Martin Billie-Jean, Mah Kandice, Eckersley Luke, Harder Joyce, Pockett Charissa, Schantz Daryl, Dyck John, Al Aklabi Mohammed, Rebeyka Ivan M, Ross David B
Department of Cardiothoracic Surgery, Stanford University, Stanford, California.
Department of Pediatrics, University of Alberta, Edmonton, Canada.
Ann Thorac Surg. 2017 Dec;104(6):2037-2044. doi: 10.1016/j.athoracsur.2017.08.032. Epub 2017 Oct 31.
An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation.
All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival. Characteristics were compared between those with and without HLHS, and the association between outcomes and HLHS were assessed.
A total of 320 children (median age 3.3 years, interquartile range 2.8 to 3.9 years; 121 [43.4%] female) underwent a Fontan procedure over the course of the study. Nearly one third of subjects had HLHS (107, 33.4%). Patients with HLHS were more likely to have abnormal ventricular function (19.6% versus 7.0%, p = 0.003) and worse than mild atrioventricular valve (AVV) regurgitation (23.4 versus 9.2%, p = 0.001) preoperatively. HLHS was not predictive of in-hospital Fontan failure (odds ratio 0.82, 95% CI 0.28, 2.39), late reintervention (hazard ratio [HR] 1.08, 95% CI 0.66, 1.76), or transplant-free survival (HR 1.58, 95% CI 0.72, 3.44). Subjects with HLHS were more likely to have more than mild AVV regurgitation (31.6% versus 13.3%, p = 0.028) and abnormal ventricular function (29.8% versus 10.7%, p < 0.0001) at late follow-up.
Patients with HLHS who survive to the Fontan procedure do no worse with the operation than those with other anatomy. Given worse late ventricular function and AVV regurgitation, equivalent survival may not persist throughout a patient's life course.
单心室生理状态的患者中，患有左心发育不全综合征（HLHS）的比例在不断增加。我们的目的是评估HLHS与Fontan手术后的预后之间的关联。
纳入1996年至2016年间在阿尔伯塔大学接受Fontan手术的所有儿科患者。收集的随访临床数据包括早期和晚期手术或导管再干预、超声心动图以及长期无移植生存率。比较有和没有HLHS患者的特征，并评估预后与HLHS之间的关联。
在研究过程中，共有320名儿童（中位年龄3.3岁，四分位间距2.8至3.9岁；121名[43.4%]为女性）接受了Fontan手术。近三分之一的受试者患有HLHS（107名，33.4%）。HLHS患者术前更有可能出现心室功能异常（19.6%对7.0%，p = 0.003）以及比轻度房室瓣（AVV）反流更严重的情况（23.4对9.2%，p = 0.001）。HLHS不能预测住院期间Fontan手术失败（比值比0.82，95%可信区间0.28，2.39）、晚期再干预（风险比[HR]1.08，95%可信区间0.66，1.76）或无移植生存率（HR 1.58，95%可信区间0.72，3.44）。在晚期随访中，HLHS患者更有可能出现比轻度AVV反流更严重的情况（31.6%对13.3%，p = 0.028）和心室功能异常（29.8%对10.7%，p < 0.0001）。
存活至Fontan手术的HLHS患者手术效果并不比其他解剖结构的患者差。鉴于晚期心室功能和AVV反流较差，同等的生存率可能不会在患者的整个生命过程中持续存在。
