Cardiopulmonary changes in scleroderma A physiologic study

Abstract

Four patients with scleroderma were studied by means of cardiac catheterization and measurements of pulmonary function. In one case the studies were repeated after versene therapy and subjective clinical improvement. In 2 cases, a single-breath carbon-monoxide test was performed. In 2 cases, marked pulmonary hypertension was found, and in all 4 cases, reduction in the cardiac output and index was demonstrated. All patients exhibited evidence of restrictive ventilatory disturbance, but only one showed evidence of an obstructive type of pulmonary ventilatory defect. All patients exhibited a large intrapulmonary venoarterial shunt. In the 2 patients in whom the single-breath carbon-monoxide technique was performed a diffusion defect was also observed. The large A-a PO 2 gradient described in these patients when they were breathing room air was the result of the combination of a diffusion defect and pulmonary venoarterial shunts. Versene therapy resulted in marked subjective improvement, but failed to improve the pulmonary ventilation or to diminish the intrapulmonary shunt.