Endomyocardial fibrosis

We report the case of a 51-year-old man of central African origin. Medical evaluation revealed severe heart failure. Echocardiography disclosed poor left ventricular function. The apex of the left ventricle showed complete obliteration and retraction. Magnetic resonance imaging revealed subendocardial hyperenhancement of the apex of the left and right ventricle, strongly suggesting endomyocardial fibrosis. For this particular patient a conservative approach (non-surgical) was decided on, and until now--12 months after termination of cardiac rehabilitation--proves successful.     

Endomyocardial fibrosis and eosinophilia.

Absolute eosinophil counts were assessed in 15 African patients with proven endomyocardial fibrosis. Though the mean eosinophil count in patients with endomyocardial fibrosis was higher compared with the normals reported from Kampala (1-13 vs 0.72X10(9)/1), the absolute range was comparable. A high percentage of patients with endomyocardial fibrosis had malarial parasites, high malarial antibody titres, hookworms, or strongyloides, but the correlation of eosinophilia to various parasitic infections was poor. Both eosinophilia and parasitic infections are common in the tropics and they effect patients with endomyocardial fibrosis no more than the population at large. Other aetiological factors, genetic, environmental, and immunological, are felt to be important in the causation of endomyocardial fibrosis in Uganda and evidence for this is reviewed. Though there is a similarity in pathological features, African endomyocardial fibrosis is a distinct entity from Löffler's endocarditis and cardiac lesions seen in eosinophilic leukaemia or reactive eosinophilia. There is no hard evidence to suggest that African endomyocardial fibrosis is a variant of Löffler's endocarditis caused by parasitic infections via eosinophilia.     

Endomyocardial fibrosis in dermatomyositis

The case reported in this paper, besides illustrating many of the clinical and pathological aspects of chronic dermatomyositis, documents a yet undescribed feature of involvement of the heart in dermatomyositis, namely endomyocardial fibrosis. The pathogenetic mechanism of such a happening is discussed, and its possible relationship to tropical endomyocardial fibrosis is considered.     

Endomyocardial fibrosis in children

We describe 10 children with endomyocardial fibrosis who underwent surgical treatment between 1978 and 1999. Seven were male and 3 female, with an age range from 4 to 15 years, having a mean age of 11 years. All were in the final stage of heart failure. Three had biventricular disease, 6 had involvement of the right ventricle alone, and one had endomyocardial fibrosis confined to the left ventricle. There were 3 deaths (30%) in the postoperative period due to low cardiac output. The 7 survivors were followed up for a period ranging from 12 to 168 months, with a mean of 72 months. Two late deaths have occurred resulting from heart failure and infectious endocarditis. Five (50%) children are still alive. Two required 3 reoperations for dysfunction of the inserted valvar prosthesis. One patient is in functional Class IV, and 4 are in Class II to III, despite intensive medical treatment. It is concluded that surgery for endomyocardial fibrosis is an essentially palliative procedure and, especially in children, the results of surgical treatment leave much to be desired.     

Endomyocardial fibrosis. Problems in differential diagnosis.

The clinical and angiographic findings in 5 consecutive patients with congestive cardiac failure are presented to illustrate the pitfalls in the clinical diagnosis of endomyocardial fibrosis. In one patient the clinical diagnosis was confirmed at angiography while another patient who had angiographic evidence of early right ventricular endomyocardial fibrosis was diagnosed clinically as mitral stenosis. In 2 patients the clinical diagnosis was erroneous, there being no evidence of endomyocardial fibrosis on angiography. The fifth patient, who had angiographic evidence of idiopathic cardiomegaly, was diagnosed clinically as either idiopathic cardiomegaly or advanced left ventricular endomyocardial fibrosis. In tropical countries, where endomyocardial fibrosis, rheumatic heart disease, and idiopathic cardiomegaly are common, accurate clinical diagnosis of endomyocardial fibrosis is often difficult and angiographic studies are essential for confirmation.     

Endomyocardial fibrosis. Problems in differential diagnosis.

The clinical and angiographic findings in 5 consecutive patients with congestive cardiac failure are presented to illustrate the pitfalls in the clinical diagnosis of endomyocardial fibrosis. In one patient the clinical diagnosis was confirmed at angiography while another patient who had angiographic evidence of early right ventricular endomyocardial fibrosis was diagnosed clinically as mitral stenosis. In 2 patients the clinical diagnosis was erroneous, there being no evidence of endomyocardial fibrosis on angiography. The fifth patient, who had angiographic evidence of idiopathic cardiomegaly, was diagnosed clinically as either idiopathic cardiomegaly or advanced left ventricular endomyocardial fibrosis. In tropical countries, where endomyocardial fibrosis, rheumatic heart disease, and idiopathic cardiomegaly are common, accurate clinical diagnosis of endomyocardial fibrosis is often difficult and angiographic studies are essential for confirmation.     

Endomyocardial fibrosis in infancy

The patient was a 4-month-old infant, who underwent persistent ductus arteriosus interruption with titanium clips at the age of 13 days and, since the age of 2 months, had crises of hypoxia and hypertonicity. After clinical investigation, the presence of pulmonary hypertension was confirmed and left ventricular inflow tract obstruction was suspected. The patient underwent surgical treatment at the age of 4 months, during which right and left ventricular endocardial fibrosis was identified. The fibrosis was resected, but the infant had an unfavorable clinical evolution with significant diastolic restriction and died on the sixth postoperative day. Anatomicopathological and surgical findings suggested endomyocardial fibrosis, although that pathology is very rare at the patient's age.     

Endomyocardial fibrosis associated with daunorubicin therapy.

A case of endomyocardial fibrosis in a patient with acute myeloblastic leukaemia treated by daunorubicin is reported. The pathological findings are indistinguishable from tropical endomyocardial fibrosis.     

Endomyocardial fibrosis associated with daunorubicin therapy.

A case of endomyocardial fibrosis in a patient with acute myeloblastic leukaemia treated by daunorubicin is reported. The pathological findings are indistinguishable from tropical endomyocardial fibrosis.