Abstract

The nucleotide-oligomerization domain (NOD)-like receptor subfamily C3 (NLRC3) is a newly discovered and incompletely characterized member of the NLR family which negatively regulates inflammatory responses. Inflammation is considered a critical pathogenesis in pulmonary hypertension (PH). This is the first study to hypothesize that NLRC3 is closely correlated with PH. Total of 43 PH patients who were diagnosed by right heart catheterization (RHC) and 20 age-matched healthy control subjects were included. Echocardiographic variables and blood biochemical parameters were tested. Results of World Health Organization functional class (WHOFC), Borg dyspnea score and 6-minute walk tests (6MWT) were recorded. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR) were measured from RHC. Serum NLRC3 concentrations were detected by ELISA. ROC curve analysis was used to evaluate the diagnostic value of NLRC3 concentrations in PH. We found that serum NLRC3 concentration was significantly decreased in PH compared to the healthy control group. Serum NLRC3 concentration correlated negatively with mPAP and PVR. In addition, a negative correlation between serum NLRC3 concentration and WHOFC were detected. We proposed a cut-off value of 2.897ng/mL for serum NLRC3 concentration which was able to predict PH with 88% sensitivity and 85% specificity. In conclusion, NLRC3 concentrations in PH were significantly decreased, suggesting that NLRC3 may potentially be a diagnosis index and represent a prognostic factor for PH patients.

Highlights

  • Pulmonary hypertension (PH) is a progressive disease which leads to elevation of pulmonary arterial pressure, pulmonary vascular resistance and right ventricular failure

  • We examined the serum NLRC3 concentration and investigated whether non-invasive serum NLRC3 measurement was closely correlated with other indexes such as hemodynamic parameters acquired by right heart catheterization (RHC) in PH patients

  • No significant differences were found between the PH patients and controls in age, gender, body mass index (BMI), or cholesterol status

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Summary

Introduction

Pulmonary hypertension (PH) is a progressive disease which leads to elevation of pulmonary arterial pressure, pulmonary vascular resistance and right ventricular failure. Considering the high mortality and disability rates, providing timely diagnosis and accurate treatment are essential for improving the prognosis for PH patients. RHC is an invasive treatment and is well-known as the gold standard for PH diagnosis [3, 4]. It can accurately measure systolic/diastolic/mean pulmonary artery pressure (PAP), pulmonary capillary wedge pressure (PCWP), cardiac output (CO) and other. BDS is often identified as part of the 6MWT in PH and often supposed as a secondary endpoint in clinical trials [15] Many factors such as gait speed, age, weight and even encouragement from others can influence 6MWT and BDS values and lead to large variations [16]

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