention paid to this distinction.3)-7) We undertook an autopsy study of papillary muscle scars in which we attempted to associate their presence with available clinical observations in the same subjects. During this study another small myocardial lesion, focal cardiac my- ocytolysis, was observed. This lesion had been described previously by Schle- singer and Reiners8)in 1955 and repeatedly since then under a variety of titles by others9)-12)culminating in the report by Baroldi13) of 3 distinguishable morphologic types of myocardial necrosis including this type which he called colliquative myocytolysis. Focal cardiac myocytolysis has attracted little organized attention and although often found associated with myocardial infarction, it may be the only myocardial lesion in some conditions and can be a cause of death (low output failure, beer drinker's heart, etc). Pathologists and clinicians are generally unfamiliar with this lesion. Focal cardiac my- ocytolysis was observed in 21 of the 375 autopsies. METHODS This study included all autopsies performed at the Radiation Effects Research Foundation (RERF, formerly the Atomic Bomb Casualty Commission, ABCC) on members of the Adult Health Study sample14> during 1965-70 in Hiroshima and 1968-70 in Nagasaki. Most of the subjects had been examined at least once in the RERF clinics at which time information was obtained on blood pressure levels, evi- dence of heart disease including electrocardiograms, and the presence of diabetes mellitus. In most instances, the only information available concerning the terminal illness was contained in the death certificate. At least 7 blocks of tissue were taken from every heart, including 2 from the interventricular septum, 4 from the left ventricle, and 1 from the right ventricle. One section of the anterior left ventricle and one of the posterior left ventricle were cut parallel to the base of the heart and each included a transection of the attached pa- pillary muscle at a level near its origin and away from the insertion of the chordae tendineae. In 2 cases there was insufficient papillary muscle for evaluation. Hema- 814 STEER ET AL. Novemberap. Heart, 1977 J. toxylin and eosin were routinely used in all cases. It was supplemented by trichrome, elastic and amyloid stains for the study of blood vessels and with lipofuscin, iron, PAS, acid fast, alcian blue and reticulum stains for study of pigment and the lesions of myocytolysis. The severity of coronary atherosclerosis was determined by the American Heart Association method15) in which the proximal 5cm of the arteries (left anterior de- scending, left circumflex, and right circumflex) were opened longitudinally and compared with a standard illustration showing 7 degrees of coronary atherosclerosis, Thickening and sclerosis of the arteries in the papillary muscles was determined by examination of histological sections. Hypertension was defined as blood pressure readings of 160mmHg or higher systolic and 95mmHg or higher diastolic on 2 or more occasions. Hypertensive heart disease was diagnosed when hypertension as defined above was accompanied by any of the following: cardiomegaly (X-ray), atrial fibrillation, left bundle branch block, left axis deviation, S-T depression, or T wave inversion of 1.0mm or more. Diabetes mellitus was diagnosed when serum glucose values exceeded any of the following on 2 or more occasions: fasting 125mg%, 1 hour 215mg%, 2 hour 145mg%, or 3 hour 130mg%. The focal type of papillary muscle fibrosis was defined as a sharply demarcated, hyalinized scar separated from the endocardial surface of the papillary muscle by a rim or palisade of viable muscle fibers (Fig.1). If patent vessels are present in the scar, they were usually thin-walled and surrounded by viable muscle fibers. Often most of the central portion of the papillary muscle was involved. In some cases of acute myocardial infarction, acute coagulative necrosis was also present in the pa- pillary muscle and then it had the same distribution as the focal type of papillary muscle fibrosis. The diffuse type of papillary muscle fibrosis was poorly demarcated. It ex- tended between and replaced muscle fibers (Fig.2). It was dense, hyalinized, and Fig.1. Focal type of papillary muscle fibrosis with palisade of viable muscle fibers beneath the moderately thickened endocardium and about small vessels. 72-year-old male, carcinoma of the stomach with healed myocardial infarct, heart weight 380 Gm. Elastica trichrome•~38 Vol.18 No.6 SMALL CARDIAC LESIONS 815 Fig.2. Diffuse type of papillary muscle fibrosis is poorly demarcated, extends to the endocardial surface and contains prominent sclerosed arteries. 66-year-old female, hypertensive heart disease, heart weight 250 Gm. Elastica trichrome•~28 Fig.3. Arterial sclerosis and diffuse type of papillary muscle fibrosis. Same patient as in Fig.2. Elastica trichrome•~68 acellular. The scars were often situated in the periphery of the papillary muscle and extended to the endocardial surface without an intervening layer of viable mus- cle fibers. Prominent, thickened, and sclerotic arteries were almost always evident and appeared to be related to the areas of fibrosis (Fig.3). An acute, necrotic or healing stage was never observed. In some cases, both the focal and diffuse types of papillary muscle fibrosis were present. The 2 types could be distinguished even when they occurred in the same papillary muscle (Fig.4). Focal cardiac myocytolysis was defined as areas of apparently simple muscle fiber lysis marked by an empty but intact scaffold of stroma and sarcolemma sheaths containing varying numbers of mononuclear cells many of which were filled with 816 STEER ET AL. Jap.November Heart J. , 1977 Fig.4. Both focal (right) and diffuse (left) types of papillary muscle fibrosis in the same papillary muscle. 71-year-old male, death due to chronic pyelonephritis, heart weight 380 Gm, healed myocardial infarct. Elastica trichrome•~6 Fig.5. Focal cardiac myocytolysis. Empty sarcolemma sheaths, round- ed pigment-containing mononuclear cells. Note absence of necrosis, inflam- matory infiltrate or evidence of repair. 75-year-old female died of carcinoma of the gall bladder. Heart weight 240 Gm. Lytic lesions were present in an- terior