Abstract
Pulmonary hypertension (PH) with exercise may be a precursor of resting PAH in SSc. Recent observations have demonstrated that both PVR and Cpa originate predominantly in the distal pulmonary vasculature and have a highly consistent inverse hyperbolic relationship. We have shown that an increase in wedge pressure (PAWP) reduces Cpa for any given PVR. We sought to characterize the clinical and hemodyanamic features of EI-PAH in SSc.
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