肺血减少型先天性心脏病合并粗大体肺侧支的诊治进展  

Diagnosis and treatment status quo of major aortopulmonary collateral arteries in congenital heart disease with decreased pulmonary blood flow

作　　者：郎欢 程真莉[1] Lang Huan;Cheng Zheni(Department of Cardiology,Children's Hospital of Chongqing Medical University,National Clinical Research Center for Child Health and Disorders,Ministry of Education Key Laboratory of Child Development and Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院心内科,国家儿童健康与疾病临床医学研究中心,儿童发育疾病研究教育部重点实验室,儿科学重庆市重点实验室,重庆400014

出　　处：《国际儿科学杂志》2024年第2期95-98,共4页International Journal of Pediatrics

基　　金：重庆市技术创新与应用示范(社会民生类)一般项目(cstc2018jscx-msybX0041)。

摘　　要：肺血减少型先天性心脏病是以肺血流量减少为特征的先天性心脏病,常见临床类型包括法洛四联症、肺动脉闭锁、右室双出口及伴有肺动脉狭窄的其他复合畸形。由于缺乏右心室来源的肺血供应,肺血减少型先天性心脏病患儿常合并粗大体肺侧支(major aortopulmonary collateral arteries,MAPCAs)或动脉导管参与肺循环供血。研究发现MAPCAs是导致肺血减少型先天性心脏病外科根治术后机械通气时间及重症监护室滞留时间延长、并发症发生率及病死率增加的重要因素。因此,MAPCAs的围手术期治疗至关重要。该文总结了MAPCAs的分布与影响、诊断与评估及治疗方式的研究进展,为临床医师规范处理MAPCAs提供参考。The congenital heart disease with decreased pulmonary blood flow is one type of congenital heart disease characterized by reduced pulmonary blood flow.Common clinical types include tetralogy of Fallot,pulmonary atresia,double outlet right ventricle,and other complex malformations associated with pulmonary artery stenosis.Due to the lack of pulmonary blood supply from the right ventricle,children with this condition often have major aortopulmonary collateral arteries(MAPCAs)or the ductus arteriosus participating in pulmonary circulation for blood supply.Research has revealed that MAPCAs are important factors which cause the increase of mechanical ventilation time,intensive care unit stay time,complication rate and mortality after radical surgical treatment of congenital heart disease with decreased pulmonary blood flow.Therefore,the treatment of MAPCAs in perioperative period is crucial.This article summarizes the research progress of MAPCAs distribution and influence,diagnosis and evaluation,and treatment methods,to provide the reference for clinicians to standardize MAPCAs treatment.

关 键 词：粗大体肺侧支 介入封堵术 “一站式”治疗 

分 类 号：R725.4[医药卫生—儿科]