[Clinical value of ADMA in patients with pulmonary arterial hypertension combination with congenital heart disease].

To study the concentration of the asymmetrical dimethyl-L-arginine (ADMA) in patients with pulmonary arterial hypertension (PAH) commbination with congenital heart disease (CHD) and its clinical value as a biomaker for diagnosis and prognosis.
 Methods: A total of 100 patients with CHD and 25 healthy adult subjects were recruited. CHD patients were divided into three groups: normal pulmonary arterial pressure group (group A, n=25), mild-to-moderate PAH group (group B, n=25), severe PAH group (group C, n=50). Twenty patients in Group C were treated with sildenafil and followed up for 6 months. The clinical data, including echocardiographic measurements, hemodynamic parameters and ADMA levels, for all subjects were collected.
 Results: The ADMA concentrations in patients with CHD-PAH significantly increased compared with that in the CHD patients without PAH or the health controls, and the ADMA concentrations in CHD patients with severe PAH were significantly higher than that in the CHD patients with mild-to-moderate PAH; serum ADMA concentration was correlated with mean pulmonary arterial pressure (mPAP) (r=0.61, P<0.001) and pulmonary vascular resistance (PVR) (r=0.417, P<0.001) in CHD patients; when using AMDA>0.485 µmol/L as criteria for diagnosis of CHD-PAH, the specificity was 82.7% and the sensitivity was 92.0%; the pulmonary arterial pressure significantly decreased after sildenafil therapy for 6 months, same as the ADMA levels.
 Conclusion: Plasma ADMA could be used as a biomarker to identify PAH in patients with CHD and as a prognosic index to reflect the sildenafil treatment effect.