Desai Manan H, Ceneri Nicolle M, Dhari Zaenab, Tongut Aybala, Ozturk Mahmut, Staffa Steven J, Zurakowski David, Schidlow David, Sinha Pranava, Jonas Richard A, Yerebakan Can
Division of Cardiovascular Surgery, Children's National Heart Institute, Children's National Hospital, The George Washington University School of Medicine and Health Sciences, Washington, DC.
Departments of Anesthesiology and Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Mass.
JTCVS Open. 2022 Dec 20;13:292-306. doi: 10.1016/j.xjon.2022.12.004. eCollection 2023 Mar.
The study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality.
A retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan-Meier analysis for survival were performed.
Patient mortality in the cohort was 34% (28/82), including 36% (20/55) for single ventricle palliation and 30% (8/27) for biventricular repair. At 5 years, the probability of survival did not differ between the groups by log-rank testing ( = .829). Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; < .001), total anomalous pulmonary venous return (hazard ratio, 4.3; 95% confidence interval, 1.7-10.8; = .002), and birth weight 2.5 kg or less (hazard ratio, 2.4; 95% confidence interval, 1.0-5.4; = .041) to be independent risk factors for mortality in all-comers. Pulmonary vein stenosis was a univariate predictor of mortality among all patients with heterotaxy (hazard ratio, 3.0; 95% confidence interval, 1.4-6.4; = .005) and in the subgroup of patients with single ventricles (hazard ratio, 4.0; 95% confidence interval, 1.7-9.7; = .002). Overall survival was 66% (54/82) at a median follow-up time of 2.2 years (0.4-4.1) from the initial surgery.
Outcomes of children with heterotaxy syndrome, irrespective of the operative pathway, remain suboptimal in the current era. Risk factors for mortality in this population include birth weight 2.5 kg or less, extracorporeal membrane oxygenation, pulmonary vein stenosis, and total anomalous pulmonary venous return, which may help to further optimize surgical decision making. Multiorgan system involvment is frequently encountered in these patients.
本研究旨在分析接受心血管手术的异相综合征患儿的手术结果，并确定死亡的预测因素。
对2008年1月至2017年12月期间接受心血管手术的82例诊断为异相综合征的患者进行回顾性分析。进行单因素和多因素Cox回归分析以确定死亡的危险因素，并进行Kaplan-Meier生存分析。
该队列患者的死亡率为34%（28/82），其中单心室姑息治疗患者的死亡率为36%（20/55），双心室修复患者的死亡率为30%（8/27）。5年时，通过对数秩检验，两组间的生存率无差异（P = 0.829）。多因素分析发现，体外膜肺氧合支持（风险比，10.4；95%置信区间，4.3 - 25.4；P < 0.001）、完全性肺静脉异位引流（风险比，4.3；95%置信区间，1.7 - 10.8；P = 0.002）以及出生体重2.5kg及以下（风险比，2.4；95%置信区间，1.0 - 5.4；P = 0.041）是所有患者死亡的独立危险因素。肺静脉狭窄是所有异相综合征患者（风险比，3.0；95%置信区间，1.4 - 6.4；P = 0.005）以及单心室患者亚组（风险比，4.0；95%置信区间，1.7 - 9.7；P = 0.002）死亡的单因素预测指标。自初次手术起，中位随访时间为2.2年（0.4 - 4.1年）时，总体生存率为66%（54/82）。
在当前时代，无论手术方式如何，异相综合征患儿的手术结果仍不理想。该人群死亡的危险因素包括出生体重2.5kg及以下、体外膜肺氧合、肺静脉狭窄和完全性肺静脉异位引流，这些因素可能有助于进一步优化手术决策。这些患者经常出现多器官系统受累的情况。
