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有症状法洛四联症新生儿的临床状况与再次干预:一项标志性分析。

Clinical Status and Reintervention in Neonates With Symptomatic Tetralogy of Fallot: A Landmark Analysis.

作者信息

Meadows Jeffery J, Zhang Yun, Petit Christopher J, Goldstein Bryan H, McCracken Courtney E, Beshish Asaad, Nicholson George T, Law Mark A, Zampi Jeffrey D, Shahanavaz Shabana, Chai Paul J, Romano Jennifer C, Batlivala Sarosh P, Maskatia Shiraz A, Asztalos Ivor B, Khan Hala Q, Kamsheh Alicia M, Healan Steven J, Smith Justin D, Ligon R Allen, Bauser-Heaton Holly, Dailey-Schwartz Andrew, Pettus Joelle A, Pajk Amy L, Glatz Andrew C, Mascio Christopher E, Qureshi Athar M

机构信息

Benioff Children's Hospital, Division of Cardiology, University of California, San Francisco, California, USA.

Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, Georgia, USA.

出版信息

JACC Adv. 2025 Jul;4(7):101919. doi: 10.1016/j.jacadv.2025.101919.

Abstract

BACKGROUND

In symptomatic neonates with tetralogy of Fallot (sTOF), the initial treatment strategy significantly affects early outcomes, but its long-term impact remains less well defined.

OBJECTIVES

The aim of the study was to compare primary (PR) vs staged repair (SR) in sTOF with respect to reintervention (RI) rates and types, clinical and echocardiographic outcomes, and medication use.

METHODS

Neonates with sTOF undergoing PR or SR and with >1 year of follow-up after complete repair were included. The primary outcome was cumulative RI incidence; secondary outcomes included mortality and late echocardiographic and clinical findings. Propensity scoring adjusted for baseline differences. Landmark analysis assessed RI risk at yearly intervals following complete repair.

RESULTS

Of 441 neonates, 182 (41%) underwent PR, and 259 (59%) underwent SR. Groups differed in gestational age, intubation, and 22q11 status. Median follow-up postrepair was 5.26 (2.91, 8.21) years. RI burden was high in both groups, with a small, consistent but nonsignificant advantage to PR. The type of RI varied over time. PR was associated with greater pulmonary insufficiency and larger pulmonary arteries. RV pressure was ≤half systemic in 80%; 10% had ≥moderate tricuspid regurgitation, without between-group difference. Elevated RV pressure was associated with ≥moderate tricuspid regurgitation.

CONCLUSIONS

Among sTOF survivors beyond the early perioperative period, late RI burden and residual hemodynamic lesions are common and largely unrelated to initial strategy. PR is associated with increased pulmonary insufficiency and pulmonary artery size.

摘要

背景

在有症状的法洛四联症新生儿(sTOF)中,初始治疗策略对早期结局有显著影响,但其长期影响仍不太明确。

目的

本研究旨在比较sTOF的一期修复(PR)与分期修复(SR)在再次干预(RI)率及类型、临床和超声心动图结局以及药物使用方面的差异。

方法

纳入接受PR或SR且完全修复后随访超过1年的sTOF新生儿。主要结局是累积RI发生率;次要结局包括死亡率以及晚期超声心动图和临床检查结果。采用倾向评分法调整基线差异。地标分析评估完全修复后每年的RI风险。

结果

441例新生儿中,182例(41%)接受PR,259例(59%)接受SR。两组在胎龄、插管情况和22q11状态方面存在差异。修复后的中位随访时间为5.26(2.91,8.21)年。两组的RI负担都很高,PR组有一个小的、持续但不显著的优势。RI的类型随时间变化。PR与更严重的肺功能不全和更大的肺动脉有关。80%的右心室压力≤体循环压力的一半;10%有≥中度三尖瓣反流,两组之间无差异。右心室压力升高与≥中度三尖瓣反流有关。

结论

在早期围手术期后的sTOF幸存者中,晚期RI负担和残余血流动力学病变很常见,且在很大程度上与初始策略无关。PR与肺功能不全增加和肺动脉大小增加有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/88a2/12418465/4abd01e4dd08/ga1.jpg

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