Abstract 17296: C-Terminal Pro Endothelin 1 Precursor Fragment is Elevated in Scleroderma-Associated Pulmonary Arterial Hypertension: Correlation with N-Terminal Pro Brain Natiruretic Peptide
Purpose: Pulmonary arterial hypertension (PAH) is a major cause of death in patients (pts) with scleroderma (SSc). Biomarkers play an increasing role in the non-invasive workup of SSc patients, regarding initial diagnosis of PAH as well as follow-up evaluations. Natriuretic peptides, especially Brain Natriuretic Peptide (BNP) and its N-terminal fragment (NTproBNP) are established biomarkers which correlate with severity of PAH. Endothelin1 (ET1) is involved in the pathobiology of PAH. We hypothesized that plasma levels of the ET1 precursor peptide, C-terminal proEndothelin-1 (CT-proET-1) are increased in SSc-associated PAH.
Methods: We prospectively recruited 40 consecutive pts with SSc, 19 without suspicion or invasive exclusion of PAH, and 21 with diagnosed PAH from our local Dermatology Clinic. NTproBNP and CT-proET-1 plasma levels were measured in each patient. Hemodynamic measurements were performed in 27 pts (all PAH pts and 6 SSc pts without PAH).
Results: CT-proET-1 levels significantly correlated with NTproBNP levels (r=0.65, p<0.001). Both CT-proET-1 and NTproBNP levels differed significantly between SSc pts with and without PAH (p<0.001 for both parameters). Neither CT-proET-1 nor NTproBNP correlated significantly with cardiac output and pulmonary vascular resistance. Both parameters significantly correlated with right atrial pressure (p<0.05 each). Only CT-proET-1 levels significantly correlated with mean pulmonary arterial pressure (r=0.50, p=0.008) and transpulmonary gradient (r=0.41, p=0.03), whereas NTproBNP levels did not (r<0.1, p>0.5 for both hemodynamic parameters, respectively).
Conclusions: CT-proET-1 levels differ significantly in SSc pts with and without PAH, and correlate with important hemodynamic parameters. CT-proET-1 levels may provide important additional information to NTproBNP levels in the diagnostic workup and risk assessment in SSc pts.
- © 2010 by American Heart Association, Inc.