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本文引用的文献

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The long-term effects of the fenestration in patients with extracardiac Fontan circulation-a multicenter Korean cohort study based on national Fontan registry.心外膜Fontan循环患者开窗术的长期影响——一项基于韩国全国Fontan登记处的多中心队列研究
Front Cardiovasc Med. 2024 May 7;11:1341882. doi: 10.3389/fcvm.2024.1341882. eCollection 2024.
2
Pregnancy Outcomes in Women after the Fontan Procedure.Fontan手术术后女性的妊娠结局
J Clin Med. 2023 Jan 18;12(3):783. doi: 10.3390/jcm12030783.
3
Forty-year survival after Glenn procedure without Fontan procedure in patients with single ventricle.Glenn 手术后 40 年无 Fontan 手术患者的单心室生存。
Eur J Cardiothorac Surg. 2023 Feb 3;63(2). doi: 10.1093/ejcts/ezac528.
4
Fontan operation at less than 3 years of age is not a risk factor for long-term failure.Fontan 手术在 3 岁以下进行不是远期失败的危险因素。
Eur J Cardiothorac Surg. 2022 Feb 18;61(3):497-504. doi: 10.1093/ejcts/ezab355.
5
Utility of late pulmonary artery banding in single-ventricle physiology: A mid-term follow-up.晚期肺动脉环扎术在单心室生理中的应用:中期随访
Ann Pediatr Cardiol. 2021 Jan-Mar;14(1):26-34. doi: 10.4103/apc.APC_128_20. Epub 2020 Sep 17.
6
Pulmonary artery banding in patients with functional single ventricle associated with pulmonary hypertension.肺动脉环缩术治疗伴有肺动脉高压的功能性单心室患者。
Clin Exp Hypertens. 2021 May 19;43(4):328-333. doi: 10.1080/10641963.2021.1883048. Epub 2021 Feb 5.
7
Protein-losing enteropathy and plastic bronchitis after the Fontan procedure.法洛四联症根治术后发生蛋白丢失性肠病和假性支气管扩张症。
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8
Pulmonary Vasodilator Therapy in Children with Single Ventricle Physiology: Effects on Saturation and Pulmonary Arterial Pressure.单心室生理患儿的肺血管扩张剂治疗:对血氧饱和度和肺动脉压的影响
Pediatr Cardiol. 2020 Dec;41(8):1651-1659. doi: 10.1007/s00246-020-02424-w. Epub 2020 Jul 30.
9
Global, regional, and national burden of congenital heart disease, 1990-2017: a systematic analysis for the Global Burden of Disease Study 2017.全球、地区和国家先天性心脏病负担,1990-2017 年:2017 年全球疾病负担研究的系统分析。
Lancet Child Adolesc Health. 2020 Mar;4(3):185-200. doi: 10.1016/S2352-4642(19)30402-X. Epub 2020 Jan 21.
10
Fontan with lateral tunnel is associated with improved survival compared with extracardiac conduit.与体外管道相比,外侧隧道 Fontan 术与改善生存相关。
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单心室延迟出现:手术选择

The single ventricle presenting late: surgical options.

作者信息

Talwar Sachin, Marathe Supreet, Desai Manan, Kisku Navnita, Bhende Vishal Vinayak

机构信息

Cardiothoracic Centre, All India Institute of Medical Sciences, Ansari Nagar (East), New Delhi, 110029 India.

Department of Cardiothoracic & Vascular Surgery, All India Institute of Medical Sciences, Ansari Nagar (East), New Delhi, 110029 India.

出版信息

Indian J Thorac Cardiovasc Surg. 2025 Jun;41(6):745-755. doi: 10.1007/s12055-025-01948-1. Epub 2025 May 3.

DOI:10.1007/s12055-025-01948-1
PMID:40417596
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12102410/
Abstract

The term single ventricle (SV) comprises a wide variety of congenital anomalies with a structurally or functionally univentricular heart where a biventricular repair is not possible or undesirable. The surgical management of these patients is often staged and the ultimate palliation is the Fontan operation (FO). Despite advances in surgical techniques, significant challenges persist in the management of SV patients, particularly in low- and middle-income countries (LMICs), where delayed presentation and limited healthcare access are common. This review explores current management strategies for patients beyond childhood, including those who have never undergone palliation and post-Fontan patients facing long-term complications. Key topics include the surgical approaches to SV, such as pulmonary artery banding (PAB), bidirectional Glenn (BDG), and FO, with particular focus on considerations for late-presenting adults in LMICs. The disparities in healthcare resources between high-income countries and LMICs are highlighted, demonstrating that resource constraints significantly impact timely interventions and on-going follow-up care.

摘要

单心室(SV)这一术语涵盖了多种先天性异常,其心脏结构或功能为单心室,无法或不宜进行双心室修复。这些患者的手术治疗通常分阶段进行,最终的姑息治疗是Fontan手术(FO)。尽管手术技术有所进步,但在单心室患者的管理方面,尤其是在低收入和中等收入国家(LMICs),仍然存在重大挑战,这些国家常见就诊延迟和医疗服务可及性有限的情况。本综述探讨了儿童期以后患者的当前管理策略,包括那些从未接受过姑息治疗的患者以及面临长期并发症的Fontan术后患者。关键主题包括单心室的手术方法,如肺动脉环扎术(PAB)、双向格林分流术(BDG)和Fontan手术,特别关注低收入和中等收入国家成年晚期患者的注意事项。强调了高收入国家和低收入及中等收入国家在医疗资源方面的差异,表明资源限制对及时干预和持续的随访护理有重大影响。