Endocardial fibro-elastosis in adolescents and adults.

Endocardial fibro-elastosis is no rare finding in infants and young children up to two years of age, and as a rule it leads rapidly to death. Panke and Rottino (1955) found that of 129 cases described 78 per cent had died below the age of twelve months. Its frequency is much less in older children and adults (Smith and Furth, 1943; Hughes and Smith, 1953; White and Fennell, 1954; Thomas et al., 1954; Panke and Rottino, 1955; Popper et al., 1956; and Guraieb and Rigdon, 1956). We have observed five older patients with this disease, three of whom showed the typical clinical picture, while the other two showed features only rarely mentioned in earlier publications. In endocardial fibro-elastosis the endocardium of one or both ventricles and sometimes also of the atrium is greatly thickened. Usually the left ventricle and left atrium are involved. The endocardium has a greyish-white appearance; it is opaque and reminds one of porcelain. The valves may be somewhat thickened, but generally their function is good. The heart is hypertrophic and its weight is twice or thrice the normal. Mural thrombi have often been found on the abnormal endocardium, and they may give rise to emboli. Microscopically, apart from a connective-tissuelike thickening of the endocardium, an increase of the elastic fibres is observed. As a rule there is a sharp demarcation between the thickened endocardium and the underlying myocardium. Sometimes a few fibrotic foci may be found in the subendocardial myocardium. There are no signs of inflammation or fibrinoid necrosis.