Iwaya Yuka, Muneuchi Jun, Inoue Yuka, Watanabe Mamie, Okada Seigo, Ochiai Yoshie
Division of Pediatric Cardiology, Department of Pediatrics, Japan Community Healthcare Organization, Kyushu Hospital, 1-8-1, Kishinoura, Yahatanishiku, Kitakyushu, Fukuoka, 806-8501, Japan.
Division of Pediatric Cardiology, Department of Cardiovascular Surgery, Japan Community Healthcare Organization, Kyushu Hospital, 1-8-1, Kishinoura, Yahatanishiku, Kitakyushu, Fukuoka, 806-8501, Japan.
Pediatr Cardiol. 2019 Apr;40(4):841-847. doi: 10.1007/s00246-019-02080-9. Epub 2019 Mar 4.
This study aimed to clarify the characteristics of pulmonary arterial resistance (Rp)-compliance (Cp) coupling in individuals with Down syndrome (DS), who have increased risks of pulmonary arterial hypertension (PAH). We performed cardiac catheterization before and after corrective surgery in 85 DS infants and 85 controls with congenital heart disease and PAH. We retrospectively collected hemodynamic data and compared Rp and Cp between the groups. Age at surgery was 3.5 (2.6-4.6) months. The first and second catheterizations were performed 1 month before and after corrective surgery in both groups. Preoperative Cp in DS patients was significantly lower than that in controls [2.27 (1.62-3.0) vs. 2.50 (1.86-3.31) mL/mmHg/m, p = 0.039], although there was no significant difference in mean pulmonary arterial pressure and Rp between the groups. Analysis of covariance revealed that the slopes of the preoperative regression lines for the logarithmic transformations of Rp and Cp were identical in DS patients and controls (p = 0.299). However, the postoperative regression line was shifted downward in DS patients after corrective surgery. Postoperative home oxygen therapy (HOT) was performed in 39 patients (36 DS patients) and multivariate logistic regression analysis revealed that postoperative HOT was significantly related to low preoperative Cp (p = 0.039) and DS (p = 0.0001). Individuals with DS have the unique pulmonary vasculature characterized with low Cp that is related to postoperative HOT.
本研究旨在阐明患有肺动脉高压（PAH）风险增加的唐氏综合征（DS）个体的肺动脉阻力（Rp）-顺应性（Cp）耦合特征。我们对85例患有先天性心脏病和PAH的DS婴儿以及85例对照进行了矫正手术前后的心脏导管插入术。我们回顾性收集了血流动力学数据，并比较了两组之间的Rp和Cp。手术年龄为3.5（2.6 - 4.6）个月。两组均在矫正手术前1个月和术后进行了首次和第二次导管插入术。DS患者术前的Cp显著低于对照组[2.27（1.62 - 3.0）对2.50（1.86 - 3.31）mL/mmHg/m，p = 0.039]，尽管两组之间的平均肺动脉压和Rp没有显著差异。协方差分析显示，DS患者和对照组中Rp和Cp对数转换的术前回归线斜率相同（p = 0.299）。然而，矫正手术后DS患者的术后回归线向下移动。39例患者（36例DS患者）进行了术后家庭氧疗（HOT），多因素逻辑回归分析显示，术后HOT与术前低Cp（p = 0.039）和DS（p = 0.0001）显著相关。DS个体具有独特的肺血管系统，其特征是Cp低，这与术后HOT有关。
