Serum Pentraxin 3 and hs-CRP Levels in Children with Severe Pulmonary Hypertension

Abstract

Pulmonary arterial hypertension (PAH) is defined as a mean pulmonary arterial pressure of >25 mmHg (1). Although there have been developments in diagnostic tools such as echocardiography and treatment modalities, PAH secondary to undiagnosed or untreated congenital heart diseases, especially left-to-right shunt defects, remains an important cause of pulmonary arterial hypertension in childhood. The pathophysiology of PAH is multifactorial, complex and incompletely understood (2-4). PAH secondary left-to-right shunt defects is related to increased pulmonary blood flow leading to endothelial dysfunction, increased pulmonary vascular resistance, vascular remodelling and luminal obstruction due to in situ thrombosis and neointimal and plexiform lesions (5-7). Some recent studies have shown that inflammation has an important role in the pathophysiology of PAH (8). C-reactive protein (CRP) is a classic short pentraxin that is produced in liver secondary to systemic inflammation. Pentraxin 3 is one of the long pentraxins and is synthesized by local vascular cells, such as smooth muscle cells, endothelium and fibroblasts, as well as innate immune cells at sites of inflammation. Pentraxin 3 plays a key role in the regulation of cell proliferation and angiogenesis (9, 10).Background: Pulmonary arterial hypertension secondary to untreated left-to-right shunt defects leads to increased pulmonary blood flow, endothelial dysfunction, increased pulmonary vascular resistance, vascular remodelling, neointimal and plexiform lesions. Some recent studies have shown that inflammation has an important role in the pathophysiology of pulmonary arterial hypertension. Aims: The aim of this study is to evaluate serum pentraxin 3 and high sensitive (hs)-C reactive protein (hs-CRP) levels in children with severe pulmonary arterial hypertension (PAH) secondary to untreated congenital heart defects and evaluate the role of inflammation in pulmonary hypertension. Study Design: Cross sectional study. Methods: After ethics committee approval and receiving consent from parents, there were 31 children were selected for the study with severe PAH, mostly with a left-to-right shunt, who had been assessed by cardiac catheterisation and were taking specific pulmonary vasodilators. The control group consisted of 39 age and gender matched healthy children. After recording data about all the patients including age, gender, weight, haemodynamic studies and vasodilator testing, a physical examination was done for all subjects. Blood was taken from patients and the control group using peripheral veins to analyse serum Pentraxin 3, N-terminal pro-Brain Natriuretic Peptide (NT-ProBNP) and hs-CRP levels. Serum Pentraxin-3 levels were measured by enzyme linked immunosorbent assay (ELISA) and expressed as ng/mL. Serum hs-CRP levels were measured with an immunonephelometric method and expressed as mg/dL. The serum concentration of NT-proBNP was determined by a chemiluminescent immunumetric assay and expressed as pg/mL. Results: Serum Pentraxin-3 levels were determ...

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