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Semin Thorac Cardiovasc Surg. 2023;35(4):733-743. doi: 10.1053/j.semtcvs.2022.07.011. Epub 2022 Aug 2.
2
Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review.肺动脉高压的诊断与治疗:综述
JAMA. 2022 Apr 12;327(14):1379-1391. doi: 10.1001/jama.2022.4402.
3
Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom.肺动脉高压治疗在 Fontan 循环患者中的应用:英国当前的实践情况。
J Am Heart Assoc. 2022 Jan 4;11(1):e023035. doi: 10.1161/JAHA.121.023035. Epub 2021 Dec 20.
4
Pulmonary vascular disease in Fontan circulation-is there a rationale for pulmonary vasodilator therapies?Fontan循环中的肺血管疾病——肺血管扩张剂治疗是否有理论依据?
Cardiovasc Diagn Ther. 2021 Aug;11(4):1111-1121. doi: 10.21037/cdt-20-431.
5
Pharmacokinetics of treprostinil in children with functional single-ventricle pulmonary arterial hypertension: a randomized controlled trial.曲前列尼尔在功能性单心室肺动脉高压患儿中的药代动力学:一项随机对照试验。
Ann Transl Med. 2021 Jul;9(14):1163. doi: 10.21037/atm-21-3188.
6
Perioperative and long-term management of Fontan patients.Fontan手术患者的围手术期及长期管理
Cardiol Young. 2021 May;31(5):775-785. doi: 10.1017/S1047951120004618. Epub 2021 Jan 12.
7
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。
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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
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9
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10
Use of subcutaneous treprostinil in pediatric pulmonary arterial hypertension-Bridge-to-transplant or long-term treatment?皮下注射曲前列尼尔在儿童肺动脉高压中的应用——过渡到移植还是长期治疗?
Pediatr Transplant. 2018 Mar;22(2). doi: 10.1111/petr.13106. Epub 2017 Dec 19.

标准化静脉注射曲前列尼尔对全腔静脉-肺动脉连接术后肺动脉高压患者的影响。

The effects of standardized intravenous treprostinil in pulmonary arterial hypertension patients after total cavo-pulmonary connection procedure.

作者信息

Wang Xiaofeng, Wang Shilin, Shen Ruihuan, Lu Zhongyuan, Wang Xu

机构信息

Department of Pediatric Intensive Care Unit, National Center for Cardiovascular Disease, Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100037, People's Republic of China.

Department of Cardiology, Beijing Hospital, National Center of Gerontology, Institute of Geriatric Medicine, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, People's Republic of China.

出版信息

Eur J Med Res. 2024 Oct 1;29(1):479. doi: 10.1186/s40001-024-02077-y.

DOI:10.1186/s40001-024-02077-y
PMID:39354532
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11445850/
Abstract

OBJECTIVE

Total cavo-pulmonary connection (TCPC) is a palliative treatment for single ventricular malformations. For high-risk patients (preoperative mean pulmonary arterial pressure, mPAP > 15 mmHg), between the inhaled and oral targeted medications, the application of intravenous treprostinil as a bridge therapy to achieve "seamless" management is core postoperative treatment. This study intends to explore the effect of different administration regimens on early postoperative recovery.

METHODS

This was a retrospective cohort study. High-risk pediatric patients (age ≤ 14 years) who underwent TCPC procedure in Fu Wai Hospital from 2015 to 2022 were included. Since the regimen of treprostinil was standardized in our center in 2021, the patients in 2020 and before were included in group 1, patients in 2021 and 2022 were included in group 2. The hemodynamic parameters were compared before and after the maintenance dose of treprostinil. The differences of demographic characteristics, surgical data and postoperative recovery were compared between the two groups.

RESULTS

A total of 51 pediatric patients were included. Group 1 included 35 patients who received treprostinil at 1-3 postoperative days and an average dose of 12 ± 4 ng/(kg·min). Group 2 included 16 patients who received treprostinil within postoperative 1 day and an average dose of 22 ± 7 ng/(kg·min). There were no significant differences between the two groups in terms of age, weight, preoperative percutaneous oxygen saturation and mPAP,  heterotaxy syndrome, TCPC procedure type, other concurrent procedure, cardiopulmonary bypass time and aortic cross-clamp proportion (p > 0.05). After 24 h of treprostinil treatment, the mPAP in group 1 reduced from 17 ± 3 mmHg to 15 ± 2 mmHg (p < 0.001), and in group 2 from 17 ± 2 mmHg to 14 ± 2 mmHg (p < 0.001), with no difference between groups. In the postoperative recovery, patients in Group 2 exhibited a reduced duration of mechanical ventilation, 19 (11, 25) hours vs 69 (23, 189) hours, p = 0.001; a shorter stay in the ICU, 8 (6, 12) days vs 16 (9,26) days, p = 0.006; and a shorter postoperative length of stay, 27 (17,55) days vs 39 (29,58) days, p = 0.032. Patients in Group 2 also exhibited a lower incidence of thromboembolic events, 0 (0/26) vs 26% (9/35), p = 0.043; and the need for renal replacement therapy, 0 (0/26) vs 31% (11/35), p = 0.011.

CONCLUSION

Treprostinil reduces pulmonary artery pressure after TCPC procedure. The standardized application of treprostinil may improve the postoperative recovery which should be proven by randomized controlled trials or matched cohort studies in the future.

摘要

目的

全腔静脉-肺动脉连接术(TCPC)是一种用于单心室畸形的姑息性治疗方法。对于高危患者(术前平均肺动脉压,mPAP>15 mmHg),在吸入和口服靶向药物之间,应用静脉注射曲前列尼尔作为桥接治疗以实现“无缝”管理是术后核心治疗。本研究旨在探讨不同给药方案对术后早期恢复的影响。

方法

这是一项回顾性队列研究。纳入2015年至2022年在阜外医院接受TCPC手术的高危儿科患者(年龄≤14岁)。由于曲前列尼尔的给药方案于2021年在我们中心实现标准化,2020年及以前的患者纳入第1组,2021年和2022年的患者纳入第2组。比较曲前列尼尔维持剂量前后的血流动力学参数。比较两组患者的人口统计学特征、手术数据和术后恢复情况。

结果

共纳入51例儿科患者。第1组包括35例患者,在术后1 - 3天接受曲前列尼尔治疗,平均剂量为12±4 ng/(kg·min)。第2组包括16例患者,在术后1天内接受曲前列尼尔治疗,平均剂量为22±7 ng/(kg·min)。两组在年龄、体重、术前经皮血氧饱和度和mPAP、心脏异位综合征、TCPC手术类型、其他同期手术、体外循环时间和主动脉阻断比例方面无显著差异(p>0.05)。曲前列尼尔治疗24小时后,第1组的mPAP从17±3 mmHg降至15±2 mmHg(p<0.001),第2组从17±2 mmHg降至14±2 mmHg(p<0.001),两组之间无差异。在术后恢复方面,第2组患者机械通气时间缩短,为19(11,25)小时,而第1组为69(23,189)小时,p = 0.001;在ICU的停留时间缩短,为8(6,12)天,而第1组为16(9,26)天,p = 0.006;术后住院时间缩短,为27(17,55)天,而第1组为39(29,58)天,p = 0.032。第2组患者血栓栓塞事件的发生率也较低,为0(0/26),而第1组为26%(9/35),p = 0.043;肾脏替代治疗的需求也较低,为0(0/26),而第1组为31%(11/35),p = 0.011。

结论

曲前列尼尔可降低TCPC术后的肺动脉压力。曲前列尼尔的标准化应用可能改善术后恢复情况,未来应通过随机对照试验或匹配队列研究加以证实。