Cystatin C: A potential biomarker for pulmonary arterial hypertension

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Background and Objective:

Cystatin C (CysC), a novel marker of renal function, predicts left heart failure and cardiovascular mortality. The hypothesis that serum CysC levels correlate with right ventricular (RV) morphology, function and pressure in pulmonary arterial hypertension (PAH) was tested.


As part of a prospective study, 14 PAH subjects and 10 matched controls underwent same-day echocardiography, cardiac magnetic resonance imaging (CMR), and phlebotomy for CysC, brain natriuretic peptide (BNP), and N-terminal BNP (NT-ProBNP). RV ejection fraction (RVEF), end-diastolic volume, end-systolic volume and mass were calculated using CMR. RV systolic pressure (RVSP), strain and diastolic function (including tricuspid valve (TV) E velocity, A velocity, e′ velocity, E/A ratio and E/e′ ratio) were assessed using echocardiography.


RVSP was significantly elevated in PAH subjects versus controls (57 ± 17 vs. 28 ± 8 mm Hg,P< 0.0001). CysC was abnormally elevated in the PAH cohort when compared with controls (1.00 ± 0.23 vs 0.78 ± 0.05 mg/L,P= 0.001). CysC positively correlated with RVSP (rho 0.61,P= 0.002), RV end-diastolic volume (rho 0.50,P= 0.01), RV end-systolic volume (rho 0.58,P= 0.003), mass index (rho 0.66,P= 0.0004), strain (rho 0.51,P= 0.01) and strain rate (rho 0.51,P= 0.01) and negatively correlated with RVEF (rho −0.58,P= 0.003) and TV e′ (rho −0.75,P< 0.0001). The same correlations with BNP and NT-ProBNP were comparable with CysC.


In a small cohort, CysC accurately correlates with RV pressure, function and morphology. CysC may represent a novel PAH biomarker.


We examined the ability of cystatin C (CysC) to predict the presence of pulmonary arterial hypertension (PAH) and found that CysC strongly correlates with right ventricular systolic pressure, function and morphology. Furthermore, CysC performed comparably with the existing biomarkers brain natriuretic peptide (BNP) and N-terminal BNP.

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