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病例报告:动脉导管未闭合并肺动脉高压患者的血管反应性测试无反应:还有扭转局面的办法吗?

Case Report: Non-reactive vasoreactivity testing in a patient with patent ductus arteriosus with pulmonary hypertension: is there still a way to turn things around?

作者信息

Siagian Sisca Natalia, Panjaitan Elsa Hedia, Nugroho Pandu Prasetyo

机构信息

Division of Pediatric Cardiology and Congenital Heart Disease, Department of Cardiology and Vascular Medicine, Faculty of Medicine Universitas Indonesia/National Cardiovascular Centre Harapan Kita, Jakarta, Indonesia.

Faculty of Medicine Universitas Indonesia, Jakarta, Indonesia.

出版信息

Front Cardiovasc Med. 2025 Aug 5;12:1569963. doi: 10.3389/fcvm.2025.1569963. eCollection 2025.

DOI:10.3389/fcvm.2025.1569963
PMID:40837243
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12361252/
Abstract

BACKGROUND

Recent guidelines recommend patent ductus arteriosus (PDA) closure in adults based on hemodynamic criteria, such as pulmonary vascular resistance (PVR) and flow ratio (Qp:Qs). However, additional parameters like acute vasoreactivity testing (AVT) and closure testing, though lacking extensive data, may assist in identifying patients eligible for closure. We present the case of an adult patient with PDA and pulmonary hypertension (PH) whosuccessfully underwent transcatheter device closure guided by AVT and closure testing.

CASE PRESENTATION

A 35-year-old female presented with a two-year history of shortness of breath, cyanosis, and peripheral edema. She had been diagnosed with PDA at birth but did not undergo ligation due to parental refusal, leading to a 33-year loss of follow-up. Echocardiography revealed a bidirectional shunt through the PDA. Following cardiac catheterization (Qp:Qs 1.38, PVR: 21.5 WU) with AVT, the patient was diagnosed with PDA, PH with low flow, high resistance, and non-reactive to vasoreactivity test. She was prescribed sildenafil and discharged. After one year, the patient reported symptom improvement, with repeat catheterization showing a slight reduction in Qp:Qs (1.25) and PVR (16.38 WU), though values remained above the guideline cut-off for closure. However, the patient was then reactive to AVT, so we decided to perform device closure and observed the patient for 10 min before releasing the device. The patient was stable following the procedure and recovered well. One month later, the patient experienced significant symptom relief and could engage in moderate physical activity without discomfort.

CONCLUSION

This case highlights the potential AVT, closure testing, and also the treat-and-repair strategy with sildenafil to expand the window of operability in adult PDA patients with PH. Further research especially on long-term outcomes, is recommended.

摘要

背景

近期指南建议根据血流动力学标准,如肺血管阻力(PVR)和分流比(Qp:Qs),对成人动脉导管未闭(PDA)进行封堵。然而,诸如急性血管反应性测试(AVT)和封堵测试等其他参数,尽管缺乏广泛的数据,但可能有助于识别适合封堵的患者。我们报告一例患有PDA和肺动脉高压(PH)的成年患者,其在AVT和封堵测试的引导下成功接受了经导管装置封堵术。

病例介绍

一名35岁女性,有两年气短、发绀和外周水肿病史。她出生时被诊断为PDA,但因家长拒绝未行结扎术,导致33年失访。超声心动图显示通过PDA的双向分流。在进行心脏导管检查(Qp:Qs 1.38,PVR: 21.5 WU)并进行AVT后,患者被诊断为PDA、低流量、高阻力且对血管反应性测试无反应的PH。她接受了西地那非治疗并出院。一年后,患者报告症状改善,再次导管检查显示Qp:Qs(1.25)和PVR(16.38 WU)略有降低,尽管这些值仍高于指南规定的封堵临界值。然而,患者随后对AVT有反应,因此我们决定进行装置封堵,并在释放装置前观察患者10分钟。术后患者情况稳定,恢复良好。一个月后,患者症状明显缓解,能够进行适度体力活动而无不适。

结论

本病例突出了AVT、封堵测试以及使用西地那非的治疗和修复策略在扩大成年PDA合并PH患者可手术窗口期方面的潜力。建议进一步开展研究,尤其是关于长期结局的研究。

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本文引用的文献

1
Feasibility of Treat and Repair Strategy in Congenital Heart Defects With Pulmonary Arterial Hypertension.先天性心脏病合并肺动脉高压的治疗与修复策略的可行性
JACC Adv. 2024 Mar 6;3(4):100887. doi: 10.1016/j.jacadv.2024.100887. eCollection 2024 Apr.
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Interventional Occlusion of Large Patent Ductus Arteriosus in Adults with Severe Pulmonary Hypertension.成人重度肺动脉高压患者大型动脉导管未闭的介入封堵术
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动脉导管未闭:儿科和成人心脏护理提供者的当代视角。
J Am Heart Assoc. 2022 Sep 6;11(17):e025784. doi: 10.1161/JAHA.122.025784. Epub 2022 Sep 3.
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Medications for the treatment of pulmonary arterial hypertension: a systematic review and network meta-analysis.肺动脉高压治疗药物:系统评价和网络荟萃分析。
Eur Respir Rev. 2022 Aug 10;31(165). doi: 10.1183/16000617.0036-2022. Print 2022 Sep 30.
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Post-interventional Evaluation and Follow-Up in Children With Patent Ductus Arteriosus Complicated With Moderate to Severe Pulmonary Arterial Hypertension: A Retrospective Study.动脉导管未闭合并中度至重度肺动脉高压患儿介入治疗后的评估与随访:一项回顾性研究
Front Cardiovasc Med. 2021 Nov 11;8:693414. doi: 10.3389/fcvm.2021.693414. eCollection 2021.
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Pulmonary Hypertension with Prolonged Patency of the Ductus Arteriosus in Preterm Infants.早产儿动脉导管持续开放合并肺动脉高压
Children (Basel). 2020 Sep 16;7(9):139. doi: 10.3390/children7090139.
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2020 ESC Guidelines for the management of adult congenital heart disease.2020年欧洲心脏病学会成人先天性心脏病管理指南。
Eur Heart J. 2021 Feb 11;42(6):563-645. doi: 10.1093/eurheartj/ehaa554.
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PDA: Does it matter?动脉导管未闭:有关系吗?
Int J Pediatr Adolesc Med. 2020 Mar;7(1):9-12. doi: 10.1016/j.ijpam.2019.12.001. Epub 2019 Dec 3.
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Is There Value in Repeating Inhaled Nitric Oxide Vasoreactivity Tests in Patients with Pulmonary Arterial Hypertension?肺动脉高压患者重复吸入性一氧化氮血管反应性测试是否有价值?
Lung. 2020 Feb;198(1):87-94. doi: 10.1007/s00408-019-00318-0. Epub 2020 Jan 1.
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