不对称二甲基精氨酸在先天性心脏病相关性肺动脉高压中的临床价值

目的：研究血浆不对称二甲基精氨酸(asymmetrical dimethyl-L-arginine，ADMA)作为先天性心脏病(congenitalheart disease，CHD)相关肺动脉高压(pulmonary arterial hypertension，PAH)患者诊断、分层与随访指标的应用价值。方法：收集湘雅二医院心血管内科2013年10月至2014年10月经超声心动图证实的CHD患者(n=100)，将其分为3组：无PAH组、轻中度PAH组、重度PAH组；重度PAH组又分为动力型亚组、艾森曼格综合征前期亚组、艾森曼格综合征亚组；同时收集健康成人作为对照组(n=25)；对20例阻力型患者使用西地那非治疗后随访6个月。采集入选病例所有临床一般情况、心脏彩色超声检查和心导管检查结果，检测血浆ADMA浓度。结果：轻中度PAH组血浆ADMA水平显著高于无PAH组及对照组(均P<0.001)；重度PAH组血浆ADMA水平显著高于无PAH组及对照组(均P<0.001)；无PAH组血浆ADMA水平与对照组无统计学差异(P=0.209)；在重度PAH组中，艾森曼格综合征亚组患者血浆ADMA水平显著高于动力型亚组(P<0.001)；ADMA水平与平均肺动脉压(mPAP)，全肺阻力(PVR)呈显著正相关(r分别为0.61，0.417，P<0.001)；血浆ADMA浓度0.485 μmol/L时诊断CHD合并重度PAH敏感度为92.0%，特异性为82.7%(P<0.001)；20例阻力型PAH患者使用西地那非治疗6个月后，肺动脉压力得到改善(P=0.001)，血浆ADMA水平显著降低(P<0.01)。结论：血浆ADMA水平可作为早期识别CHD-PAH的无创性筛查指标，在一定程度上反映西地那非治疗效果，有望成为CHDPAH患者重要的随访指标。

Clinical value of ADMA in patients with pulmonary arterial#br#hypertension combination with congenital heart disease

Objective: To study the concentration of the asymmetrical dimethyl-L-arginine (ADMA) inpatients 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. CHDpatients 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). Twentypatients 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 allsubjects were collected.Results: The ADMA concentrations in patients with CHD-PAH significantly increased comparedwith that in the CHD patients without PAH or the health controls, and the ADMA concentrationsin CHD patients with severe PAH were significantly higher than that in the CHD patients withmild-to-moderate PAH; serum ADMA concentration was correlated with mean pulmonary arterialpressure (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, thespecificity was 82.7% and the sensitivity was 92.0%; the pulmonary arterial pressure significantlydecreased 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 CHDand as a prognosic index to reflect the sildenafil treatment effect.