The Research Of L-arg-NO Path In Patients With Pulmonary Arterial Hypertension Related Congenital Heart Disease

ObjectiveDetect several important substances such as the serum levels of nitric oxide (NO), nitric oxide synthase (NOS), L-arginineacid (L-arg) and L-citrulline (L-cit) through L-arg-NO pathway in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH), research the relationship between congenital heart disease and pulmonary arterial hypertension (CHD-PAH), to reveal the pathogenesis of CHA-PAH and discuss the feasibility of amino acid supplement to treat CHD-PAH.Methods80patients with CHD were Selected, according to pulmonary artery pressure (mPAP) measured by the right heart catheterization, divided into four groups:group A, Unconsolidated PAH (mPAP≤25mmHg),20cases; group B, combined with mild PAH(25mmHg<mPAP≤35mmHg),21cases; group C, combined with moderate PAH (35mmHg<mPAP of≤45mmHg),14cases; group D, combined with severe PAH(mPAP>45mm Hg),25cases. Pulmonary blood in patients during right heart catheterization was centrifugal and refrigerated, then the serum levels of NO, NOS L-arg and L-cit were assessed through enzyme-linked immunosorbent assay (ELISA). Result1) The serum levels of NO, NOS, L-arg and L-cit decreased with the pulmonary hypertension increased, which in group A were significantly higher than these in group B, C, and D, with statistically significant (P <0.05). The serum levels of NO, NOS, L-arg and L-cit in group B was significantly higher than C and group D, with statistically significant (P <0.05), and group D reduced more obvious, but these in group C and D were not statistically significant (P=0.523, P=0.392, P=0.268).2) The levels of NO, NOS, L-arg and L-cit had a negative correlation with MPAP degree (P<0.05).Conclusion1) The serum levels of NO, NOS, L-arg and L-cit decreased with the pulmonary hypertension increased.We think the important substances in L-arg-NO pathway may involve in the formation and development of PAH, and these levels can reflect PAH severity to a certain extent, and be used as clinical indicators to monitor the PAH degree.2) The levels of NO, NOS, L-arg and L-cit had a negative correlation with MPAP degree, We think PAH lead to the impairment for L-arg-of NO pathway.