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Elevation of plasma cell-free hemoglobin in pulmonary arterial hypertension.

ABSTRACT: Cell-free hemoglobin (CFH) is a potent nitric oxide scavenger associated with poor outcomes in several diseases. Pulmonary arterial hypertension (PAH) is characterized by reduced nitric oxide availability. We hypothesized that CFH would be elevated in PAH and would associate with hemodynamics and clinical outcomes.We measured CFH in 200 consecutively evaluated patients with PAH, 16 unaffected bone morphogenetic receptor protein type 2 (BMPR2) mutation carriers, 19 healthy subjects, and 29 patients with pulmonary venous hypertension (PVH). CFH values were tested for association with hemodynamics, time to hospitalization, and death.CFH was elevated in patients with PAH and BMPR2 carriers compared with healthy subjects and patients with PVH (P ? .01 all comparisons). There were no differences in CFH across PAH subtypes. CFH modestly correlated with mean pulmonary artery pressure (? = 0.16, P = .03) and pulmonary vascular resistance (? = 0.21, P = .01) and inversely with cardiac index (? = -0.18, P = .02) in patients with PAH. CFH was not associated with hemodynamic response to nitric oxide or death. Patients with the highest CFH levels had increased risk of PAH-related hospitalization when adjusted for age, sex, and PAH cause (hazard ratio, 1.69; 95% CI ,1.08-2.66; P = .02).CFH is elevated in patients with PAH and BMPR2 carriers compared with healthy subjects and patients with PVH. Elevated CFH levels are independently associated with an increased risk of hospitalization. Further study is required to understand the mechanism of CFH elevation and the potential pathologic contribution of CFH in PAH.

SUBMITTER: Brittain EL

PROVIDER: S-EPMC4251612 | biostudies-other | 2014 Dec

REPOSITORIES: biostudies-other

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Elevation of plasma cell-free hemoglobin in pulmonary arterial hypertension.

Brittain Evan L EL Janz David R DR Austin Eric D ED Bastarache Julie A JA Wheeler Lisa A LA Ware Lorraine B LB Hemnes Anna R AR

Chest 20141201 6

<h4>Background</h4>Cell-free hemoglobin (CFH) is a potent nitric oxide scavenger associated with poor outcomes in several diseases. Pulmonary arterial hypertension (PAH) is characterized by reduced nitric oxide availability. We hypothesized that CFH would be elevated in PAH and would associate with hemodynamics and clinical outcomes.<h4>Methods</h4>We measured CFH in 200 consecutively evaluated patients with PAH, 16 unaffected bone morphogenetic receptor protein type 2 (BMPR2) mutation carriers, ...[more]

PMID: 24945582

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Project description:AimsRight ventricular dysfunction may arise because of pulmonary arterial hypertension (PAH). Development of new diagnostic methods able to identify PAH and allow for earlier treatment initiation, before the development of vascular remodelling and manifest right heart failure (HF), could potentially improve prognosis. Proteoglycans and inflammatory proteins are involved in vascular remodelling. We aimed to investigate their potential as biomarkers to differentiate PAH in a dyspnoeic population.Methods and resultsPlasma from 152 patients with PAH (n = 48), chronic thrombo-embolic pulmonary hypertension (n = 20), pulmonary hypertension due to HF with reduced (n = 36) or preserved (n = 33) ejection fraction, and HF without pulmonary hypertension (n = 15) and 20 healthy controls were analysed with proximity extension assays. Haemodynamics were assessed in the patients with right heart catheterization. Plasma prolargin levels in PAH were lower compared with all the other studied disease groups (P < 0.001) but higher than the controls' levels (P = 0.003). Receiver operating characteristic curve of prolargin as a PAH-differentiating marker in a pooled population, encompassing all the other studied disease groups, had a sensitivity of 74% and a specificity of 83.3% (area under the curve = 0.84, P < 0.001). Prolargin correlated with the mean right atrial pressure (rs = 0.65, P < 0.001), N-terminal pro-brain natriuretic peptide (rs = 0.64, P < 0.001), cardiac index (rs = -0.31, P = 0.029), stroke volume index (rs = -0.41, P = 0.004), right ventricular stroke work index (rs = -0.31, P = 0.032), six-minute walking distance (rs = -0.41, P = 0.005), and mixed venous blood oxygen saturation (rs = -0.42, P = 0.003).ConclusionsPlasma prolargin levels differentiate PAH patients from controls and the other investigated dyspnoea groups including HF. Its potential in PAH differentiation may be enhanced by inclusion in a multi-marker panel. Larger studies are needed to evaluate its discriminative ability of PAH in relation to other dyspnoea aetiologies and its potential role in PAH risk stratification and pathobiology.

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Elevation of plasma cell-free hemoglobin in pulmonary arterial hypertension.

Publications

Elevation of plasma cell-free hemoglobin in pulmonary arterial hypertension.

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