肺血减少型先天性心脏病研究进展  

Research progress on congenital heart diseases of diminutive pulmonary blood

作　　者：陈虹宇 吕铁伟[1] Chen Hongyu;Lyu Tiewei(Department of Cardiology,Children′s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical 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血管内科,儿童发育疾病研究教育部重点实验室,国家儿童健康与疾病临床医学研究中心,儿童发育重大疾病国家国际科技合作基地,儿科学重庆市重点实验室,400014

出　　处：《中华实用儿科临床杂志》2020年第13期1031-1034,共4页Chinese Journal of Applied Clinical Pediatrics

摘　　要：肺血减少型先天性心脏病是存在肺动脉狭窄或闭锁且以肺血流量减少为特征的先天性心脏病,因肺血流减少导致肺发育异常,同时伴随体肺侧支血管的形成和复杂的心脏解剖结构异常,因而导致患儿临床症状重、严重影响生活质量甚至生命健康,是临床上心脏畸形矫治的难点问题。而该病的病因及发病机制目前尚未阐明,临床处理方案及时机取决于具体疾病类型及体肺侧支血管情况。现就肺血减少型先天性心脏病的病因、发病机制及诊治进展作一综述。Congenital heart diseases of diminutive pulmonary blood are characterized by the decrease of pulmonary blood flow accompanied with pulmonary stenosis or atresia.Diminutive pulmonary blood leads to abnormal development of lung,the formation of major aorta pulmonary collateral arteries(MAPCAs)and a complex cardiac anatomy structure in children,thereby resulting in serious clinical symptoms,poor quality of life and even life-threatening conditions.Therefore,diminutive pulmonary blood is a thorny problem in the treatment of cardiac malformation.However,the etiology and pathogenesis of this disease have not been elucidated yet.The clinical treatment plan and time depend on the disease type and MAPCAs.In this paper,the etiology,pathogenesis,diagnosis and treatment progress of congenital heart diseases of diminutive pulmonary blood were reviewed.

关 键 词：肺血减少型先天性心脏病 儿童 发病机制 治疗 

分 类 号：R541.1[医药卫生—心血管疾病]