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左心发育不全综合征右心室重构受体外循环影响较小:比较 Norwood 与杂交术。

Right Ventricular Remodeling in Hypoplastic Left Heart Syndrome is Minimally Impacted by Cardiopulmonary Bypass: A Comparison of Norwood vs. Hybrid.

机构信息

Division of Pediatric Cardiology, Stollery Children's Hospital, 8440 112 St NW, unit 4C2.36, Edmonton, AB, T6G2B7, Canada.

Mazankowski Alberta Heart Institute, Alberta Health Services, 11220 83 Ave NW, Edmonton, AB, T6G 2B7, Canada.

出版信息

Pediatr Cardiol. 2021 Feb;42(2):294-301. doi: 10.1007/s00246-020-02482-0. Epub 2020 Oct 10.

Abstract

Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV. This study aims to determine the impact of CPB on RV remodeling in HLHS. Echocardiograms of HLHS survivors undergoing stage 1 Norwood (n = 26) or Hybrid (n = 20) were analyzed at pre- and post-stage 1, pre- and post-bidirectional cavo-pulmonary anastomosis (BCPA), and pre-Fontan. RV fractional area change (FAC), vector velocity imaging for longitudinal & derived circumferential deformation (global radial shortening (GRS) = peak radial displacement/end-diastolic diameter), and deformation ratio (longitudinal/ circumferential) were assessed. Both groups had similar age, clinical status and functional parameters pre-stage 1. No difference in RV size and sphericity at any stage between groups. RVFAC was normal (> 35%) throughout for both groups. Both Norwood and Hybrid patients had increased GRS (p = 0.0001) post-stage 1 and corresponding unchanged longitudinal strain, resulting in decreased deformation ratio (greater relative RV circumferential contraction), p = 0.0001. Deformation ratio remained decreased in both groups in subsequent stages. Irrespective of timing of the first CPB exposure, both Norwood and Hybrid patients underwent similar RV remodeling, with relative increase in circumferential to longitudinal contraction soon after stage 1 palliation. The observed RV remodeling in HLHS survivors were minimally impacted by CPB.

摘要

左心发育不全综合征(HLHS)患者的右心室(RV)重构始于产前,并在分期姑息治疗过程中持续进行。然而,目前尚不清楚术后 Norwood 手术中观察到的最明显的重构是继发于体外循环(CPB)暴露,还是继发于系统 RV 的内在适应。本研究旨在确定 CPB 对 HLHS 患者 RV 重构的影响。对接受第 1 阶段 Norwood 手术(n=26)或 Hybrid 手术(n=20)的 HLHS 幸存者的超声心动图进行分析,在第 1 阶段前、后,双向腔静脉肺动脉吻合术(BCPA)前、后和 Fontan 前进行分析。评估 RV 分数面积变化(FAC)、纵向和衍生圆周应变的向量速度成像(整体径向缩短(GRS)=峰值径向位移/舒张末期直径)和应变比(纵向/圆周)。两组患者在第 1 阶段前均具有相似的年龄、临床状况和功能参数。两组在任何阶段的 RV 大小和球形度均无差异。两组患者的 RVFAC 在整个过程中均正常(>35%)。Norwood 和 Hybrid 组患者在第 1 阶段后均出现 GRS 增加(p=0.0001),而纵向应变不变,导致应变比降低(RV 圆周收缩相对增加),p=0.0001。两组在随后的阶段中应变比均降低。无论首次 CPB 暴露的时间如何,Norwood 和 Hybrid 患者的 RV 重构方式相似,第 1 阶段姑息治疗后不久,圆周向与纵向收缩的相对增加。HLHS 幸存者的 RV 重构受 CPB 的影响极小。

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