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英国对患有法洛四联症的有症状幼儿的当代管理方法。

Contemporary Approach to Managing Young Symptomatic Infants With Tetralogy of Fallot in the United Kingdom.

作者信息

Hribernik Ines, Boardman Stacey, Crossland David, Hermuzi Antony, Bellsham-Revell Hannah, Elnazir Patrick, Ho Andrew, Yap Norah, Taliotis Demetris, Gibb Jack, Bentham James R

机构信息

Department of Paediatric Cardiology, Leeds Teaching Hospitals Trust, Leeds, UK.

Department of Paediatric Cardiology, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK.

出版信息

World J Pediatr Congenit Heart Surg. 2025 Nov;16(6):817-826. doi: 10.1177/21501351251322879. Epub 2025 May 29.

Abstract

ObjectivesWe set out to determine the characteristics and outcomes of symptomatic infants with tetralogy of Fallot who due to insufficient pulmonary blood flow required transcatheter or surgical intervention in early infancy.MethodsFive centers in the United Kingdom contributed data for infants born with tetralogy of Fallot who underwent an initial procedure between January 2015 and January 2022. The studied group were infants requiring palliative or reparative procedure at less than three months of age. The primary outcome was survival to one year; secondary outcomes were periprocedural complications, cumulative mechanical ventilation time, length of hospital stay, and need for reintervention.ResultsA total of 535 infants with tetralogy of Fallot were identified during the study period. 96 infants underwent initial palliation (58 right ventricular outflow tract stent, 7 ductal stent, 17 balloon pulmonary valvuloplasty, 9 modified Blalock-Taussig-Thomas shunt, and 5 right ventricular outflow tract patch augmentation). 37 infants underwent primary repair at less than three months of age, 402 infants had primary repair at 3 to 12 months of age. Median age and weight were 26 days and 3.1 kg for initial palliation; 68 days and 4.7 kg for primary repair under three months ( < .001). One year survival post-procedure was 95.7% for initial palliation, and 97.3% for primary repair under three months ( = .55, hazard ratio [HR] 1.97, 95% confidence interval [CI] 0.33-11.67). Five-year freedom from reintervention on the right ventricle-to-pulmonary artery segment was 76% after staged repair and 88% after primary repair under three months ( = .23, HR 1.90, 95% CI 0.75-4.80).ConclusionsInitial palliation with staged repair remains the predominantly employed approach for the highest risk young infants with tetralogy of Fallot in the United Kingdom. Survival outcomes are excellent; concerns remain regarding increased cumulative costs and associated reintervention rates. Primary repair under three months of age has also proven to be feasible with excellent results, although with individual candidate selection bias.

摘要

目的

我们旨在确定患有法洛四联症且因肺血流量不足在婴儿早期需要经导管或手术干预的有症状婴儿的特征和预后。

方法

英国的五个中心提供了2015年1月至2022年1月期间出生并接受了初次手术的法洛四联症婴儿的数据。研究组为在三个月龄以下需要姑息或修复手术的婴儿。主要结局是存活至一岁;次要结局是围手术期并发症、累计机械通气时间、住院时间以及再次干预的需求。

结果

在研究期间共识别出535例法洛四联症婴儿。96例婴儿接受了初次姑息治疗(58例右心室流出道支架置入、7例动脉导管支架置入、17例球囊肺动脉瓣成形术、9例改良布莱洛克 - 陶西格 - 托马斯分流术以及5例右心室流出道补片扩大术)。37例婴儿在三个月龄以下接受了一期修复,402例婴儿在3至12个月龄时接受了一期修复。初次姑息治疗的中位年龄和体重分别为26天和3.1千克;三个月龄以下一期修复的中位年龄和体重分别为68天和4.7千克(P < 0.001)。初次姑息治疗术后一年生存率为95.7%,三个月龄以下一期修复术后一年生存率为97.3%(P = 0.55,风险比[HR] 1.97,95%置信区间[CI] 0.33 - 11.67)。分期修复后右心室至肺动脉段五年无再次干预率为76%,三个月龄以下一期修复后为88%(P = 0.23,HR 1.90,95% CI 0.75 - 4.80)。

结论

在英国,对于风险最高的法洛四联症小婴儿,初次姑息治疗分期修复仍然是主要采用的方法。生存结局良好;但对于累计成本增加和相关再次干预率仍存在担忧。三个月龄以下的一期修复也已证明是可行的,结果良好,尽管存在个体候选者选择偏倚。

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