Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease.

Chew, Christopher; Ziady, Galal M.; Raphaël, Maurice; Nellen, M; Oakley, Celia M.

doi:10.1136/hrt.39.4.399

Cited by 92 publications

(17 citation statements)

References 52 publications

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“…The only other report on coronary arteries in EMF is that of Chew el al. [16] who did not find any abnormality in 3 patients with nontropical EMF with hypereosinophilia that they studied.…”

Section: Discussionmentioning

confidence: 88%

Coronary Angiographic Features in Endomyocardial Fibrosis

Balakrishnan¹,

Sasidharan²,

Venkitachalam³

et al. 1983

Cardiology

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Selective coronary cineangiography was performed in 24 patients with angiographic features suggestive of endomyocardial fibrosis (EMF). 8 patients had right ventricular disease, 2 had left ventricular disease and 14 had biventricular disease. The abnormal features found in the coronary arteries of these patients were: (a) vascular blush or neovascularity in 54.2% of patients and (b) localized ventricular filling from the region of the blush in 16.7%. Generalized tortuosity of the coronary arteries, considered to be a nonspecific change associated with ventricular enlargement and hypertrophy, was seen in 50%. This study lends support to one of the hypotheses which implies the role of mural thrombus in the evolution of the pathological lesion of EMF.

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“…The only other report on coronary arteries in EMF is that of Chew el al. [16] who did not find any abnormality in 3 patients with nontropical EMF with hypereosinophilia that they studied.…”

Section: Discussionmentioning

confidence: 88%

Coronary Angiographic Features in Endomyocardial Fibrosis

Balakrishnan¹,

Sasidharan²,

Venkitachalam³

et al. 1983

Cardiology

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“…We studied 12 Cardiac involvement in Churg-Strauss syndrome shown by echocardiography posterior wall. We took care to limit sampling to these areas on the myocardium itself and to avoid specular echoes from the endocardium or epicardium.…”

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confidence: 99%

Cardiac involvement in Churg-Strauss syndrome shown by echocardiography.

Morgan

Raposo

Gibson

1989

Heart

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SUMMARY To define the range of cardiac involvement in the Churg-Strauss syndrome, M mode, continuous wave Doppler, and cross sectional echocardiograms were recorded in twelve patients with the disorder. The M mode recordings were digitised and the cross sectional images were recorded with standardised gain settings to determine regional myocardial echo amplitude. Left ventricular end diastolic and end systolic dimensions were increased above the normal 95% confidence interval in four patients, three of whom showed a depressed shortening fraction. Mitral regurgitation was present in six patients; this was severe enough to need valve replacement in two. Mean echo amplitude in both the septum and the posterior wall was significantly increased above normal by a mean (SD) of4-87 (2-57) dB, suggesting the presence ofmyocardial fibrosis. There was no evidence of subendocardial involvement as there is in other hypereosinophilic syndromes.Mitral regurgitation is common in the Churg-Strauss syndrome. This cannot be ascribed to involvement ofthe cusps or chordae and it occurs even when ventricular function is well preserved. It is suggested that mitral regurgitation is caused by diffuse myocardial fibrosis.The Churg-Strauss syndrome is recognised by the combination of blood eosinophilia, eosinophilic tissue infiltration, extravascular fibrinoid necrotising (allergic) epithelioid and eosinophilic granuloma formation, and disseminated necrotising vasculitis occurring in asthmatic patients.' 2 Lanham et al noted that "the histological components of the syndrome often do not coexist temporally or spatially" and suggested that the clinical pattern of a prodromal phase of allergic disease, followed by a second phase characterised by eosinophilic tissue infiltration and peripheral blood eosinophilia culminating in a third vasculitic phase, should supercede strict adherence to histopathological criteria for the purposes of diagnosis.' Myocardial fibrosis, particularly in the subendocardial region, is a characteristic feature of the idiopathic hypereosinophilic syndrome4 and it has been attributed directly to the eosinophilia itself. Possible cardiac involvement in patients with ChurgStrauss syndrome and comparable blood eosinophilia, however, is less well defined. We therefore studied a series of patients with Churg-Strauss syndrome by M mode, continuous wave Doppler, and cross sectional echocardiography to assess whether they were also at risk for heart disease.Requests for reprints to Dr J M Morgan, National Heart Hospital, London WIM 8BA.Accepted for publication 6 April 1989 Patients and methodsWe studied 12 patients in whom the diagnosis of Churg-Strauss syndrome was made on the basis of histological criteria" and clinical features.' The blood eosinophil count was raised in all (eosinophil counts ranged from 2-3 to 11 x 109 1 at presentation). The results of echocardiography were compared with those in 20 age and sex matched controls with no clinical evidence of heart disease in whom stress test had been negative at high wo...

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“…[1][2][3] The pathologic basis for most cases of restrictive cardiomyopathy includes infiltrative disorders such as amyloidosis, hemochromatosis, glycogen storage disease, mucopolysaccharidoses, endomyocardial fibrosis with or without eosinophilia, endocardial fibroelas-tosis, sarcoidosis, and collagen-vascular diseases like scleroderma. [1][2][3][4][5][6][7][8][9] In this report we describe the clinical, hemodynamic, and morphologic data from four patients who presented with restrictive cardiomyopathy that was not associated with the usual pathologic entities responsible for this clinical-hemodynamic syndrome.…”

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confidence: 99%

Idiopathic restrictive cardiomyopathy.

Siegel¹,

Shah²,

Fishbein³

1984

Circulation

116

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This report details the clinical, hemodynamic, and morphologic data from four patients 59 to 77 years old (mean 66) with a primary restrictive cardiomyopathy. All patients had symptoms of congestive heart failure, jugular venous distention, and murmurs of mitral and tricuspid regurgitation. Four patients required pacemakers, three for the brady-tachy syndrome and one for complete heart block. Chest x-ray demonstrated cardiomegaly in all four patients and pulmonary congestion and/or pleural effusions in three. Echocardiographic examination documented left atrial enlargement in all patients, along with normal left ventricular internal dimensions. Global left ventricular systolic function was normal in all, and left and right ventricular filling pressures were elevated and similar in three. A dip and plateau pattern was present in the pressure tracings of two of three patients. Unlike previous reports of restrictive cardiomyopathy, in our four patients there was no specific morphologic cause noted at necropsy. Pathologic evaluation demonstrated biatrial dilatation in all patients, with thrombi present in the atrial appendages in three. Normal ventricular cavity size and mild right ventricular hypertrophy were present in all patients and mild-to-moderate left ventricular hypertrophy was present in two. There were no significant pericardial, endocardial, or valvular abnormalities and no infiltrative myocardial disorders were present. Light and electron microscopic studies demonstrated only interstitial fibrosis of the myocardium. A restrictive hemodynamic profile may be observed in the absence of specific infiltrative disorders and affected patients may exhibit a prolonged clinical course of 4 to 14 years (mean 9). However, in these patients congestive heart failure responded poorly to medical therapy or surgical correction of valvular regurgitation, which is common in this disorder.

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Primary restrictive cardiomyopathy. Non-tropical endomyocardial fibrosis and hypereosinophilic heart disease.

Cited by 92 publications

References 52 publications

Coronary Angiographic Features in Endomyocardial Fibrosis

Coronary Angiographic Features in Endomyocardial Fibrosis

Cardiac involvement in Churg-Strauss syndrome shown by echocardiography.

Idiopathic restrictive cardiomyopathy.

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