Wei Zhenglun Alan, Johnson Camille, Trusty Phillip, Stephens Morgan, Wu Wenjun, Sharon Ritchie, Srimurugan Balaji, Kottayil Brijesh P, Sunil G S, Fogel Mark A, Yoganathan Ajit P, Kappanayil Mahesh
Department of Biomedical Engineering, Georgia Institute of Technology, Suite 200, 387 Technology Circle, Atlanta, GA, 30313-2412, USA.
Amrita Institute of Medical Sciences and Research Centre, Kochi, India.
Pediatr Cardiol. 2020 Jun;41(5):1021-1030. doi: 10.1007/s00246-020-02353-8. Epub 2020 May 6.
Apicocaval juxtaposition (ACJ) is a rare form of viscerocardiac malpositions in association with single-ventricle congenital heart defects. The Fontan surgery is the common palliation, and possible surgical options include ipsilateral, contralateral, and intra-atrial conduits. Concerns include lower hemodynamic performances or risks of conduit compression by the cardiac mass. This study investigates the hemodynamics and clinical outcomes of ACJ patients and potential surgical improvements. Ten consecutive ACJ patients were included, along with a reference cohort of ten non-ACJ patients. Magnetic resonance images were acquired at 6 ± 0.6 year follow-up for anatomical analysis and hemodynamic assessments using computational fluid dynamics. Metrics of interest are deformation index (DI), indexed power loss (iPL), and hepatic flow distribution (HFD). A "virtual" surgery was performed to explore potential hemodynamic improvements using a straightened conduit. DI for ACJ patients fell within the DI range of non-ACJ patients. Contralateral conduits had insignificantly higher iPL (0.070 [0.032,0.137]) than ipsilateral conduits (0.041 [0.013,0.095]) and non-ACJ conduits (0.034 [0.011,0.061]). HFD was similar for the ipsilateral (21 [12,35]), contralateral (26 [7,41]), and non-ACJ Fontan conduits (17 [0,48]). Virtual surgery demonstrated that a straightened conduit reduced HFD and iPL for the contralateral and ipsilateral conduits, potentially leading to improved clinical outcomes. In this limited sample, the hemodynamic performance of ACJ patients was not significantly different from their non-ACJ counterparts. The use of a straightened conduit option could potentially improve patient outcomes. Additionally, the fear of significant compression of conduits for ACJ patients was unsupported.
心尖-腔静脉并列（ACJ）是一种罕见的内脏-心脏位置异常形式，与单心室先天性心脏缺陷相关。Fontan手术是常见的姑息治疗方法，可能的手术选择包括同侧、对侧和心房内管道。相关问题包括较低的血流动力学性能或心脏肿块压迫管道的风险。本研究调查了ACJ患者的血流动力学和临床结果以及潜在的手术改进方法。纳入了连续的10例ACJ患者，以及10例非ACJ患者作为对照队列。在6±0.6年的随访中获取磁共振图像，用于解剖分析和使用计算流体动力学进行血流动力学评估。感兴趣的指标是变形指数（DI）、指数功率损失（iPL）和肝血流分布（HFD）。进行了一项“虚拟”手术，以探索使用拉直管道潜在的血流动力学改善情况。ACJ患者的DI落在非ACJ患者的DI范围内。对侧管道的iPL（0.070[0.032,0.137]）略高于同侧管道（0.041[0.013,0.095]）和非ACJ管道（0.034[0.011,0.061]）。同侧（21[12,35]）、对侧（26[7,41]）和非ACJ Fontan管道的HFD相似（17[0,48]）。虚拟手术表明，拉直管道可降低对侧和同侧管道的HFD和iPL，可能导致临床结果改善。在这个有限的样本中，ACJ患者的血流动力学性能与非ACJ患者没有显著差异。使用拉直管道选项可能会改善患者的预后。此外，对ACJ患者管道严重受压恐惧的担忧没有依据。
