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Title: [Improving outcomes of patients with heterotaxy and functional single ventricle: a 10-year follow-up of 70 cases in a single institution].
Author: Cao F, Zou MH, Ma L, Xia YS, Yang SC, Chen WD, Huang GD, Chen XX.
Journal: Zhonghua Wai Ke Za Zhi; 2018 May 01; 56(5):379-385. PubMed ID: 29779315.
Abstract:
Objective: To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution. Methods: The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children's Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months). Single ventricle multistage palliation included 1(st) stage palliation surgery, 2(nd) bidirectional Glenn shunt, and 3(rd) stage modified Fontan.The Kaplan-Meier method was used to estimate the probability of survival. Multivariate analysis was performed by Cox regression model. Results: Sixty-two patients had right atrial isomerism while eight had left atrial isomerism. Eighteen patients (25.7%) required the first stage palliation. Fifty-five patients received the Glenn shunt, and the Fontan procedure was completed in 27 patients. Overall, mortality occurred in 17 patients (24.3%) including 9 after the first stage palliation, 6 after the Glenn shunt, and 2 following the Fontan procedure. Survival estimates for the entire cohort following surgery were 81% (95%CI: 72% to 90%), 74%(95%CI: 64% to 85%), and 74% (95%CI: 64% to 85%) at 1 year, 5 years and 10 years, respectively. Survival estimates following the first stage palliation were 56% (95%CI: 33% to 79%) and 49% (95%CI: 26% to 73%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (TAPVC) (HR=6.16, 95%CI: 1.65 to 22.95, P=0.007), atrioventricular valve regurgitation more than moderate (HR=3.81, 95%CI: 1.32 to 10.94, P=0.013) and required the first palliation surgery (HR=4.58, 95%CI: 1.34 to 15.72, P=0.016) were prognosis factors for overall mortality. Conclusions: The management of heterotaxy patients with functional single ventricle remains challenging, and the outcomes are continously improving in china. TAPVC, atrioventricular valve regurgitation more than moderate and required the first palliation surgery still are prognosis factors for overall mortality in heterotaxy syndrome. 目的： 评价内脏异位综合征合并单心室的外科治疗效果，分析其死亡预后因素。 方法： 回顾性分析广州市妇女儿童医疗中心心脏中心2008年1月至2017年12月连续收治的70例接受单心室系列手术的内脏异位综合征患者的临床资料及随访结果。男性53例，女性17例；中位年龄为13.3个月(范围：6 d至150个月)。单心室系列手术包括第一期姑息手术、第二期双向Glenn术、第三期改良Fontan术。早期死亡定义为术后30 d死亡或未能出院。采用Kaplan-Meier法进行生存分析，采用Cox回归模型分析患者死亡预后因素。 结果： 右心房异构62例，左心房异构8例。需要第一期姑息手术18例，第二期Glenn术55例，完成Fontan术27例。累计死亡17例，其中早期死亡10例，晚期死亡7例。1年、5年和10年总体生存率分别为81%(95% CI：72%～90%)、74%(95% CI：64%～85%)和74%(95% CI：64%～85%)。第一期姑息术后1年和5年生存率分别为56%(95% CI：33%～79%)和49%(95% CI：26%～73%)。多因素Cox回归分析结果显示，合并完全性肺静脉异位引流(HR＝6.16，95% CI：1.65～22.95，P＝0.007)、中度及以上房室瓣关闭不全(HR＝3.81，95% CI：1.32～10.94，P＝0.013)和需要第一期姑息手术(HR＝4.58，95% CI：1.34～15.72，P＝0.016)为死亡独立预后因素。 结论： 内脏异位综合征合并单心室的外科治疗效果正在持续改善。合并完全性肺静脉异位引流、中度及以上房室瓣关闭不全和需要第一期姑息手术，仍是导致患者死亡的独立预后因素。.

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