Hemodynamic and angiographic aspects of endomyocardial fibrosis. Apropos of 19 cases

1980 
: 19 cases of endomyocardial fibrosis were studied. Angiocardiography localises the site of fibrosis and seems to be the best diagnostic method. All cases in this series had left ventricular involvement which resulted in changes of the silhouette (square, polylobulated or deformed like the shape of a heart on a playing card) and of the ventricular contour (smooth, lacunar or "doubled"). Ten patients had mitral incompetence. The ejection fraction was normal in 8 patients but significantly reduced in the series as a whole (EF = 0,56, p less than 0,05). 15 patients had right ventricular involvement. Apart from the smooth contour of the anterior wall, the only abnormality in the mild cases, the most suggestive feature was an amputation of the ventricular apex, giving rise to a ventricular appearance of a narrow, akinetic (apart from the infundibular region) tube. The catheter data demonstrated the haemodynamic changes due to the fibrosis. A constrictive syndrome was observed in all the severe poorly tolerated cases. This was not apparent under basal conditions in milder cases. The value of pharmacodynamic testing and endomyocardial biopsy in cases where the diagnosis is uncertain should be stressed. The results of resection of the fibrosis and valvular replacement in severe cases depend to a large extent on the degree of myocardial involvement.
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