Alsoufi Bahaaldin, Rosenblum Joshua, Travers Curtis, Kanter Kirk, Trusty Philip M, Yoganathan Ajit P, Slesnick Timothy P
1 Department of Cardiothoracic Surgery, University of Louisville, Norton Children's Hospital, Louisville, KY, USA.
2 Department of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA.
World J Pediatr Congenit Heart Surg. 2019 Jan;10(1):20-27. doi: 10.1177/2150135118809082.
Current technology advances in virtual surgery modeling and computational flow dynamics allow preoperative individualized computer-based design of Fontan operation. To determine potential role of those innovations in patients undergoing hepatic vein incorporation (HVI) following Kawashima operation, we retrospectively examined historic cohort of patients who underwent HVI following Kawashima with focus on regression of pulmonary arteriovenous malformations (PAVMs).
Twenty-two children with single ventricle and interrupted inferior vena cava underwent Kawashima operation (2002-12). Twenty-one (96%) patients had left atrial isomerism and 21 (96%) had undergone prior first-stage palliation. Clinical outcomes were examined.
Mean O saturation (SaO) increased from 77% ± 8% to 85% ± 6% ( P = .002) after Kawashima. Fifteen (68%) patients developed PAVMs. Eighteen patients underwent HVI (median age and interval from Kawashima: 4.4 and 3.7 years, respectively). Mean SaO prior to HVI was 77% ± 8% and increased to 81% ± 10% at the time of hospital discharge ( P = .250), with five patients requiring home oxygen. On follow-up, mean SaO increased to 95% ± 4% ( P < .001). Overall ten-year survival following Kawashima was 94%.
A large number of patients develop PAVMs and subsequent cyanosis after Kawashima operation. Early following HVI, SaO is commonly low and insignificantly different from that prior to HVI. Although SaO will improve on follow-up in most patients, a number of patients continue to have low saturations, indicating incomplete resolution of PAVMs. Given the heterogeneity of those patients and lack of preoperative predictors for complete PAVM regression, our findings suggest a role for virtual surgery to determine optimal individual procedure design that would provide even distribution of hepatic blood flow to both pulmonary arteries.
虚拟手术建模和计算流体动力学方面的当前技术进步使得能够在术前基于计算机进行Fontan手术的个体化设计。为了确定这些创新技术在川岛手术（Kawashima operation）后接受肝静脉纳入术（hepatic vein incorporation, HVI）的患者中的潜在作用，我们回顾性研究了川岛手术后接受HVI的患者的历史队列，重点关注肺动静脉畸形（pulmonary arteriovenous malformations, PAVMs）的消退情况。
22名单心室和下腔静脉中断的儿童接受了川岛手术（2002 - 2012年）。21名（96%）患者存在左心房异构，21名（96%）患者曾接受过一期姑息手术。对临床结果进行了检查。
川岛手术后，平均氧饱和度（SaO）从77%±8%升至85%±6%（P = 0.002）。15名（68%）患者出现了PAVMs。18名患者接受了HVI（中位年龄和距川岛手术的间隔时间分别为4.4岁和3.7年）。HVI术前的平均SaO为77%±8%，出院时升至81%±10%（P = 0.250），5名患者需要家庭吸氧。在随访中，平均SaO升至95%±4%（P < 0.001）。川岛手术后总体十年生存率为94%。
大量患者在川岛手术后出现PAVMs及随后的青紫。HVI术后早期，SaO通常较低，且与HVI术前无显著差异。尽管大多数患者随访时SaO会改善，但仍有一些患者饱和度持续较低，表明PAVMs未完全消退。鉴于这些患者的异质性以及缺乏PAVM完全消退的术前预测指标，我们的研究结果表明虚拟手术在确定最佳个体化手术设计方面具有作用，该设计可使肝血流均匀分布至双侧肺动脉。
