Portal de Periódicos da CAPES

DIFFUSE ENDOMYOCARDIAL SCLEROSIS

1957; BMJ; Volume: 19; Issue: 2 Linguagem: Inglês

10.1136/hrt.19.2.173

1468-201X

J. B. LYNCH, J Watt,

Viral Infections and Immunology Research

In recent years increasing attention has been focused upon a group of cases of congestive cardiac failure that present a distinctive cardiac morphology.There is diffuse thickening of the mural endocardium of one or both ventricles and fibrosis of the myocardium which is largely confined to the inner third of the ventricular wall; the coronary arteries are normal or show minimal arteriosclerosis.Since little is known of the pathogenesis, and the morphology of the lesion is somewhat variable, we have used the general term " diffuse endomyocardial sclerosis ".From Europe, particularly Switzerland, there have been several reports of endomyocardial sclerosis, but usually of single cases only.Loeffler (1936) described two under the term " endo- carditis parietalis fibroplastica with -eosinophilia ", and similar single case reports are those of Mumme (1940), Roulet (1944), Berblinger (1948), Lenegre and Gerbaux (1952) and others.Prior to 1948, only 22 cases had been recorded (Loeffler, 1946-47).Similarly, from America there have been very few examples described (Smith and Furth, 1943; McKusick and Cochran, 1952; McNichol et al., 1953; and others).We have found only two relevant British case reports (Edge, 1946; and Lennox, 1948).A closely similar condition occurring in Africa is endomyocardial fibrosis: several detailed clinical and pathological studies on a large number of these cases have been made and Davies and Ball (1955) note that it was responsible for 33 of the 231 deaths in Uganda from cardiac failure in the period 1951-53.This curious anomaly of the geographical incidence of the condition remains unexplained.A lesion morphologically allied to endomyocardial sclerosis occurs in infants, and has been described as fcetal endomyocarditis (Farber and Hubbard, 1933), endocardial dysplasia (Prior and Wyatt, 1950), and congenital fibroelastosis (Gowing, 1953).The relationship of this condition to that in the adult remains sub judice at present.We have collected four adult cases of diffuse endomyocardial sclerosis during the past four years and it is possible that the condition in this country, though rare, may escape notice.Material from cases of endomyocardial sclerosis occurring in African natives and from cases of congenital fibroelastosis in infancy has been studied for comparison.A composite clinical and pathological picture of diffuse endomyocardial sclerosis will be given and only a brief resume of each case is presented.CASE SUMMARIES Case 1.A Jewess, aged 51 years, suffered from recurrent attacks of asthma sometimes with pr=cordial pain, for six months before admission to hospital.On examination the essential findings were diffuse bronchial spasm, a mild degree of hypertension (B.P. 170/100), and eosinophilia.The total white cell count varied from 8000 to 18,000 per c.mm., the eosinophilia fluctuating within the range 4 to 31 per cent.Her death was sudden and unexpected and was presumed to be the result of acute coronary ischlemia.Post mortem there was a diffuse vesicular emphysema of the lungs and a moderate degree of chronic venous congestion of the abdominal viscera.The heart was enlarged, weighing 400 g.In the left ventricle there was an extensive endomural thrombus overlying a grossly thickened endocardium (Fig. 1).On histological examination the thickened endocardium consisted of granulation tissue showing numerous capillaries and a diffuse cellular infiltration comprising eosinophil leucocytes, plasma cells, lymphocytes,