[Fibroplastic endocarditis of the right heart. Apropos of a case and review of the literature].
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A patient with advanced X-linked myodystrophy and marked cardiovascular changes is described. Current understanding of the problem of heart muscle involvement in the myodystrophic process as well as treatment of such patients are analysed on the basis of data reported in Soviet and foreign literature.
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Endomyocardial biopsy is a safe, reliable, and reproducible technique to confirm or establish the diagnosis of the pathology that affects cardiac function in conditions previously classified as cardiomyopathies of unknown cause. The first 64 consecutive patients to undergo endomyocardial biopsy were reviewed to determine its utility in various clinical settings. Furthermore a review of literature relating to endomyocardial biopsy is presented. On the basis of these findings, we conclude that endomyocardial biopsy is indicated in the clinical setting of dilated cardiomyopathy, for evaluating underlying myocarditis. In patients with dysrhythmias or clinical myocarditis, endomyocardial biopsy may disclose myocarditis or changes consistent with congestive cardiomyopathy. In patients with systemic disease endomyocardial biopsy is instructive to prove or to exclude myocardial involvement. In patients with angina-like chest pain but with normal coronary angiograms or exercise tests, endomyocardial biopsy may prove underlying heart disease.
The author offers a critical review of the present knowledge about myocardiopathies and points out discordant opinions of various authors. He presents his own classification of this inhomogeneous group of myocardial diseases, based on the etiological principle. Special attention is given to the present status of diagnostic methods, the author pointing out the nonspecificity of individual signs and symptoms of the disease. It is emphasized that in two thirds of patients even a general practitioner is capable of diagnosing myocardiopathy on the basis of routine cardiological examinations (patient's history, physical examination, ECG and X-ray). This is of great practical importance because the prognosis--although essentially unfavourable--depends to a great extent on an early diagnosis. Finally, principles of therapy at myocardiopathies are presented.
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The case of a 71 year old woman with an idiopathic hypereosinophilic syndrome is reported. Bidimensional ultrasonography was in favor of endomyocardial involvement. This case was original as nephrotic syndrome developed, due to membranous glomerulopathy which was complicated with renal vein thrombosis. Both eosinophilia and nephrotic syndrome greatly improved with corticosteroid treatment. Nonetheless, the patient died suddenly after 2 years, and this demise is in keeping with the overall poor prognosis of idiopathic eosinophilic syndrome.
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Monoclonal antibodies are being used as specific therapeutic agents for treating organ rejection. This type of treatment requires that the pattern and type of infiltrating cells in the graft during acute rejection be defined. In this study 24 endomyocardial biopsies from cyclosporine-treated human heart allograft recipients were obtained during acute rejection. The biopsies were stained with immunological markers to define the infiltrating cells. The biopsies were divided into three groups; mild, moderate and resolving acute rejection. In all groups T-lymphocytes (marked with the monoclonal Leu-4) were the predominant cells with absence of B-lymphocytes (marked with T015). The results of this study showed that cyclosporine-treated heart transplant recipients have a mixed suppressor/cytotoxic (Leu-2a) and helper/inducer (Leu-3) myocardial infiltrate in contrast to the biopsies of recipients treated with azathioprine, in which suppressor/cytotoxic T-lymphocytes (Leu-2a) were predominant. A clear sequential pattern of changes in the T-cell subpopulations did not emerge, although macrophages became more prevalent with resolving rejection.
Reference to the background of myocarditis since the early nineteenth century has been made to emphasize the problem of diagnosis of this condition which has been largely overcome by the introduction of the bioptome and morphological evaluation of the fresh endomyocardial biopsies obtained by these means. A definition and classification into active, healing and healed phases has been provided and constitutes a rational approach to therapy with corticosteroids and immunosuppressive agents. The observation that myocarditis is evident in biopsy tissue from patients suspected with dilated cardiomyopathy and additional virological and immunological investigations have shed light on a possible pathogenetic mechanism which in some patients with this condition is an infectious immunological process. In addition, when myocarditis in the active or early healing phases is found, it has been shown that therapy is beneficial to the patient. Myocarditis is also the initial morphological expression of endomyocardial disease associated with eosinophilia. The eosinophils are in addition morphologically abnormal in the form of degranulation. Examination of blood films and endomyocardial biopsies has defined the earliest form of this disease process and early treatment can also now be instituted. This up-to-date overview of our present state of knowledge has repeatedly demonstrated that biopsy examination in the diagnosis of myocarditis plays a vital role.
In patients with impaired left ventricular function in whom dilated cardiomyopathy is initially suspected after performance of comprehensive diagnostic studies, histologic evidence of myocarditis or status-post myocarditis is being documented with increasing frequency since the systematic use of endomyocardial biopsy has been incorporated into the work-up. This investigation was undertaken to analyze the histologic, clinical, hemodynamic and immunologic findings in these patients to delineate possible relationships between histologically-documented myocarditis and dilated cardiomyopathy. Incidence of histologically-documented myocarditis. In our patient population, 41 of 150 patients with impaired left ventricular function, the etiology of which had been unknown, histologic evidence of myocarditis was documented. In seven of eight in whom active myocarditis was diagnosed at initial biopsy, after a mean follow-up period of two years the histologic findings were consistent with dilated cardiomyopathy. In similar groups of patients comparable incidences of myocarditis have been reported by Parrillo et al. in 19 of 100 (19%) and Fenoglio et al. in 34 of 135 patients (25%). A higher incidence has been reported by Zee-Cheng et al. in 22 of 35 patients (63%). Accordingly, pooled data indicate that the overall incidence of histologically-documented myocarditis was 30% in 528 patients initially suspected to have dilated cardiomyopathy. The variances in the incidence reported by the respective authors may be attributable to differences in the histologic definition of myocarditis, number of biopsies obtained, size of the patient population and the epidemiologically-related incidence of viral disease.(ABSTRACT TRUNCATED AT 250 WORDS)
Ninety-eight subjects with a diagnosis of primary rheumocarditis made at a pediatric clinic in childhood were examined for the central hemodynamics by tetrapolar chest rheography. Based on a clinical and instrumental study rheumatic heart disease was diagnosed in 3 subjects; in 37 subjects, no heart pathology was recognized; myocarditic cardiosclerosis was diagnosed in 22, obesity in 20, and neurocirculatory dystonia of the cardial type in 16 subjects. The data obtained point to the overdiagnosis of rheumatic fever.
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