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法洛四联症衰竭的经导管开窗术:我们的临床经验。

Transcatheter fenestration in Fontan failure: Our clinical experience.

作者信息

Duras Ensar, Siyar Balik Recep, Arslan Perver, Cilsal Erman, Sahin Murat, Cansaran Tanidir Ibrahim, Ergul Yakup, Guzeltas Alper

机构信息

Department of Pediatric Cardiology, University of Health Sciences, İstanbul Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, İstanbul, Türkiye.

出版信息

Turk Gogus Kalp Damar Cerrahisi Derg. 2025 Jul 21;33(3):288-300. doi: 10.5606/tgkdc.dergisi.2025.27159. eCollection 2025 Jul.

Abstract

BACKGROUND

The aim of our study was to evaluate the hemodynamic effects and clinical outcomes of transcatheter fenestration in patients who developed Fontan failure.

METHODS

Between January 2014 and December 2022, among a total of 187 patients undergoing the Fontan operation, 10 (6 males, 4 females; median age: 10.9 years; range, 4.2 to 23 years) who underwent transcatheter creation or dilation of a Fontan fenestration due to the development of Fontan failure were retrospectively analyzed. Demographic data, laboratory results, echocardiographic findings, and catheterization parameters of the patients were recorded. The patients were classified into two groups based on the timing of Fontan failure: those who underwent fenestration before hospital discharge constituted the early-stage group, while those who underwent fenestration after discharge were assigned to the late-stage group. These two groups were compared in terms of hemodynamic parameters, changes in oxygen saturation, and complication rates.

RESULTS

Transcatheter fenestration was performed in five patients due to Fontan failure in the early postoperative period, and in five patients in the late period. The median follow-up duration was 2.2 (range, 0.1 to 6) years. Following the procedure, pleural effusion regressed in four patients; median systemic venous pressure decreased by 3.4 (range, 2 to 9) mmHg, while median oxygen saturation dropped by 5.9% (range, 3 to 9%). Clinical improvement was observed in 70% of the patients within the first month. However, one patient died on postoperative Day 44 due to low cardiac output. Fenestration was performed in three patients with protein-losing enteropathy, and clinical improvement was achieved in all of them.

CONCLUSION

Transcatheter Fontan fenestration is a reliable therapeutic option for reducing systemic venous pressure and achieving hemodynamic improvement in symptomatic Fontan patients. Our study highlights the low complication rates and the potential of this procedure to yield favorable clinical outcomes. Transcatheter fenestration plays an important role in the management of high-risk Fontan patients.

摘要

背景

我们研究的目的是评估经导管开窗术对发生Fontan循环衰竭患者的血流动力学影响及临床结局。

方法

在2014年1月至2022年12月期间,在总共187例行Fontan手术的患者中,对10例(6例男性,4例女性;中位年龄:10.9岁;范围4.2至23岁)因发生Fontan循环衰竭而接受经导管创建或扩张Fontan开窗术的患者进行回顾性分析。记录患者的人口统计学数据、实验室检查结果、超声心动图检查结果和心导管检查参数。根据Fontan循环衰竭的时间将患者分为两组:在出院前接受开窗术的患者构成早期组,而出院后接受开窗术的患者被分配到晚期组。比较这两组的血流动力学参数、血氧饱和度变化和并发症发生率。

结果

5例患者因术后早期Fontan循环衰竭接受经导管开窗术,5例患者在晚期接受该手术。中位随访时间为2.2年(范围0.1至6年)。术后,4例患者胸腔积液消退;中位体静脉压下降3.4 mmHg(范围2至9 mmHg),而中位血氧饱和度下降5.9%(范围3至9%)。70%的患者在第一个月内临床症状改善。然而,1例患者在术后第44天因心输出量低死亡。3例蛋白丢失性肠病患者接受了开窗术,所有患者均实现了临床改善。

结论

经导管Fontan开窗术是降低有症状Fontan患者体静脉压并实现血流动力学改善的可靠治疗选择。我们的研究强调了该手术并发症发生率低以及产生良好临床结局的潜力。经导管开窗术在高危Fontan患者的管理中发挥着重要作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/af2e/12421547/3c8e8f419942/TJTCS-2025-33-3-288-300-F1.jpg

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