Heart and vessels. Supplement最新文献

Immunofluorescent and electron-microscopic studies were performed to determine the distribution of viral antigens and particles and to clarify the relationship to myocardial lesions in two autopsy cases with generalized infection of Coxsackie virus B3 (CVB3) or cytomegalovirus (CMV). Case 1 was a full-term newborn female infant, without any congenital anomalies, who died of cardiac failure 10 days after birth. CVB3 was isolated from the blood before death. Necrosis of the muscle fibers was observed, frequently accompanying calcification. Numerous histiocytes and a few lymphocytes and neutrophils had infiltrated in and around the necrotic areas. Immunofluorescent study (IF) revealed CVB3 antigen in the muscle fibers and vascular endothelial cells. Case 2 was a female infant, born at 28 weeks of gestation, who died of fatal arrhythmia 50 days after birth. The infant had hemocephalus and a history of idiopathic respiratory distress and underwent an operation for patent ductus arteriosus. Cytomegalic cells were frequently found in the vascular endothelial cells in the myocardium and occasionally in muscle fibers. IF showed the presence of CMV antigen in both endothelial cells and muscle fibers. CVB3 and CMV antigens were detected predominantly in vascular endothelial cells rather than in the muscle fibers. Blood flow disturbance due to endothelial damage is a cause of the myocardial lesion in addition to the direct invasion of the muscle fibers by the virus.

Fourteen cases of heart disease with hypereosinophilia were analyzed employing conventional cardiologic methods, including echocardiography, cardiac catheterization, and endomyocardial biopsy. The cases were divided into four types: Acute carditic (endocarditis, myocarditis, pericarditis; five cases); ventricular dilation (three cases); restrictive (three cases); electric disturbance (three cases). Biopsy revealed significant changes in all cases. In one case of the ventricular dilation type, endomyocardial fibrosis with myocardial degeneration was seen, and in another case mural thrombus formation was shown to be present. In three cases of the restrictive type, endomyocardial fibrosis (EMF) was observed. In two cases of the electric disturbance type, minor right ventricular myocardial degeneration was observed. In two of the three cases of the carditic type and in three of eight cases in other categories, postmyocarditic changes were observed. The course of the disease compared with the type of disorder revealed a short course in the carditic type and a longer course, ranging from 2 to 24 years, with one exception, in the other types. It is also confirmed that the various histopathologic changes can be related to particular clinical presentations. We have shown that the basic changes in eosinophilic heart disease are not restricted to the endomyocardium and that they occur in various parts of the heart causing more widespread manifestations. The more comprehensive term "eosinophilic heart disease" is a preferable description.

Sixty-eight patients (24 males, 44 females, mean age 37.2 years) belonging to NYHA classes I and II were investigated. All patients had a nondilated ventricle as well as hemodynamic criteria of "latent cardiomyopathy." In 78% of the patients, a high susceptibility to infection, frequent angina, or possible myocarditis were found. In 75.5%, the mean pulmonary artery pressure was abnormal. T1-201 scintigraphy revealed perfusion defects in 78%. Dyskinesia or hypokinesia were found in 52%. The left ventricular ejection fraction was normal in 74%. On biopsy, pathologic findings were detected in 60.9% of patients with a high frequency of hypertrophy and fibrosis (50%). In two patients, lymphocytic infiltrates were found. Immunohistologically, deposits of complement, IgG, IgM, and gammaglobulin, mainly in the sarcolemma, could be identified in 19.1% of cases. The myocardium was normal in 39.1%; insufficient material was found in 5.9% of patients. Morphologic, scintigraphic, and hemodynamic findings could not be correlated but the combination of two pathologic parameters permitted diagnosis. Biopsy was the most effective diagnostic method in myocarditis.

Cardiomyopathy associated with vitamin E deficiency was diagnosed in more than 100 ruminants and primates and in 106 embryos and newly hatched chicks and ducklings. Affected bird embryos failed to pip the eggshell and died inside. Newly hatched chicks and ducklings and neonatal ruminants were weak, had difficulty standing or rising, and died within a few days. Death usually occurred without premonitory signs of disease in juvenile and adult animals. On gross examination, the hearts of the neonatal ruminants had areas of mottled, pinkish-tan myocardium. The hearts of the embryos and newly hatched birds were edematous, pinkish, and pale. In the juvenile ruminants, irregular, whitish patches or pale areas were seen in the myocardium. Histologically, there was multifocal myocytolysis in the myocardium of the neonatal and juvenile ruminants and embryos and newly hatched birds. Focal disseminated or diffuse myocardial fibrosis and myocytolysis were observed in the hearts of the adult animals. Plasma alpha tocopherol values were low enough in all species to be considered deficient. These values increased significantly after the addition of alpha tocopherol and/or vitamin E to the diets of the animals. Cardiomyopathy has not been diagnosed in any of the same groups of animals since supplementation was initiated.

Morphologic characteristics of granulomatous inflammation in the heart and pericardium are discussed. In rheumatic fever, two types of myocardial lesion are present--a nonspecific myocarditis and a specific lesion characterized by granulomas known as Aschoff's nodules. The latter undergo a cycle of development and resolution; in their mature stage, they contain Aschoff's cells which are uni- or multinucleated histiocytes with a serrated nuclear chromatin bar. Ultrastructural studies do not suggest a relationship between these cells and cardiac or smooth muscle cells. In metabolic disorders, granulomas occur in Farber's disease (lipogranulomatosis), gout (in which tophi are associated with calcific deposits and with a foreign body cellular reaction), the various syndromes of oxalosis (in which oxalate deposits also lead to a foreign body reaction), and in chronic granulomatous disease of childhood. Foreign body giant-cells can also be found in association with calcification of necrotic myocytes and in the syndromes of "cholesterol pericarditis." Well-developed granulomas occur in sarcoidosis, giant cell myocarditis, as a reaction to foreign bodies and devices implanted within the cardiovascular system, and in certain diseased caused by infective agents (tuberculosis, fungal and parasitic disorders). Infiltration of the heart by nongranulomatous masses of histiocytes can occur in Whipple's disease, Niemann-Pick disease, the hyperlipoproteinemias, Gaucher's disease, and in proliferative disorders of the mononuclear phagocyte system (juvenile xanthogranuloma, Chester-Erdheim syndrome, and malignant histiocytosis).

In the Annuals of Autopsy Records for Japan from 1958 to 1977, 377,841 autopsy cases were registered; a short summary of pathologic findings was included. Of the 377,841 cases, 409 (0.11%) were registered as idiopathic, nonspecific, interstitial, or viral myocarditis and 25 cases (0.007%) as giant-cell myocarditis. The annual incidence of the myocarditis varied at 5-year intervals and a remarkable increase was observed after 1974. An analysis of 19 cases of myocarditis and 19 of postmyocarditic cardiomegaly (PMC) showed a preponderance of males. Dilatation of ventricles was found in all cases of acute, subacute, and healing myocarditis, which histologically showed interstitial mononuclear cell infiltration, necrosis, and disarray of myocytes. Hypertrophy with ventricular dilatation, ventricular dilatation without hypertrophy, endo- or epicardial involvement, and right ventricular lipomatosis were found in 70%, 20%, 30%, and 10% of cases with chronic myocarditis and PMC, respectively. In these cases, residual inflammation, fibrosis, and hypertrophy was observed histologically.

Because of its nondestructive nature, topical nuclear magnetic resonance spectroscopy (TMR) was employed for the noninvasive evaluation of congenital cardiomyopathy (CM) in Syrian hamsters (BIO 14.6 strain). In a preliminary study, the size and pulse duration of the surface coil was determined to ensure that the detecting field covered the animal heart. The phosphate metabolite signals of the heart were distinguished from those of organs near the heart. At the cardiac apex, spectroscopically obtained constituent ratios of lipid/water by 1H-TMR and creatine phosphate/ATP by 31P-TMR were less in hamsters with CM than in the age-matched normal control animals, which was in accordance with biochemical analyses performed after killing the animals.

The results presented here show the inadequate attention that has been paid to myocarditis as a clinical entity and to employing endomyocardial biopsy examination as a valuable tool for diagnostic and research purposes. The results must be interpreted with caution, as otherwise false conclusions may be drawn as far as the epidemiological profile of cardiovascular diseases in Brazil is concerned. The data are likely to be influenced by the striking regional differences in development which are reflected in the scientific output. The high incidence of positive serology in patients in endemic areas of Trypanosoma cruzi infection is a further complication. It is possible that Chagas' disease may be overestimated clinically, decreasing therefore the interest in clarifying diseases with comparable clinical features. Myocarditis (excluding a protozoal etiology) and dilated (congestive) cardiomyopathy may serve as examples. Considering these various points, together with the complex and multi-disciplinary requirements of obtaining and interpreting catheter biopsies of the heart explains the limited clinical application of this technique in Brazil.

The diagnosis of myocarditis is discussed with reference to endomyocardial biopsy and the possible relation of dilated cardiomyopathy to myocarditis is explored. The various degrees of immune damage to the myocardium produced by myocarditis are reviewed, and evidence for altered immunity in dilated cardiomyopathy is assessed. The rationale for immunosuppressive therapy is surveyed. Both clinical and experimental data suggest that viral myocarditis is biphasic. The initial phase is infective with myocytolysis, lymphocytic infiltration, and a humoral immune response. The second phase is associated with a persistent antigen-antibody reaction between the virus and the myocardium. Myocarditis may be acute with lymphocytic infiltration and myocytolysis; persistent active, with continuing changes including widening of the interstitium and fibrosis; healing, with persistent inflammatory cell exudate but no myocyte necrosis; and healed, with the absence of necrosis and of inflammatory cell infiltrates but widening of the interstitium and fibrosis. This state is indistinguishable from dilated cardiomyopathy. The selection of patients for treatment and the regimens of treatment are discussed. Acute myocarditis or persistent active myocarditis are indications for therapy with steroids and the immunosuppressive agent azathioprine. Benefit is unlikely when myocarditis is healed. Lymphocytic inflammatory cell infiltration alone is not sufficient indication for such therapy, because such infiltration may be found in dilated cardiomyopathy and also in toxic myocarditis due to drugs. Results of immunosuppressive therapy for acute and active myocarditis are encouraging, but a prospective randomized study is needed.

The difficulties in diagnosing acute idiopathic myocarditis have been highlighted. Only about 30% of clinically suspected cases show morphologic evidence of an inflammatory infiltrate. Difficulties experienced in obtaining positive results include timing of the biopsy in relation to the acute symptoms of the patient, sampling error, and quantitative criteria. In addition, pressure from the referring physician may influence the pathologic interpretation, i.e., in making a morphologic diagnosis on slender evidence. Caution is also necessary in the interpretation of end-stage disease of dilated cardiomyopathy and "chronic myocarditis." This is important as it influences therapy with immunosuppressive agents. The importance of obtaining a detailed history of drugs to which the patient might have been exposed and can result in myocarditis is also stressed. Only if an accurate and unbiased pathologic evaluation can be made will a prospective, randomized multicenter trial yield useful information. The Dallas Myocarditis Panel has set forth useful criteria and guidelines in an attempt to classify the morphologic diagnosis of myocarditis. Semantic and diagnostic criteria for myocarditis can still be challenged, but the Dallas criteria for evaluation does allow an accurate assessment by all pathologists, in spite of individual variation.