The heart in scleroderma

Abstract “Scleroderma heart disease” is a pathologic rather than a clinical diagnosis. This condition, in its advanced stage, is characterized by widespread proliferation of connective tissue and degeneration of myocardial fibers, but by itself rarely produces significant clinical manifestations. Indeed, when criteria for diagnosis require changes throughout multiple histologic sections, the incidence of “scleroderma heart disease” is low even at necropsy. Only 3 of our 25 necropsy cases of scleroderma met these criteria. Pulmonary and systemic vascular abnormalities played a more important role in disturbing cardiac function. Thus, right ventricular hypertrophy was caused by pulmonary hypertension found alone or in association with pulmonary fibrosis. Left ventricular hypertrophy was caused by malignant systemic hypertension produced by “scleroderma kidney.” The preceding extracardiac factors occurred in all 3 of our cases of “scleroderma heart disease” and caused the major clinical symptomatology. The most frequent site of cardiac involvement in cases of scleroderma is the pericardium. Pathologically, the commonest lesion is a chronic adhesive pericarditis (36%, of our cases) which does not restrict cardiac filling. Acute pericarditis during the clinial course of the disease is far less frequent than the pathologic lesions would indicate. Large pericardial effusions associated with a relatively normal pericardium histologically may be seen clinically. Minor deformities of the mitral and tricuspid valves occur uncommonly in scleroderma; valvular lesions of hemodynamic significance are due to coexistent rheumatic lesions. Dyspnea, particularly upon exertion, occurred during the course of the disease in 80 per cent of our cases. It can be generally ascribed to pulmonary hypertension, pulmonary fibrosis, or left heart failure associated with “scleroderma kidney.” It therefore is rarely necessary to invoke “scleroderma heart disease” as a cause for dyspnea. Right ventricular hypertrophy associated with pulmonary hypertension can be suspected clinically by the loud intensity of the pulmonic component of the second heart sound at Erb's point or by its transmission to the apex and by the presence of a presystolic gallop. There is also a good correlation between the hemodynamic severity of the pulmonary hypertension and the incidence of the electrocardiographic pattern of right ventricular hypertrophy and the roentgenographic analysis of the prominence of the pulmonary artery sector. From a physiologic and pathologic standpoint, the pulmonary hypertension and pulmonary fibrosis appear to be two independent phenomena. It is postuated that pulmonary hypertension in scleroderma is another expression of the heightened vasomotor tone in the disease, namely, Raynaud's phenomenon and “scleroderma kidney.” Although electrocardiograms and ballistocardiograms are often abnormal in scleroderma, the findings are generally of a nonspecific nature. In only 1 of our 3 cases of “scleroderma heart disease” did the electrocardiogram show an unusual pattern—that of apical infarction. In the 2 remaining cases the pattern of right ventricular hypertrophy dominated in 1 as a result of coexisting pulmonary hypertension, and left ventricular hypertrophy as a result of coexisting systemic hypertension in the other. In conclusion , “scleroderma heart disease” is an uncommon clinicopathologic entity. Nevertheless, abnormalities of cardiac function in scleroderma often are of major clinical significance because myocardial function is affected secondarily by (1) pulmonary hypertension, (2) systemic hypertension associated with “scleroerma kidney,” (3) pericarditis and pericardial effusion and (4) hypoxia associated with restrictive pulmonary disease.