[Anatomo-clinical forms of left endomyocardial fibrosis. Study of 22 surgically treated cases]

The authors studied 22 patients with operated left sided endomyocardial fibrosis (EMF). Twenty patients had pure left sided and 2 bilateral (mainly right sided) EMF. The mean age of the patients was 14 years (range 7-51 years). Three clinico-pathological forms of the disease were identified: obliterating, diffuse and limited. The mechanism of associated mitral regurgitation was papillary muscle and/or posterior leaflet involvement. The anterior mitral leaflet was not affected in any of these cases. Echocardiographic diagnosis was possible in 18 cases by M mode recording showing M-shaped septal wall motion. The diagnosis was made in 5 out of 13 patients by 2D echocardiography. This method was satisfactory in the obliterating form of the disease but direct visualisation of the fibrosis in the diffuse and limited forms was very difficult. Catheter studies showed severe pulmonary hypertension in all cases. The presence of a dip-plateau pressure recording was inconstant and was only observed in the obliterating and diffuse forms of EMF. Angiocardiography was characteristic in the obliterating and diffuse forms and enabled an accurate anatomical diagnosis before surgery. The diagnosis of the limited form was difficult and could only be suspected in cases of mitral regurgitation associated with right ventricular EMF or in the presence of parietal recesses. This surgical series does not include cases of EMF limited to the ventricular apex without mitral regurgitation or adiastole, treated medically.