Endocardial Fibroelastosis

Abstract

During the 15-year period 1948–62 433 patients with congenital heart disease died or were stillborn and were examined after death by the regional pathology service. These represented 5% of all paediatric necropsies. Thirty-seven per cent, died within the first week of life and 54% within the first month. Seventy-two (17%) cases of congenital heart disease were found to have endocardial fibroelastosis. Sixteen of these cases were uncomplicated by any other congenital cardiac anomaly and 56 had associated congenital cardiac lesions. Of patients with uncomplicated endocardial fibroelastosis 31% died within three months, 75% within a year; of complicated cases 64% were fatal within three months and 95% within e vear. Endocardial fibroelastosis was present in the left side of the heart in 89% of the cases and in the right side in 38%. The ventricles were involved more often than the atria. Enlargement of the chamber in which endocardial fibroelastosis occurred was a frequent occurrence; so too was abnormal smallness of the left ventricle when endocardial fibroelastosis occurred in itai along with aortic stenosis or atresia. There was a significantly increased association between endocardial fibroelastosis and a small left ventricle, aortic stenosis and atresia, coarctation and hypoplasia oI the aorta, mitral stenosis, and patent ductus arteriosus. There was a diminished association between endocardial fibroelastosis and a number of congenital cardiac anomalies where the common factor appeared to be that they caused or were associated with intracardiac shunts. Evidence is put forward that endocardial fibroelastosis tends to be a secondary condition commonly associated with certain cardiac lesions. Obstructive lesions of the aortic valve and aorta particularly favour its occurrence. Stagnation of blood in a chamber and local trauma to the endocardium caused by abnormal jets of blood also favour its occurrence and increased blood-flow helps to prevent it. Hereditary and familial factors appear to be involved in its transmission. The prominent symptoms and signs were those of respiratory embarrassment and cardiac failure. Cardiac failure developed in 45% of cases. In 70% of cases the onset of symptoms occurred in the first six weeks of life, the average time of survival between onset of symptoms and death in these cases being 10 weeks. In 25%, onset of symptoms occurred between 6 weeks and 6 months, the average survival time being 13 weeks. With onset of symptoms after 6 months (5% of cases) the average survival time was 15 months. Treatment had some effect in prolonging the course of the disease. onar radiological examination 93 % of cases showed cardiomegaly. On electrocardiographic examination 40% of cases showed right atrial enlargement, 42% left atrial enlargement, 83% right ventricular enlargement, and 54% left ventricular enlargement. Certain electrocardiographic changes—naaely, enlargement of the P waves, RS-T depression, and T-wave inversion—are of some value in diagnosis. © 1964, British Medical Journal Publishing Group. All rights reserved.