手术修复肺动脉瓣缺失综合征后的结果:瑞典三级转诊中心30年的经验。

IF 1.6 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Vasileios Avdikos, Jens Johansson Ramgren, Katarina Hanséus, Torsten Malm, Petru Liuba
{"title":"手术修复肺动脉瓣缺失综合征后的结果:瑞典三级转诊中心30年的经验。","authors":"Vasileios Avdikos,&nbsp;Jens Johansson Ramgren,&nbsp;Katarina Hanséus,&nbsp;Torsten Malm,&nbsp;Petru Liuba","doi":"10.1093/icvts/ivac193","DOIUrl":null,"url":null,"abstract":"<p><strong>Objectives: </strong>Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable.</p><p><strong>Methods: </strong>We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018.</p><p><strong>Results: </strong>Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency.</p><p><strong>Conclusions: </strong>The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.</p>","PeriodicalId":13621,"journal":{"name":"Interactive cardiovascular and thoracic surgery","volume":null,"pages":null},"PeriodicalIF":1.6000,"publicationDate":"2022-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9341845/pdf/","citationCount":"1","resultStr":"{\"title\":\"Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre.\",\"authors\":\"Vasileios Avdikos,&nbsp;Jens Johansson Ramgren,&nbsp;Katarina Hanséus,&nbsp;Torsten Malm,&nbsp;Petru Liuba\",\"doi\":\"10.1093/icvts/ivac193\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Objectives: </strong>Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable.</p><p><strong>Methods: </strong>We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018.</p><p><strong>Results: </strong>Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency.</p><p><strong>Conclusions: </strong>The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.</p>\",\"PeriodicalId\":13621,\"journal\":{\"name\":\"Interactive cardiovascular and thoracic surgery\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":1.6000,\"publicationDate\":\"2022-07-09\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9341845/pdf/\",\"citationCount\":\"1\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Interactive cardiovascular and thoracic surgery\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1093/icvts/ivac193\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"CARDIAC & CARDIOVASCULAR SYSTEMS\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Interactive cardiovascular and thoracic surgery","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1093/icvts/ivac193","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 1

摘要

目的:无肺动脉瓣综合征是一种罕见的先天性心脏缺损,伴有肺动脉扩张和继发性气道压迫。虽然术前呼吸支持和肺动脉成形术的早期手术修复通常需要气道受损的患者,但这些患者是否需要广泛的成形术,以及一般没有或轻度呼吸问题的患者是否需要成形术,仍然存在争议。方法:我们对1988年至2018年诊断并修复的患者进行回顾性调查。结果:确定了20例患者。修复时的中位年龄和体重分别为0.8(0.1-2.4)岁和7.0 (2.5-13.8)kg,包括17例(85%)患者的带瓣导管和3例患者的经环补片。5例(29%)患者在0.3(0.1-0.4)岁时进行修复前依赖呼吸机。7例患者(35%)行肺动脉成形术,其中5例患者依赖呼吸机,2例患者因复发性感染而出现呼吸道症状。术前依赖呼吸机的2例患者(10%)接受了广泛的门内动脉成形术。术前呼吸机依赖与早期修复和再干预相关(P结论:该罕见缺损患者的长期预后良好,与其他既往研究相当。肺动脉复位成形术,在本研究中仅用于呼吸窘迫和呼吸机依赖的患者,与良好的生存率相关。再干预在这些患者中很常见。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre.

Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre.

Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre.

Objectives: Absent pulmonary valve syndrome is a rare congenital heart defect with pulmonary artery dilatation and secondary airway compression. Although preoperative respiratory support and early surgical repair with pulmonary arterioplasty are often required in patients with airway compromise, the need for extensive plasty in these patients and for plasty in general in those with no or mild respiratory issues remains debatable.

Methods: We performed a retrospective survey of patients with this diagnosis and repair from 1988 to 2018.

Results: Twenty patients were identified. The median age and weight at repair were 0.8 (0.1-2.4) years and 7.0 (2.5-13.8) kg and included a valved conduit in 17 (85%) patients and a transannular patch in 3 patients. Five (29%) patients were ventilator-dependent prior to repair at the age of 0.3 (0.1-0.4) years. Pulmonary arterioplasty was performed in 7 patients (35%), including all 5 with ventilator dependency and 2 with respiratory symptoms due to recurrent infections. Two patients (10%) with preoperative ventilator dependency underwent extensive intrahilar arterioplasty. Preoperative ventilator dependency was associated with earlier repair and reinterventions (P < 0.05). There were 3 late deaths among cases with repair after 2000 (n = 14), none with preoperative ventilator dependency.

Conclusions: The long-term outcomes of patients with this rare defect are good, comparable to those of other previous studies. Reduction pulmonary arterioplasty, which in this study was used only in patients with respiratory distress and ventilator dependency, is associated with excellent survival. Reinterventions are common in these patients.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Interactive cardiovascular and thoracic surgery
Interactive cardiovascular and thoracic surgery CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
3.30
自引率
0.00%
发文量
292
审稿时长
2-4 weeks
期刊介绍: Interactive CardioVascular and Thoracic Surgery (ICVTS) publishes scientific contributions in the field of cardiovascular and thoracic surgery, covering all aspects of surgery of the heart, vessels and the chest. The journal publishes a range of article types including: Best Evidence Topics; Brief Communications; Case Reports; Original Articles; State-of-the-Art; Work in Progress Report.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信