Latus Heiner, Karanatsios Georg, Basan Ulrike, Salser Kirstin, Müller Simon, Khalil Markus, Kreuder Joachim, Schranz Dietmar, Apitz Christian
Pediatric Heart Center, Justus-Liebig-University Giessen, Giessen, Germany.
MUON-STAT Statistical Consulting, Stuttgart, Germany.
Heart. 2016 Jul 1;102(13):1052-8. doi: 10.1136/heartjnl-2015-308743. Epub 2016 Mar 8.
Pulmonary arterial hypertension is known to be associated with increased expression of endothelin (ET)-1 and its precursor big ET-1. Therefore, we hypothesised that in children with pulmonary hypertension (PH) altered levels of ET-1 and big ET-1 may have clinical and prognostic impact.
Sixty-six children with different forms of PH (mean age 10.4±9.7 years) were included. Blood samples were taken from the pulmonary artery and a systemic artery. Levels of ET-1/big ET-1 were measured via ELISA method and compared with clinical and haemodynamic data. To assess prognostic relevance, Kaplan-Meier survival analysis was conducted with definition of end point as the composite of mortality, lung transplantation, use of intravenous prostanoids and Potts shunt creation.
ET-1 levels ranged between 0.09 and 11.64 (mean 1.48±2.34) fmol/mL, and big ET-1 levels between 0.05 and 2.92 (mean 0.84±0.58) fmol/mL. No significant relationships were found between ET-1/big ET-1 levels and functional class as well as haemodynamic indices of PH severity. Mean follow-up after catheterisation was 63.2±44.1 months. While 31 of the 66 (47%) patients with PH reached a predefined end point, there was no significant relation between levels of ET-1/big ET-1 and patient outcome.
Although children with PH had alterations in ET-1/big ET-1 expression, which may reflect changes in net release or lung clearance, levels of ET-1/big ET-1 showed no correlation with clinical and haemodynamic parameters, and were not able to predict outcome.
已知肺动脉高压与内皮素（ET）-1及其前体大ET-1的表达增加有关。因此，我们推测在患有肺动脉高压（PH）的儿童中，ET-1和大ET-1水平的改变可能具有临床和预后影响。
纳入66例患有不同形式PH的儿童（平均年龄10.4±9.7岁）。从肺动脉和体动脉采集血样。通过酶联免疫吸附测定法测量ET-1/大ET-1水平，并与临床和血流动力学数据进行比较。为评估预后相关性，进行了Kaplan-Meier生存分析，将终点定义为死亡、肺移植、使用静脉内前列腺素和进行Potts分流术的综合情况。
ET-1水平在0.09至11.64（平均1.48±2.34）fmol/mL之间，大ET-1水平在0.05至2.92（平均0.84±0.58）fmol/mL之间。未发现ET-1/大ET-1水平与功能分级以及PH严重程度的血流动力学指标之间存在显著关系。导管插入术后的平均随访时间为63.2±44.1个月。虽然66例（47%）PH患者中有31例达到了预先定义的终点，但ET-1/大ET-1水平与患者预后之间无显著关系。
虽然患有PH的儿童ET-1/大ET-1表达存在改变，这可能反映了净释放或肺清除的变化，但ET-1/大ET-1水平与临床和血流动力学参数无相关性，且无法预测预后。
