Refer for right heart catheterization for evaluation of pulmonary hypertension.    

Approximately 15% of patients with scleroderma develop clinically significant pulmonary hypertension, and pulmonary complications, including pulmonary hypertension, are the leading cause of death among that patient population. In patients with connective tissue diseases presenting with clinical findings suggestive of elevated right heart pressures and evidence of right heart failure, group 1 pulmonary hypertension should be ruled out.

The next best step would be to obtain a right heart catheterization with vasodilator challenge, especially before considering starting vasodilators. The goal of the right heart catheterization is to confirm the pulmonary pressures and differentiate between pulmonary arterial hypertension vs pulmonary venous hypertension due to left ventricular dysfunction. Both groups will have elevated pulmonary artery systolic and mean pressures; however, patients with pulmonary arterial hypertension will have pulmonary capillary wedge pressures that are near normal (<15 mm Hg) with elevated pulmonary arterial resistance (>3 wood units). Those patients with pulmonary hypertension due to left heart disease will have elevated pulmonary capillary wedge pressures with normal pulmonary resistance. 

Related Articles

Reference

Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004;351(16):1655-1665.

This article originally appeared on The Cardiology Advisor