Abstract
Background: Connective tissue growth factor (CTGF) has diagnostic value for pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) in children; however, its value in adult patients remains unclear. This study evaluated CTGF as a biomarker in adult PAH-CHD patients.
Methods and Results: Based on mean pulmonary artery pressure (mPAP), 56 CHD patients were divided into 3 groups: without PAH (W; mPAP <25 mmHg; n=28); mild PAH (M; mPAP 25–35 mmHg; n=18); and moderate and severe PAH (H; mPAP ≥35 mmHg; n=10). The control group consisted of 28 healthy adults. Plasma CTGF and B-type natriuretic peptide (BNP) concentrations were determined. Plasma CTGF concentrations were higher in the H and M groups than in the W and control groups, and were higher in the H than M group. Plasma CTGF concentrations were positively correlated with pulmonary artery systolic pressure (PASP), mPAP, and pulmonary vascular resistance, and negatively correlated with mixed venous oxygen saturation. CTGF, BNP, red blood cell distribution width, and World Health Organization Class III/IV were risk factors for PAH in CHD patients, and CTGF was an independent risk factor for PAH-CHD. The efficacy of CTGF in the diagnosis of PAH was not inferior to that of BNP.
Conclusions: CTGF is a biomarker of PAH associated with CHD. It can be used for early diagnosis and severity assessment in adult patients with CHD-PAH.
