THE HEART IN SCLERODERMA

Abstract

Involvement of the heart by scleroderma is comparatively rare. Only 28 reported patients have been found in whom adequate clinical description has been coupled with autopsy confirmation. In this series of 21 cases, evidence that the heart was affected by scleroderma was beyond doubt and confirmed by autopsy in 4. Heart failure in systemic scleroderma is more commonly due to chronic cor pulmonale than to direct cardiac involvement. Clinical evidence of cardiac involvement usually arises late in the disease, but may precede the recognition of scleroderma elsewhere in the body. The myocardium is most commonly affected, the pericardium next, and the valves and endocardium least frequently. The pericardial lesion is usually clinically silent. The earliest evidence of scleroderma heart is found in the ecg, which should not be omitted from the investigation of any case of systemic scleroderma. The cardiographic changes are numerous and not specific, but the diagnosis is suggested if the appearance resembles that of cardiac infarction in the absence of any history of cardiac pain. In any obscure cardiopathy, evidence of calcinosis should be sought, not only in the heart, lungs, and pleura, but elsewhere in the body in apparently unaffected soft tissues.