Latus Heiner, Werz Anna, Kock Ines, Rupp Stefan, Kerst Gunter, Kreuder Joachim, Schranz Dietmar, Apitz Christian
Pediatric Heart Centre, Justus-Liebig-University, Giessen, Germany,
Pediatr Cardiol. 2014 Jun;35(5):844-50. doi: 10.1007/s00246-014-0864-z. Epub 2014 Jan 19.
Pulmonary arterial endothelial function is known to be affected in patients with idiopathic pulmonary arterial hypertension (IPAH). Current reports also detected peripheral systemic arterial dysfunction in IPAH patients. The purpose of this study was to assess whether there is a relation between pulmonary arterial and systemic arterial endothelial function. Pulmonary arterial endothelium-dependent relaxation was assessed by changes in pulmonary blood flow in response to acetylcholine which were determined using intravascular Doppler flow measurements. Pulmonary flow reserve (PFR) was calculated as the ratio of pulmonary blood flow velocity in response to acetylcholine relative to baseline values. Systemic arterial endothelial function was assessed by the vascular response to reactive hyperemia, and was recorded non-invasively by peripheral arterial finger tonometry under standardized conditions. Thirteen children and young adults [mean age 16.7 (±5.6) years] with IPAH and 13 age-/gender-matched controls were included in the study. Digital reactive hyperemic index (RHI) of the IPAH patients was 1.54 (±0.69), and of the controls was 1.67 (±0.66) [p = 0.64]. The mean baseline flow velocity in the segmental pulmonary artery of all patients was 18.5 (±5.5) cm/s, increasing to 27.4 (±12.3) cm/s (p = 0.003) during acetylcholine infusion. The calculated mean PFR was 1.48 (±0.4). There was no significant correlation between the PFR and RHI (r = 0.19; p = 0.54). According to our results, systemic arterial endothelial function assessed by peripheral arterial finger tonometry was not significantly impaired in children and young adults with IPAH compared with age-/gender-matched controls. There was no correlation between systemic arterial and pulmonary arterial endothelial function, suggesting that different mechanisms may contribute to their pathogenesis and progression.
已知特发性肺动脉高压（IPAH）患者的肺动脉内皮功能会受到影响。目前的报告还检测到IPAH患者存在外周系统性动脉功能障碍。本研究的目的是评估肺动脉和系统性动脉内皮功能之间是否存在关联。通过血管内多普勒血流测量法测定乙酰胆碱引起的肺血流变化，以此评估肺动脉内皮依赖性舒张功能。肺血流储备（PFR）计算为乙酰胆碱刺激后肺血流速度与基线值的比值。通过对反应性充血的血管反应评估系统性动脉内皮功能，并在标准化条件下通过外周动脉手指张力测量法进行无创记录。本研究纳入了13名儿童和青年[平均年龄16.7（±5.6）岁]IPAH患者以及13名年龄和性别匹配的对照组。IPAH患者的数字反应性充血指数（RHI）为1.54（±0.69），对照组为1.67（±0.66）[p = 0.64]。所有患者节段性肺动脉的平均基线血流速度为18.5（±5.5）cm/s，在输注乙酰胆碱期间增加至27.4（±12.3）cm/s（p = 0.003）。计算得出的平均PFR为1.48（±0.4）。PFR与RHI之间无显著相关性（r = 0.19；p = 0.54）。根据我们的结果，与年龄和性别匹配的对照组相比，通过外周动脉手指张力测量法评估的系统性动脉内皮功能在IPAH儿童和青年中未受到显著损害。系统性动脉和肺动脉内皮功能之间无相关性，这表明不同机制可能导致它们的发病机制和进展。
