ECHOCARDIOGRAPHIC IDENTIFICATION OF CARDIAC ABNORMALITY IN SCLERODERMA AND RELATED DISORDERS

Although pathologic examination may readily disclose cardiac abnormality in patients with scleroderma, clinical identification of primary heart involvement can be difficult. In order to assess left ventricular systolic function, chamber size and wall thickness, and to determine whether pericardial effusion is present, echocardiograms were obtained in 11 patients with progressive systemic sclerosis (PSS) and in 13 patients with forms of scleroderma in which visceral involvement has been considered rare or absent: three with CREST syndrome, three with morphea, three with diffuse fasciitis with eosinophilia, and four with mixed connective tissue disease. Increased left ventricular wall thickness was noted in 13 of 23 (57 per cent) patients who could be evaluated, including six (46 per cent) from subgroups other than PSS. Left atrial dimension was increased in 12 patients (52 per cent) whereas the left ventricular end-diastolic dimension was increased in only three (13 per cent). Mitral valve closure velocity, an index of left ventricular compliance, was diminished in 10 (42 per cent) patients. However, left ventricular systolic contractile performance was normal in all. Pericardial effusion was detected in five patients (21 per cent), including one patient each with morphea, mixed connective tissue disease and diffuse fasciitis. Cardiac abnormalities were evident even in patients with PSS and no renal or severe pulmonary involvement. Thus, primary cardiac involvement, characterized by left ventricular wall thickening, decreased left ventricular compliance, left atrial enlargement and pericardial effusion, may be common in patients with scleroderma. These abnormalities occur in patients with PSS as well as in those with nonsystemic" forms of scleroderma and are readily detected by echocardiography. © 1979."