根据第六对主动脉弓发育评价合并室间隔缺损的肺动脉闭锁形态学变化规律

Morphological analysis of pulmonary atresia with ventricular septal defect based on embryologic development of the sixth aortic arch

目的:根据第六对主动脉发育理论分析合并室间隔缺损的肺动脉闭锁患者的病理分型。方法:回顾74例合并室间隔缺损肺动脉闭锁患者的影像学特点,根据胚胎学第六对主动脉发育理论,分析其不同程度的发育异常导致的形态学变化并探讨新的肺动脉闭锁分类方法。结果:根据是否合并动脉导管将合并室间隔缺损的肺动脉闭锁患者分为两类,其中以动脉导管作为最主要供血途径的34例,肺血流灌注有三种形式:①动脉导管连接左右肺动脉融合部,供应全部肺血29例;②双侧动脉导管存在,分别连接同侧肺门部肺动脉,供应全部肺血,无肺动脉融合部患者2例;③一侧动脉导管连接同侧肺门部肺动脉,供应同侧肺血,另一侧肺组织由体肺侧枝供血患者2例。当动脉导管未发育体肺侧枝成为最主要供血途径,肺血灌注有两种形式:①体肺侧枝可连接固有肺动脉供应部分肺组织19例;②体肺侧枝供应全部肺组织22例。结论:根据第六对主动脉弓发育情况对合并室间隔缺损的肺动脉闭锁进行分类,较经典的肺动脉闭锁分类方法涵盖更多的解剖学变异,并对治疗方案选择提供必要的理论支持。

Objective: Analysis of embryogenesis and pathological classification of pulmonary atresia with ventricular septal defect. Methods: We reviewed imaging data of 74 patients with pulmonary atresia with ventricular septal defect,analyzed the types of pulmonary vascular blood supply and discussed a new classification with reference to embryologic change. Results: Depending on whether the ductus arteriosus( DA) present the patients were broadly classified into two types. When DA was present,the blood flow to the lung arose from one of three possible sources as follows: DA connected to confluence of pulmonary arteries in 29 cases;bilateral DA connected to ipsilateral pulmonary artery without confluent pulmonary arteries in 2 cases;unilateral DA connected to ipsilateral pulmonary artery,the contralateral lung received blood flow through aortopulmonary collaterals in 2 cases. When DA was unpresent,pulmonary blood perfusion was exclusively provided by aortopulmonary collaterals. Collaterals connected to true pulmonary artery in 19 cases and perfuse intrapulmonary arteries directly in 22 cases. Conclusions: Pulmonary artery atresia with ventricular septal defect can be classified according to the development the sixth pair of aortic arch. It can cover most of the anatomical variations and provide necessary theoretical support for the selection of treatment options.