摘要
目的:探讨单侧肺动脉缺如(UAPA)胚胎学发生机制及病理分型。方法:回顾7例UAPA患者的心导管造影及心脏CT检查结合胚胎学理论,分析不同影像学表现的UAPA的发生机制。结果:7例患者中,5例为左肺动脉缺如,2例为右肺动脉缺如,1例患者降主动脉发出2条粗大体肺侧枝供应患侧肺,6例患者支气管动脉、肋间动脉及胸廓内动脉发出细小体肺侧枝供应患侧肺。结论:当一侧第六对血管弓的近端未发育或早期退化,动脉导管与肺门部肺动脉直接相连,出生后若动脉导管闭塞临床上表现为UAPA;当一侧第六对血管弓的近端及远端均均未发育,粗大体肺侧枝形成供应患侧肺血供。此类UAPA极为罕见,本病例是国内外文献的首次报道。
Objective： To discuss the pathological type and embryological mechanism of unilateral absence of pulmonary artery（ UAPA）. Methods： Retrospectively analyze imaging data of 7 patients with UAPA,combined with embryology theory to analyze the embryological mechanism of patients with different imaging characteristics. Results： Five of the seven patients had absence of the left main pulmonary artery,whilst the remaining two patients had absence of the right main pulmonary artery. The perfusion of the affected lung arise from two major aortopulmonary collateral arteries（ MAPCAs） in one patient,from bronchial,intercostal,subdiaphragmatic and subclavian arteries in 6 patients. Conclusion： When ipsilateral proximal sixth arch is failure to develop or involution,the distal sixth arch persists and connects the pulmonary artery to the aortic arch,if the DA closed after birth,the affected lung lose its source of blood supply,small collaterals can develop to increase systemic-to-pulmonary blood ow. When ipsilateral proximal and distal sixth aortic arch are failure to develop or involution in early gestation,the primitive vessels persist after birth and function as MAPCAs for the affected lung.
作者
陈国良
靳永强
张福强
吴清玉
CHEN Guoliang;JIN Yongqiang;ZHANG Fuqiang;WU Qingyu(Heart Center,First Hospital of Tsinghua University,Beijing 100016,China)
出处
《心肺血管病杂志》
2018年第9期862-865,共4页
Journal of Cardiovascular and Pulmonary Diseases
关键词
单侧肺动脉缺如
动脉导管未闭
第六对主动脉弓
Unilateral absence of pulmonary artery
Ductus arteriosus
The sixth aortic arch
