SIGNAL-AVERAGED ELECTROCARDIOGRAPHY AND ECHOCARDIOGRAPHY IN THE EVALUATION OF MYOCARDIAL INVOLVEMENT IN PROGRESSIVE SYSTEMIC-SCLEROSIS

To assess the myocardial involvement in progressive systemic sclerosis we evaluated the presence of late potentials by signal-averaged elect rocardiography (signal-averaged EGG) and the left ventricular function by M-mode, two dimensional and Doppler echocardiography. Fifteen outp atients, 7 with diffuse progressive systemic sclerosis and 8 with CRES T syndrome variant, without clinical or electrocardiographic evidence of cardiac disease were studied and compared with 18 normal subjects. Late potentials occurred in 5 out of 15 progressive systemic sclerosis patients (33%) with a significant difference versus controls (P < 0.0 5) and were present only in the patients with diffuse progressive syst emic sclerosis (P less than or equal to 0.001 vs. controls). All progr essive systemic sclerosis patients showed a normal left ventricular sy stolic function, Abnormal left ventricular filling was found in 9 prog ressive systemic sclerosis patients (5 with diffuse progressive system ic sclerosis and 4 with CREST). A more severe impairment of the mean v alues of diastolic function indexes was found in diffuse progressive s ystemic sclerosis than in CREST. In all diffuse progressive systemic s clerosis patients at least one method showed altered results, whereas half the CREST patients showed no pathological findings with both tech niques. These results confirm a lower myocardial involvement in the CR EST syndrome than in diffuse progressive systemic sclerosis and conseq uently this is probably related to a better prognosis.