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PubMed1994

Restrictive cardiomyopathies.

Restrictive cardiomyopathy has always been a fairly common cause of cardiac death in the tropics through endomyocardial fibrosis. In temperate climates, amyloidosis is the most common form of the disease, whereas Löffler's endocarditis is quite rare. Amyloidosis is more frequently encountered as the population ages, but restrictive cardiomyopathy is still rare outside the tropics. Differentiation between cardiac restriction and constriction is still the major clinical consideration as treatment for constrictive pericarditis is easily administered.

Amyloidosis↗

PubMed1991

Hypereosinophilic syndrome evolving to acute lymphoblastic leukemia.

We report a case of hypereosinophilic syndrome (HES) which later evolved into acute lymphoblastic leukemia (ALL). A 37-year-old man showed typical clinical manifestations of HES: pulmonary infiltrates, erythematous skin rash, deep vein thrombosis, endomyocardial fibrosis, and diffuse central nervous system dysfunctions. Although he was treated with prednisolone and hydroxyurea, marked eosinophilia persisted and lymphoblasts gradually increased in the bone marrow. He died of severe disseminated fungal infection after anti-leukemic therapy. Autopsy revealed marked fibrous thickening of the endocardium, bilateral common iliac vein thrombosis, and chronic hepatitis with fibrosis. Neither eosinophilic nor leukemic cell infiltration was seen in any tissue at autopsy. Including this case, 24 patients with ALL and hypereosinophilia have been reported in English-language literature. We discuss the relationship between eosinophilia and ALL, and the mechanisms, particularly the role of eosinophil cationic protein (ECP), in causing various organ system dysfunctions in HES.

Adult↗

PubMed1987

[Loeffler's endocarditis and disseminated viral infection].

We report a case of Löffler's endocarditis in a 60-year-old woman with multiple lymphadenopathies, blood eosinophilia and acute heart failure. The eosinophilia developed over a six-month period to a maximum of 54.5% eosinophiles for 7600 X 10(9) leukocytes/l. Five weeks before death lymph node biopsy revealed Hodgkin's disease with mixed cellularity. Autopsy disclosed parietal endocarditis with endomyocardial fibrosis and mural thrombi, principally in the inflow tract of the left ventricle, lesions typical of the fibroplastic parietal endocarditis described by Löffler. Examination of the lymph nodes at autopsy showed a disseminated viral infection probably due to a virus of the herpes group, as was confirmed by electron microscopy. Since these findings disagree with the interpretation of the lymph node biopsy we think the diagnosis of Hodgkin's disease should be withdrawn. Löffler's endocarditis is discussed with reference to pathological features, differential diagnosis and lymph node changes in the patient.

Diagnosis, Differential↗

PubMed1978

Left anterior hemiblock in adult Africans.

A total of 4,000 consecutive electrocardiograms covering an 8-yr period was studied and all cases with pure left anterior hemiblock reviewed on the basis of clinical diagnosis and subsequent follow-ups. There were 66 cases in all, representing 1.6% of the total series with an age range of 30--81 and a mean of 53.4 yr; 43 males to 23 females--a ratio of approximately 2 : 1. 34 cases (51.5%) were hypertensives all with a minimum diastolic pressure of 120 mm Hg before treatment. Congestive cardiomyopathy accounted for 16 cases (24.3%) and diabetes mellitus unassociated with other ailments for another 6 cases (9.1%). Other causes included mixed aortic valve disease with 2 cases (3%), endomyocardial fibrosis with 2 cases (3%). In 6 patients (9.1%), all above the age of 70, who had been admitted for minor surgical operations, no cause could be found. This etiological pattern differs from that seen in white populations where ischemic heart disease is by far the commonest cause. The extreme rarity to left anterior hemiblock in rheumatic mitral valve disease is considered of help in separating cases of lone rheumatic regurgitation from those of mitral regurgitation complicating congestive cardiomyopathy if and when diagnostic difficulty arises.

Adult↗

PubMed1977

Pathology of cardiomyopathy. An autopsy analysis of twenty-five cases.

Pathology of twenty-five autopsied causes of cardiomyopathy is presented. Twenty cases were those of congestive cardiomyopathy. The ages varied between eleven and sixty-eight years and there were nine females and eleven males. The heart weights ranged between 240 gm and 600gm. Thrombi in the heart were encountered in 60% cases. Five cases conformed to endomyocardial fibrosis. The age ranged between twelve and forty years and all were males. Three cases out of five had severe left inflow tract involvement. Only one case showed predominant right sided involvement. No cases of hypertrophic cardiomyopathy were encountered. There appears to be a higher incidence of EMF in the Southern parts of India as compared to the other regions where COCM is most commonly encountered. The variation in the type of cardiomyopathy encountered in different parts of India, may well be related to dietary, climatic conditions or infections, peculiar to that area.

Adolescent↗

PubMedDate not supplied

[Parietal fibroplastic endocarditis].

Parietal fibroplastic endocarditis is commonly observed in two main diseases: Löffler' endocarditis and Davies' endomyocardial fibrosis. Endocarditis' damage is explained by the cytolytic effects of basic proteins present in eosinophil granules (major basic protein and cationic protein); long standing and amount of eosinophilic cells condition anatomic lesions. The clinical features are explained by the ventricular seat of endocarditis lesions (right, left, or both). They include in all cases: tricuspid and/or mitral insufficiency, adiastolic syndrome. Two-dimensional echocardiography has improved the diagnosis; ventricular angiocardiography remains the reference examination. Prognosis has been largely improved these recent past years by surgical endocardiectomy and valvular conservative surgery among children.

Endocarditis↗

PubMed1994

Endomyocardial biopsy--technical aspects experience and current status. An Indian perspective.

Our study ellucidates the utility of endomyocardial biopsy (EMB) in various cardiac-muscle disorders seen in a tropical country like India. The procedure has been successfully performed in 501 patients (572 procedures) at our centre from April 1985 to December 1992. This included 60 infants and children. The indications were dilated cardiomyopathy (DCM) in 214, non-specific aortoarteritis in 91, rheumatic heart disease in 75, restrictive cardiomyopathy in 45, constrictive pericarditis in 14 and miscellaneous in 62 patients. There was no mortality, however, one patient developed cardiac tamponade and another sustained ventricular tachycardia requiring cardioversion. There was transient atrial fibrillation in six patients and all these had acute rheumatic heart disease. Transient complete heart block occurred in six patients with underlying left-bundle branch-block. Histological examination of EMB revealed myocarditis in 34/214 (15.4%) patients in DCM group and helped in following up these cases on immunosuppressive treatment. In the presence of restrictive haemodynamics it could identify amyloidosis in four patients. It was also helpful in differentiating between endomyocardial fibrosis and chronic constrictive pericarditis. In patients with non-specific aorto-arteritis significant histological changes of inflammatory myocarditis were observed in patients especially in congestive heart failure. Furthermore, it was helpful in identifying the nature of cardiac tumour in one patient. Its utility has also been evaluated in disorders, including rheumatic heart disease, peripartum cardiomyopathy and systemic disorders like systemic lupus erythematosis. Even in the absence of cardiac-transplant programmes at national level we have found EMB to be a useful investigation in a tropical country like India.

Adolescent↗

PubMed1976

Myocardial disease in Nigerians.

The problem of heart disease of unknown aetiology as it is found in the Nigerian environment is presented. The fact that it is nearly impossible on many occasions to make a conclusive diagnosis on clinical grounds alone of either endomyocardial fibrosis and heart muscle disease is discussed and the need for haemodynamic and angiographic studies to make the differentiation is stressed. The clinical, radiological, electrocardiographic, and haemodynamic features of each of the two varieties of "cardiomyopathy" is presented and the distinguishing features are emphasised. The occasional difficulty in differentiating heart muscle disease from hypertensive heart disease is discussed and the fact that the two differ distinctly on haemodynamic pressure studies is established. Previous published hypotheses regarding the aetiology of these cardiac conditions are discussed, and suggestions are made as to the lines of future research including virological studies and the possible myocardiopathic effect of some of the local traditional herbs.

Cardiomyopathies↗

PubMed1987

[Roentgenologic data patterns and hemodynamics in biopsy-proven dilated myocardial diseases].

The chest plain images of 63 patients suffering from histologically proved dilated myocardial diseases: primary cardiomyopathy (27), active and residual myocarditis (23), toxic cardiomyopathy (3), endomyocardial fibrosis (2), thesaurismotic cardiomyopathy (2), obstructive intramural fine vessel diseases (6) were analysed and correlated with invasively determined parameters: end-diastolic volume index, wall mass index, left ventricular end-diastolic pressure, pulmonary artery mean pressure, ejection fraction. The x-ray findings proved to occur in four different patterns which were defined as left heart involvement and three different patterns of bilateral left and right heart involvement representing various stages of left heart failure. This kind of image evaluation with functional classification is characterized by increased sensitivity, accuracy and information in detail in comparison with the heart lung index or other usual procedures.

Adult↗

PubMed1998

[Löffler fibroblastic endocarditis in the thrombotic stages in isolated right ventricular tissue eosinophilia].

We report on a male, 31 year old, Turkish patient with an intracardiac mass in the right ventricle, reduction of performance and weight, as well as intermittent fever. No eosinophilia was documented in the peripheral blood; cardiac function was primarily normal. Besides the differential diagnosis of Löffer's endocarditis (endomyocardial fibrosis) an inflammatory disease and a malignant cardiac tumor were suggested. The diagnosis of Löffler's endocarditis could not be confirmed morphologically by echocardiography nor histologically by right ventricular biopsy. Operative removal of the mass lesion was necessary because of fast tumor progression, fulminant pulmonary embolism, and infiltration of the tricuspid valve. Only then, histologically Löffler's eosinophilic endocarditis of thrombotic stage was diagnosed. Antiphlogistic therapy with cortisone was initially performed. With a dose reduction after 6 months, a relapse of the thrombotic mass occurred. Therefore, continuous treatment with cortisone and azathioprine was induced followed by further tumor regression and further clinical stabilization since 8 months of treatment.

Adult↗

PubMed1983

Endomyocardial disease--clinical features.

The clinical features of tropical and temperate zone endomyocardial fibrosis (EMF) are the same, allowing for certain regional, environmental and possibly genetic variations. For example, the seasonal incidence in rainy humid areas probably reflects the large and repeated parasitic infestations in tropical EMF, while the absence of tissue eosinophilia in organs other than the heart in tropical EMF may reflect racial and environmental differences between tropical and western geographical areas that have still to be elucidated. That EMF occurs in Europeans who have lived in the tropics is undoubted, but the absence of right ventricular involvement in Europeans in the tropics, but not in temperate climes, is unexplained; perhaps it is a chance finding. It is also apparent that the extreme degrees of right ventricular EMF that are commonly seen in the tropics, with almost complete obliteration of the ventricular cavity are not usually seen in eosinophilic EMF in temperate areas. Involvement of both ventricles and of both atrioventricular valves is, however, common both in the tropics and in temperate climate EMF.

Endomyocardial Fibrosis↗

PubMed1984

Serum immunoglobulins in children with cardiomyopathies.

Serum immunoglobulins G, A, M and E were determined in four groups of children, including 14 patients with dilated cardiomyopathy (DCM), 17 with endomyocardial fibrosis (EMF), 18 with rheumatic heart disease (RHD) and 35 age-matched controls, in an attempt to assess the role of infections in the aetiology of childhood cardiomyopathies. Mean IgA and IgM concentrations in all four groups were not significantly different. However, mean IgG concentrations were higher in patients with EMF (1729 mg/100 ml), DCM (1745 mg/100 ml) and RHD (1978 mg/100 ml) than in controls (1429 mg/100 ml), and in about 30% of these cardiac patients the levels were higher than the 97th percentile for controls. In addition, mean serum IgE was higher in EMF (3577 iu/ml) than in RHD (2814 iu/ml), DCH (1000 iu/ml) or controls (1666 iu/ml), and in 29% of the EMF patients serum IgE exceeded 6834 iu/ml, the 89th percentile for controls. Intestinal helminths and moderate eosinophilia (mean count, 10%) were detected in most of the EMF patients. These results suggest that some cases of EMF and DCM are, like RHD, complications of infections, and that intense helminth-induced anaphylaxis is a probable cause of cardiac damage in such cases of EMF.

Adolescent↗

PubMedDate not supplied

Cardiac manifestations in dermato-polymyositis.

OBJECTIVE: To describe the functional and anatomic heart abnormalities of patients with dermato-polymyositis. METHODS: Thirty-two consecutive patients with dermato-polymyositis (DM/PM) were studied. Patient assessments included a questionnaire for cardiac symptoms, physical examination, muscular enzymes, standard electrocardiogram (EKG), chest radiographs, spirometry, Holter monitoring and color Doppler flow (CDF) studies. Features evaluated with CDF included left ventricular diastolic dysfunction (LVDD), hyperdynamic heart, mitral valve prolapse, and endomyocardial fibrosis. RESULTS: Twenty-five patients (78%) were female. The mean age was 43 years and the mean disease duration 6 years. Twenty-six (81%) patients had dermatomyositis. Only 2 (6%) had cardiac symptoms. Spirometry was performed in 20 patients, Holter monitoring in 23 and CDF in 26 patients. Electrical disturbances detected by either EKG or Holter were present in more than half of the patients studied. LVDD was identified in 11 patients (42%) and hyperdynamic heart in 4 (15%). None of the 26 patients studied by CDF had mitral valve prolapse. No significant differences in the frequency of cardiac abnormalities, including LVDD, were observed in relation to the diagnosis of DM or PM, the presence of Raynaud's phenomenon or calcinosis, or the disease activity status at the moment of the study. CONCLUSIONS: Subclinical heart abnormalities are frequent in patients with DM/PM. LVDD appears to be common. Our findings suggest that cardial assessment of patients should be performed in order to detect abnormalities requiring specific therapy.

Adolescent↗

PubMed1985

Eosinophilic disorders affecting the myocardium and endocardium: a review.

A wide range of disorders give rise to eosinophil counts greater than 1.5 X 10(9)/l (hypereosinophilia) and cardiac injury. The best known of these is eosinophilic endomyocardial disease (Löffler's endomyocardial fibrosis), which occurs as a major complication of the idiopathic hypereosinophilic syndrome. Here the heart damage appears to be a direct result of tissue injury produced by toxic eosinophil granule proteins within the heart. However, it is not known what causes the eosinophilia in these patients, why the eosinophils degranulate, or why the endocardium is especially susceptible to this type of injury. A number of parasitic infections may give rise to eosinophilic myocarditis. This is usually the result of the presence of the parasites within the myocardium where they die within inflammatory lesions, which may be extensive. Occasionally, drug reactions and rejection of a transplanted heart may produce eosinophilic myocarditis. Allergic granulomatosis and vasculitis (the Churg-Strauss syndrome), which gives rise to granulomas involving the myocardium, and eosinophilic (hypersensitivity) myocarditis usually respond rapidly to treatment with steroids. However, diffuse myocardial involvement may lead to heart failure, and some of these patients may later develop dilated cardiomyopathy. It is concluded that the heart may be affected by a variety of diseases in which eosinophils are a prominent component in the inflammatory cell infiltrates. Eosinophils themselves may contribute to some of the myocardial cell injury which occurs in these diseases, and attempts to limit this with steroids may be worthwhile in some patients.

Cardiomyopathies↗

PubMed2004

Environmental influences on the trace element content of teeth--implications for disease and nutritional status.

The aim of this study was to compare the trace element content of children's primary teeth from Uganda and the UK. The Ugandan teeth were from children living in an area where endomyocardial fibrosis (EMF), a cardiac disease, is prevalent. The latter has been putatively linked to insufficient magnesium intake and excess cerium exposure. Primary teeth were collected from 21 Ugandan and 27 UK children. The crowns and roots of the teeth were separated and the former digested and analysed for several major and trace elements by inductively coupled plasma mass spectrometry (ICP-MS) and atomic emission spectrometry (ICP-AES). In addition, the enamel and dentine of eight UK and seven Ugandan primary teeth were isolated via density separation and analysed as above. The data were assessed using non-parametric statistical tests. The Ugandan teeth contained significantly (P < 0.05) greater concentrations of strontium, barium, cerium, lanthanum, praseodymium and significantly less zinc than the UK teeth. No significant difference in the concentrations of aluminium, calcium, copper, magnesium, lead and uranium were found. Analysis of enamel and dentine demonstrated that the former was enriched with several elements including cerium. It is concluded, that the environment, influences the trace element content of primary teeth and this may be useful for monitoring nutritional status. With respect to a geochemical cause for EMF, there is no positive evidence that EMF in Uganda is associated with reduced magnesium and increased cerium uptake in primary teeth. This does not, however, exclude cerium from playing a role in the aetiology of EMF.

Adolescent↗

PubMed1985

[Reconstructive mitral valvuloplasty. Results apropos of 53 cases].

In the last three years, 53 patients have undergone a valvuloplasty according to the Carpentier's principles. The patients age ranged from 5 to 35 years, with a mean of 14.3 years. The aetiology was congenital in one case, RHD in 45 cases, and endomyocardial fibrosis (EMF) in 7. The pre-operative condition was severe with 25 patients in class III and 10 patients in class IV. There was a cardiomegaly with a mean CTR of 0.70 (extremes 0.50 and 0.85). The mitral lesions were pure mitral insufficiency in 49 cases, and mixed lesions with predominant insufficiency in 4 cases. There were also 6 aortic and 4 tricuspid significant insufficiencies. The patients have had a valvuloplasty with chordae shortening (n: 46) valvular resection (n: 3), chordae resection (n: 8), annuloplasty without ring (n: 38) or with ring (n: 2). There were associated procedures: endocardectomy (n: 7), aortic valvuloplasty (n: 3), aortic valve replacement (n: 3), tricuspid annuloplasty (n: 4). There were 3 post-operative deaths in children in class IV, with cardiomegaly (CTR 0,80), and systemic pulmonary hypertension. There were 3 mitral valve replacements (MVR) in the first month for failure of the plasty, 2 late MVR for endocarditis and rheumatic evolution. There were 3 mitral valve replacements (MVR) in the first month for failure of the plasty, 2 late MVR for endocarditis and rheumatic evolution. There were 2 late deaths (recurrent mitral insufficiency, serum hepatitis), Among 43 long-term follow-up of patients with a plasty (mean follow-up 18 months), the result has been very good 39 times. There were 4 patients with a significant residual mitral incompetence.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1988

[Cardiac involvement in Behçet's disease. 12 cases].

Fifteen cardiac manifestations observed in 12 of a series of 196 patients with Behçet's disease are reported. In this retrospective study conducted in an internal medicine department, 5 cases of pericarditis and 4 cases of myocardial infarction were encountered. The other cardiac manifestations were observed only once. They included ventricular aneurysm, endomyocardial fibrosis of the right heart, aortic insufficiency, mitral valve insufficiency, mitral valve prolapse, and right heart failure consecutive to pulmonary arterial hypertension. There was no relationship between the severity of cardiac lesions and that of the extracardiac manifestations of the disease. Comparison of these data with those found in the literature showed that pericarditis is the most frequent pathology, but it usually regresses rapidly. Lesions of the coronary arteries, with or without myocardial infarction, consist of stenosis, occlusion or pseudoaneurysm requiring surgical treatment. Myocardial lesions (with the possibility of pseudoaneurysm) and endomyocardial right heart fibrosis are exceptional but fairly characteristic of Behçet's disease which they should suggest. Hughes-Stovin's syndrome may be complicated by pulmonary arterial hypertension, although death is generally caused by massive haemoptysis.

Adult↗

PubMed1990

[A case of myxedema with diffuse myocardial fibrosis proven by endomyocardial biopsy].

We report a middle-aged woman with myxedema heart who presented both clinical features resembling dilated cardiomyopathy and the diffuse myocardial fibrosis proven by endomyocardial biopsy. Thirty years previously, when she was 36 years old, partial thyroidectomy had been performed after a diagnosis of hyperthyroidism was made. Four years later, she experienced dry skin and peripheral edema, and hypothyroidism was diagnosed. Several months after, replacement therapy for hypothyroidism improved her symptoms. However, the therapy was discontinued because of her ignorance of the disease. Twenty six years later (64 years old), she felt exertional dyspnea, and was admitted to Fukuoka University Hospital for evaluation of her cardiac state. Thyroid function test revealed primary hypothyroidism with low T3, low T4 and high TSH levels. Cardiothoracic ratio on chest X-ray film was 69%. Electrocardiogram showed low voltage in the limb leads and intraventricular conduction disturbance. Echocardiogram demonstrated marked dilatation and severely reduced wall motion of the left ventricle and pericardial effusion. Left ventriculogram showed diffuse hypokinesis with 27% of the ejection fraction. No significant stenosis was observed on coronary arteriogram. Seventeen-month replacement therapy did not improve these cardiac findings significantly. Transvenous right ventricular endomyocardial biopsy demonstrated diffuse myocardial fibrosis without inflammatory infiltrate, which was interpreted as a sequel of interstitial lesions of the myxedema heart such as edema or mucoid infiltration. This pathological finding suggests that long-standing untreated hypothyroidism can cause irreversible myocardial damage.

Aged↗