Role of plasma connective tissue growth factor levels in assessment of pulmonary arterial hypertension in Children with congenital heart disease

Abstract

We aimed to investigate plasma connective tissue growth factor (CTGF) levels in pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH–CHD) in children and the relationships of CTGF with hemodynamic parameters. Plasma CTGF levels were calculated in 30 children with PAH–CHD and 30 health volunteers, using the subtraction method. Cardiac catheterization was performed to measure clinical hemodynamic parameters. Plasma CTGF levels were significantly higher in PAH–CHD than in health volunteers (p < 0.01). In cyanotic PAH–CHD, plasma CTGF levels were significantly elevated compared with acyanotic PAH–CHD in the same group (p < 0.05). oxygen saturation was inversely related to plasma CTGF levels (r = −0.436, p < 0.05). it revealed a moderately better specificity, positive predictive value and positive likelihood ratio. Right heart catheterization (RHC) is the gold standard method for diagnosis of PAH and an important tool for testing the eligibility of patients with CHD to undergo operations for correction of heart defects. However, it is an invasive test and not always practical for repeated ongoing evaluation.So, accurate noninvasive assessment of PAH is desirable both for diagnostic purposes and to assess response to therapy.