Objective To investigate the expression of serum connective tissue growth factor(CTGF) in congenital heart disease(CHD) with pulmonary artery hypertension(PAH) and its significance. Methods Fifty children with CHD in the Affiliated Hospital of Luzhou Medical College from May 2009 to Feb.2012 were selected in the study.Pulmonary artery systolic pressure(PASP) were measured by right heart catheterization.They were divided into 3 groups according to PASP levels as follows: normal PAH group(PASP<3.99 kPa, n=21), mild PAH group(3.99 kPa≤PASP<6.65 kPa, n=17), moderate or severe PAH group(PASP≥6.65 kPa, n=12); serum CTGF and transforming growth factor-β1(TGF-β1) were detected by using double-antibody sandwich enzyme linked immunosorbent assay.The relationship among the 3 groups and their PASP were analyzed. Results In comparison to normal PAH group, serum CTGF and TGF-β1 were significantly elevated in mild PAH group and moderate or severe PAH group(all P<0.01); compared with mild PAH group, serum CTGF and TGF-β1 were higher in moderate or severe PAH group(all P<0.01); CTGF levels were positive correlated with PASP both in mild PAH group and moderate or severe PAH group(r=0.828, 0.849, all P<0.01); there were positive correlations between CTGF and TGF-β1 level in mild PAH group and moderate or severe PAH group(r=0.714, 0.713, all P<0.01); there were positive correlations between TGF-β1 and PASP in mild PAH group and moderate or severe PAH group(r=0.623, 0.639, all P<0.05). Conclusions Serum CTGF levels are elevated in children with CHD and PAH, which may be an important factor for the formation and development of CHD with PAH. Key words: Connective tissue growth factor; Heart defect, congenital; Transforming growth factor-β; Hypertension, pulmonary
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