[Hypereosinophilic syndrome. Report of 2 cases with different clinical features and identical cardiac involvement].
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Many investigators consider viral myocarditis as an important cause of a dilated cardiomyopathy (DCM)-like state. In assessing myocarditis in endomyocardial biopsy samples, two sets of criteria, i.e., the Edwards and the Dallas criteria, are employed. However, no criteria have been established for cell infiltration in autopsy cases, nor have biopsy criteria for myocarditis been applied in autopsy cases. We analyzed the clinicopathologic features and small round cell infiltration in 42 autopsy cases whose clinical diagnoses were DCM, employing both the Edwards and the Dallas criteria. Of the 42 cases, 12 (29%) showed positive results for both sets of criteria. These 12 positive patients were proven to have myocarditis by autopsy and they showed more severe clinical features than 30 cases with negative cell infiltration.
In patients with arrhythmogenic right ventricular disease (ARVD), life threatening ventricular tachyarrhythmias and sudden cardiac death mostly occur in adolescence, or in young adults before the age of 40. In the right ventricle, progressive fibrolipomatous replacement of the ventricular myocardium is pathognomonic. In severe cases progressive congestive right heart failure can develop although mild forms are extremely difficult to recognized. In most cases the disease can be diagnosed only by elaborate investigation. Right ventricular cineangiography, myocardial biopsy, MRI, and electrophysiological investigation are the most important diagnostic procedures. If the disease is diagnosed in an early stage the risk of life-threatening arrhythmias can be reduced by carefully selected antiarrhythmic therapy.
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A new operation, the resection of the endocardium, is proposed in the case of fibrous constrictive endocarditis. I performed this operation in 1971, for the first time, with good results. I operated on two other patients with success in 1973 and 1975. The results I have obtained indicate that this new technique is possibly the answer to a disease which otherwise ultimately results in death.
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Carcinoid heart disease is a complication of a neuroendocrine carcinoid tumor. Morphologically, it is characterized by the formation of fibrotic plaques with deposition of extracellular matrix in the subendocardium, frequently causing heart valve dysfunction and cardiac failure. Because members of the transforming growth factor-beta (TGF-beta) family are known to stimulate fibroblasts in their production of extracellular matrix, we investigated the expression of the three isoforms of TGF-beta and the binding protein for latent TGF-beta 1 (LTBP) in carcinoid plaques of the right side of the heart, as well as from control tissue, using immunohistochemistry. Tissue specimens were obtained intraoperatively from nine consecutive patients undergoing valve replacement surgery. TGF-beta 1 and TGF-beta 3 were detected in the fibroblasts of all plaques analyzed, whereas TGF-beta 2 was only rarely expressed. The localization of LTBP was partly concordant with that of TGF-beta 1, but the positive staining for LTBP was extracellular. Sections from unaffected heart tissue contained few fibroblasts in the subendocardium, showing only weak or no immunostaining for TGF-beta 1, -beta 2, and -beta 3 and no staining for LTBP. These results suggest that TGF-beta may play a role in the proliferation of fibroblasts and their matrix production in carcinoid heart lesions.
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After presenting five individual cases, the authors confirm the possibility of a diagnosis of sudden cardiac death where myocardial and/or coronary arteries lesions were present, by performing an exhumed body's autopsy even after a considerable amount of time after death. With regard to this procedure, performed only by experienced consultants specializing, a particularly accurate verification is necessary.
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Chronic constrictive pericarditis is a difficult diagnosis and may present atypically. The authors report three clinical cases and review the diagnostic strategy of constrictive pericarditis. In these three patients, the diagnosis was finally made after one or more years of symptomatic disease and after several diagnostic work ups and ineffective treatments. In cardiac failure, pericardial calcification is often not observed on chest X-ray and Doppler echocardiography is usually the diagnostic investigation. Adiastole presents with dilatation of the vena cava and atria, contrasting with normal ventricles without major valvular disease. Doppler echocardiography enables distinction of constrictive pericarditis from restrictive cardiomyopathy: normal myocardium, thickened pericardium, specific septal motion, inspiratory increase in right ventricular dimensions, premature opening of the pulmonary valve, important variations in ventricular filling with respiration, expiratory diastolic reflux in the hepatic veins. Catheterisation confirms adiastole and may suggest a pericardial aetiology in characteristic cases, associated with only mild increases in pulmonary artery pressure. If need be, the pericardial thickening > 4 mm may be observed with magnetic nuclear resonance imaging and, when a doubt remains with respect to the diagnosis of cardiomyopathy, the absence of fibrosis on endomyocardial biopsy provides the diagnosis and indication for curative surgery: pericardectomy.