Clinical characteristics and survival of systemic sclerosis patients with pulmonary hypertension and elevated wedge pressure: Observations from the PHAROS cohort.

ABSTRACT:

Background and objective

Systemic sclerosis (SSc) is a complex autoimmune disease commonly associated with pulmonary hypertension (PH). When associated with elevated pulmonary artery wedge pressure (PAWP), pulmonary artery pressure (PAP) is either in-proportion (post-capillary PH) or higher than expected (combined PH) relative to the increased PAWP.

Methods

Patients from the PHAROS registry (a prospective observational cohort of SSc-PH patients) who had mean PAP ≥ 25 and PAWP > 15 on right heart catheterization were stratified based on diastolic pressure gradient (DPG). Kaplan-Meier analysis was performed to compare survival and PH-related hospitalization. Baseline factors were compared between patients dying and those who survived using Cox regression analysis.

Results</

SUBMITTER: Lammi MR 

PROVIDER: S-EPMC5597455 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature