Allen S W, Chatfield B A, Koppenhafer S A, Schaffer M S, Wolfe R R, Abman S H
Department of Pediatrics, University of Colorado School of Medicine, Denver.
Am Rev Respir Dis. 1993 Aug;148(2):519-22. doi: 10.1164/ajrccm/148.2.519.
To determine whether circulating levels of endothelin-1 (ET-1), a potent vasoconstrictor peptide, are elevated in children with pulmonary hypertension and related to the degree of hypoxic pulmonary vasoconstriction, we measured arterial and mixed venous plasma concentrations of immunoreactive ET-1 (irET-1) in 13 children during cardiac catheterization. Clinical diagnoses in seven children with pulmonary hypertension (PH) included chronic lung disease (four children), congenital heart disease after surgical repair (two children), and primary ("reactive") pulmonary hypertension (one child). Blood samples were simultaneously obtained from pulmonary artery (venous) and systemic arterial sites during baseline conditions. Plasma irET-1 was elevated in children with PH (12.3 +/- 3.4 versus 3.6 +/- 0.7 pg/ml, PH versus non-PH; p < 0.01). Arterial/venous irET-1 ratios in the PH group (1.1 +/- 0.2) were not different from those in the non-PH group. During acute hypoxia, mean Ppa increased from 27 +/- 3 to 40 +/- 5 mm Hg. Basal irET-1 correlated strongly with the degree of elevation of mean Ppa during acute hypoxia (r = 0.69; p < 0.02). We conclude that irET-1 levels are often elevated in children with PH, and they are strongly correlated with pulmonary vasoreactivity during acute hypoxia. Whether elevated irET-1 levels contribute directly to or are markers of altered pulmonary vascular tone and reactivity in children with PH remains speculative.
为了确定强效血管收缩肽内皮素 -1（ET-1）的循环水平在患有肺动脉高压的儿童中是否升高，并与缺氧性肺血管收缩的程度相关，我们在13名儿童进行心导管检查期间测量了动脉和混合静脉血浆中免疫反应性ET-1（irET-1）的浓度。7名患有肺动脉高压（PH）的儿童的临床诊断包括慢性肺病（4名儿童）、手术修复后的先天性心脏病（2名儿童）和原发性（“反应性”）肺动脉高压（1名儿童）。在基线条件下，同时从肺动脉（静脉）和体动脉部位采集血样。PH儿童的血浆irET-1升高（12.3±3.4对3.6±0.7 pg/ml，PH对非PH；p<0.01）。PH组的动脉/静脉irET-1比值（1.1±0.2）与非PH组无差异。在急性缺氧期间，平均肺动脉压从27±3升高到40±5 mmHg。基础irET-1与急性缺氧期间平均肺动脉压的升高程度密切相关（r = 0.69；p<0.02）。我们得出结论，PH儿童的irET-1水平通常升高，并且它们与急性缺氧期间的肺血管反应性密切相关。irET-1水平升高是否直接导致PH儿童肺血管张力和反应性改变或作为其标志物仍有待推测。
