Arunamata Alisa, Tacy Theresa A, Kache Saraswati, Mainwaring Richard D, Ma Michael, Maeda Katsuhide, Punn Rajesh
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Stanford, CA, USA.
Department of Pediatrics, Division of Critical Care Medicine, Stanford University School of Medicine, Stanford, CA, USA.
Ann Pediatr Cardiol. 2020 Jul-Sep;13(3):186-193. doi: 10.4103/apc.APC_5_20. Epub 2020 Jun 29.
To investigate patient-related factors, echocardiographic, and anatomic variables associated with immediate and long-term clinical outcomes after extracardiac Fontan procedure at our institution.
Retrospective review of preoperative cardiac catheterizations and echocardiograms as well as medical records of all children with hypoplastic left heart syndrome (HLHS) who underwent Fontan between June 2002 and December 2018.
Seventy-seven patients with HLHS were included (age 4 years [1.5-11.7]). Seventy patients (91%) received a nonfenestrated Fontan and 57 patients (74%) underwent Fontan without cardiopulmonary bypass (CPB). Presence of a Fontan fenestration ( = 0.69) and use of CPB ( = 0.79) did not differ between those with <2 weeks compared to those with ≥2 weeks of chest tube drainage. There were no differences in either pre- or intra-operative hemodynamics between patients who weighed <15 kg compared to those who weighed ≥15 kg at time of surgery; incidence of death, transplant, and transplant listing were similar between weight groups. Inferior vena cava (IVC) diameter z-score did not differ among patients with and without chylous chest tube drainage ( = 0.78), with and without development of protein losing enteropathy ( = 0.23), or death/heart transplant/transplant listing compared to survivors without transplant ( = 0.26).
In HLHS patients undergoing Fontan, preoperative weight and IVC diameter appeared to have no influence on immediate postoperative outcomes. Performing the Fontan off CPB and with a fenestration also conferred no added clinical benefit. These observations should be considered when deciding optimal timing for Fontan completion.
研究我院心外Fontan手术术后近期和远期临床结局相关的患者因素、超声心动图及解剖学变量。
回顾性分析2002年6月至2018年12月期间所有接受Fontan手术的左心发育不全综合征（HLHS）患儿的术前心导管检查、超声心动图及病历资料。
纳入77例HLHS患者（年龄4岁[1.5 - 11.7]）。70例（91%）接受了无开窗Fontan手术，57例（74%）在非体外循环（CPB）下进行Fontan手术。胸腔闭式引流<2周与≥2周的患者相比，Fontan开窗的存在（P = 0.69）和CPB的使用（P = 0.79）无差异。手术时体重<15 kg与≥15 kg的患者相比，术前或术中血流动力学无差异；各体重组的死亡、移植及列入移植名单的发生率相似。下腔静脉（IVC）直径z评分在有或无乳糜胸闭式引流的患者之间（P = 0.78）、有或无蛋白丢失性肠病发生的患者之间（P = 0.23）以及与未接受移植的幸存者相比的死亡/心脏移植/列入移植名单的患者之间（P = 0.26）无差异。
在接受Fontan手术的HLHS患者中，术前体重和IVC直径似乎对术后近期结局无影响。在非CPB下且有开窗进行Fontan手术也未带来额外的临床益处。在决定Fontan手术的最佳时机时应考虑这些观察结果。
