Cardiopulmonary exercise testing with pulmonary artery catheterization detects early pulmonary vasculature changes in patients with connective tissue diseases.

Pulmonary hypertension is a well-recognized complication of connective tissue diseases (CTD), with clinical symptoms typically limited to those with relatively advanced disease. The objective of this study was to identify patients with early pulmonary vasculature changes using cardiopulmonary exercise testing with pulmonary artery catheterization. Four patients with CTD and normal Doppler echocardiogram results identified as high risk for pulmonary hypertension on the basis of disease process underwent cardiopulmonary exercise testing with pulmonary artery catheterization. All patients had normal mean pulmonary artery pressures at rest (<25 mmHg), but exhibited an abnormal rise in mean pulmonary artery pressure with exercise. Cardiopulmonary exercise testing should be considered in patients at high risk for pulmonary hypertension to identify those individuals who may benefit from early therapeutic intervention.