Elevated PAP in Systemic Sclerosis: What Is the Connection?

Individuals with systemic sclerosis who have mildly elevated pulmonary artery pressure are more likely to have lower pulmonary arterial compliance both at rest and at different stages of exercise than their counterparts with normal pulmonary artery pressure, according to a new study.

To make this determination, the researchers had 112 participants with systemic sclerosis undergo right heart catheterization both at rest and during exercise.

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Based on their resting mean pulmonary artery pressure (mPAP), the participants were divided into 3 groups: normal mPAP (≤20 mmHg), mildly elevated mPAP (21‐24 mmHg), and pulmonary hypertension (PH, mPAP ≥25 mmHg).

Compared with participants with normal mPAP, participants with mildly elevated mPAP had lower 6‐minute‐walking distance; lower cardiac index and higher pulmonary vascular resistance during exercise; and lower pulmonary arterial compliance at rest and different stages of exercise.

“The results of this study suggest that impaired 6‐minute‐walking distance systemic sclerosis patients with mildly elevated mPAP might be caused by reduced pulmonary arterial compliance during exercise and reduced RV output reserve, presumably due to impaired coupling between the right ventricle and the pulmonary vasculature,” the researchers concluded. “These findings give further evidence for the clinical relevance of mildly elevated mPAP in patients with systemic sclerosis.”

—Colleen Murphy

Reference:

Nagel C, Marra AM, Benjamin N, et al. Reduced right ventricular output reserve in patients with systemic sclerosis and mildly elevated pulmonary arterial pressures [published online January 7, 2019]. Arthritis Rheumatol. https://doi.org/10.1002/art.40814.