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法洛四联症矫正术后的右心室功能障碍:解剖学决定因素及治疗策略

Right ventricular dysfunction following tetralogy of Fallot correction: anatomical determinants and therapeutic strategies.

作者信息

Fan Qiang, Wang Yabo, An Qi, Ling Yunfei

机构信息

Department of Cardiovascular Surgery, West China Hospital of Sichuan University, Chengdu, Sichuan, People's Republic of China.

出版信息

Int J Surg. 2025 Jun 1;111(6):3979-3988. doi: 10.1097/JS9.0000000000002367. Epub 2025 Apr 1.

DOI:10.1097/JS9.0000000000002367
PMID:40171563
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12165513/
Abstract

Right ventricular dysfunction following surgical correction of tetralogy of Fallot (TOF) remains a major determinant of long-term morbidity and mortality in survivors. Despite advancements in surgical techniques, residual anatomical abnormalities - including pulmonary regurgitation, right ventricular outflow tract obstruction, abnormal coronary artery anatomy, scar formation, and tricuspid regurgitation - synergistically drive ventricular remodeling and functional decline. This review synthesizes evidence on the pathophysiological interplay of these anatomical substrates. Key imaging modalities, such as 3D late gadolinium enhancement cardiac magnetic resonance, and artificial intelligence tools enhance risk stratification for ventricular arrhythmias. We propose a hierarchical management framework prioritizing hemodynamic stabilization, electrophysiological substrate modification and individualized strategies for concomitant lesions considerations. This work aims to bridge anatomical insights with therapeutic innovations, offering a roadmap for improving longevity and quality of life in repaired TOF patients.

摘要

法洛四联症(TOF)手术矫正后的右心室功能障碍仍然是幸存者长期发病率和死亡率的主要决定因素。尽管手术技术有所进步,但残余的解剖学异常——包括肺动脉反流、右心室流出道梗阻、冠状动脉解剖异常、瘢痕形成和三尖瓣反流——协同驱动心室重塑和功能衰退。本综述综合了关于这些解剖学基础病理生理相互作用的证据。关键的成像方式,如三维延迟钆增强心脏磁共振成像,以及人工智能工具可增强室性心律失常的风险分层。我们提出了一个分级管理框架,优先考虑血流动力学稳定、电生理基质改良以及针对合并病变的个体化策略。这项工作旨在将解剖学见解与治疗创新联系起来,为提高TOF修复患者的寿命和生活质量提供路线图。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3157/12165513/c6d3ce3c1e8d/js9-111-3979-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3157/12165513/18c5e517e906/js9-111-3979-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3157/12165513/c6d3ce3c1e8d/js9-111-3979-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3157/12165513/18c5e517e906/js9-111-3979-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3157/12165513/c6d3ce3c1e8d/js9-111-3979-g002.jpg

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本文引用的文献

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The impact of tricuspid regurgitation on timing pulmonary valve replacement in ACHD patients after tetralogy of Fallot repair.法洛四联症修复术后先天性心脏病患者中三尖瓣反流对肺动脉瓣置换时机的影响。
Int J Cardiol Congenit Heart Dis. 2025 Mar 13;20:100577. doi: 10.1016/j.ijcchd.2025.100577. eCollection 2025 Jun.
2
Long-Term Management of Right Ventricular Outflow Tract Dysfunction in Repaired Tetralogy of Fallot: A Scientific Statement From the American Heart Association.法洛四联症修复术后右心室流出道功能障碍的长期管理:美国心脏协会科学声明
Circulation. 2024 Dec 17;150(25):e689-e707. doi: 10.1161/CIR.0000000000001291. Epub 2024 Nov 21.
3
Slowly conducting anatomic isthmuses of tetralogy of Fallot: An opportunity for "prophylactic" ventricular tachycardia ablation.
Heart Rhythm. 2025 Oct;22(10):2669-2677. doi: 10.1016/j.hrthm.2024.10.073. Epub 2024 Nov 17.
4
Electrocardiogram-based deep learning to predict mortality in paediatric and adult congenital heart disease.基于心电图的深度学习预测小儿及成人先天性心脏病死亡率
Eur Heart J. 2025 Mar 3;46(9):856-868. doi: 10.1093/eurheartj/ehae651.
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Burden of reintervention after tetralogy of Fallot repair: A joint pediatric and adult congenital experience over 30 years.
J Thorac Cardiovasc Surg. 2025 Mar;169(3):985-998.e4. doi: 10.1016/j.jtcvs.2024.09.042. Epub 2024 Oct 3.
6
Ventricular Tachycardia Substrates in Children and Young Adults With Repaired Tetralogy of Fallot.法洛四联症修补术后儿童及青年人心室性心动过速的基质
JACC Clin Electrophysiol. 2024 Dec;10(12):2613-2624. doi: 10.1016/j.jacep.2024.07.016. Epub 2024 Sep 25.
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Deep Learning-Based Electrocardiogram Analysis Predicts Biventricular Dysfunction and Dilation in Congenital Heart Disease.基于深度学习的心电图分析预测先天性心脏病的双心室功能障碍和扩张。
J Am Coll Cardiol. 2024 Aug 27;84(9):815-828. doi: 10.1016/j.jacc.2024.05.062.
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Can J Cardiol. 2024 Dec;40(12):2461-2472. doi: 10.1016/j.cjca.2024.06.009. Epub 2024 Jun 16.
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