肺动脉起源于动脉导管远端的影像学特点分析

Morphological analysis to distal ductal origin of pulmonary artery

目的:总结肺动脉起源于动脉导管远端的先天性心脏病影像学特点并探讨其形成机制。方法:回顾16例符合肺动脉起源于动脉导管远端的先天性心脏病患者的影像学资料结合胚胎学理论,总结、分析其形态学特点。结果:一侧肺动脉起自主肺动脉,对侧肺动脉缺如5例;一侧肺动脉起自主肺动脉,对侧肺动脉起源于动脉导管远端2例;一侧肺动脉起源于动脉导管远端,对侧肺组织由粗大体肺侧枝供应2例;双侧肺动脉分别起源于同侧动脉导管远端5例;一侧肺动脉起源于动脉导管远端,对侧肺动脉缺如1例;一侧肺动脉起源于动脉导管远端,对侧肺动脉连接分流管道1例。结论:肺动脉起源于动脉导管的先天性心脏病的影像学表现多样,其病理解剖特点与第六对主动脉弓发育异常的范围、出生后导管是否闭合及合并畸形相关。

Objective: To identify the morphological characteristics of distal ductal origin of pulmonary artery and discuss the possible pathogenesis based on the embryologic theory.Methods: We reviewed imaging data of 16 patients diagnosed as distal ductal origin of pulmonary artery from June 2011 to November 2018. Results: Unilateral absence of pulmonary artery and the contralateral pulmonary artery originating from main pulmonary artery was diagnosed in 5 patients. Distal ductal origin of left pulmonary artery and right pulmonary artery originating from main pulmonary artery in 2 patients with tetralogy of Fallot. Distal ductal origin of one pulmonary artery and the contralateral lung provided by aortopulmonary collaterals was identified in 2 patients. Bilateral distal ductal origin of the pulmonary arteries was identified in 5 patients with pulmonary atresia. Distal ductal origin of right pulmonary artery and absence of left pulmonary artery was identified in 1 patient with pulmonary atresia. Distal ductal origin of right pulmonary artery and left pulmonary artery was supplied through the BT shunt in 1 patient. Conclusions: Distal ductal origin of the right or/and left pulmonary artery reflected absence of the proximal right or/and left sixth aortic arch respectively during the embryologic development. The extent of the sixth aortic arch maldevelopment,the closure of the ductus arteriosus and the intracardiac malformation determined the clinical manifestation and imaging performance.