The echocardiogram in scleroderma endocarditis of the mitral valve

Echocardiography was performed in a woman with long-standing scleroderma with the CRST syndrome in whom the murmur of mitral regurgitation had been present for one year. The echocardiogram showed an irregular shaggy thickening of the anterior mitral valve leaflet. At autopsy three months later, myocardial fibrosis, small coronary artery sclerosis, and nodular thickening of the mitral valve were found. To our knowledge, anterior mitral leaflet thickening in scleroderma has not been reported previously. In this patient, it was associated with a poor prognosis.