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单心室姑息治疗中改良上腔静脉肺动脉吻合术的当代疗效:是否有改进空间?

Contemporary Outcomes for Superior Cavopulmonary Connection in Single Ventricle Palliation: Is There Room for Improvement?

作者信息

John Mohan M, Maher Kevin O, Adamson Marissa, Menon Ambika, Qian Joshua, Brady Maximilian, Golloshi Klevi, Cote Olivia, Gathoo Asmita, Mohnalkar Esha, Zinyandu Tawanda, Shaw Fawwaz R, Deshpande Shriprasad R, Beshish Asaad G

机构信息

Department of Surgery, Division of Cardiothoracic Surgery, Emory University School of Medicine, Children'S Healthcare of Atlanta, Atlanta, GA, USA.

Department of Pediatrics, Division of Cardiology, Emory University School of Medicine, Children'S Healthcare of Atlanta, Atlanta, GA, USA.

出版信息

Pediatr Cardiol. 2025 Apr 30. doi: 10.1007/s00246-025-03853-1.

Abstract

The superior cavopulmonary connection (SCPC) is performed as a part of staged palliation for single ventricle disease. With improvements in interstage management and patient selection, the attrition rate following SCPC is less than 10% in the modern era. We sought to examine the trends and outcomes of SCPC at our institution over the past 14 years. A retrospective review was conducted of patients who underwent SCPC at our institution from 2007 to 2020. Patients were divided into two groups based on date of surgery-pre- 2014 (2007-201) and 2014 + (2014-2020). Demographic and clinical characteristics were compared, along with follow-up survival data. Five hundred and fifty two patients underwent SCPC during the study period, of whom 306 (55.4%) were in the 2014 + era. Patients in the 2014 + era were more likely to be African American (40.2% vs. 31.7%, p = 0.02) or have a genetic abnormality (28.4% vs. 20.6%, p = 0.04). On preoperative echocardiogram, 2014 + patients had more significant (≥ moderate) atrioventricular valve regurgitation (AVVR) (19.6 vs. 10.6%, p = 0.003). Operative and interstage mortality did not differ between eras (p > 0.05). In addition, there was no difference in long-term survival (HR 0.66 [CI 0.34-1.29], p = 0.22) between groups. On multivariable analysis, only ≥ moderate ventricular dysfunction (HR 4.23 [CI 1.18-12.02], p = 0.01) and hypoplastic pulmonary arteries (HR 3.03 [CI 1.17-10.36], p = 0.04) were associated with lower survival. Overall survival following SCPC is excellent, with no improvement in survival in the 2014 + era. More patients with high-risk factors-genetic syndromes and significant AVVR-were operated on in the recent era, without an adverse effect on survival.

摘要

上腔静脉肺动脉连接术(SCPC)是单心室疾病分期姑息治疗的一部分。随着过渡期管理和患者选择的改善,在现代,SCPC后的损耗率低于10%。我们试图研究过去14年我院SCPC的趋势和结果。对2007年至2020年在我院接受SCPC的患者进行了回顾性研究。根据手术日期将患者分为两组——2014年前(2007 - 2013年)和2014年及以后(2014 - 2020年)。比较了人口统计学和临床特征以及随访生存数据。在研究期间,552例患者接受了SCPC,其中306例(55.4%)在2014年及以后时期。2014年及以后时期的患者更可能是非裔美国人(40.2%对31.7%,p = 0.02)或有基因异常(28.4%对20.6%,p = 0.04)。在术前超声心动图检查中,2014年及以后时期的患者有更显著(≥中度)的房室瓣反流(AVVR)(19.6%对10.6%,p = 0.003)。不同时期的手术死亡率和过渡期死亡率无差异(p > 0.05)。此外,两组之间的长期生存率无差异(风险比0.66 [可信区间0.34 - 1.29],p = 0.22)。在多变量分析中,只有≥中度心室功能障碍(风险比4.23 [可信区间1.18 - 12.02],p = 0.01)和肺发育不全(风险比3.03 [可信区间1.17 - 10.36],p = 0.04)与较低的生存率相关。SCPC后的总体生存率良好,2014年及以后时期的生存率没有改善。在最近时期,有更多具有高危因素——基因综合征和显著AVVR的患者接受了手术,且对生存率没有不利影响。

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