肺动脉异常起源于升主动脉的病理分型及胚胎发生机制探讨

Embryological Mechanism and Pathological Type of Anomalous Origin of Pulmonary Artery from Ascending Aorta

目的 肺动脉异常起源升主动脉的病理分型及胚胎发生机制探讨。方法 5例肺动脉异常起源于升主动脉 ,作心血管造影 (ACG)及磁共振 (MRI)检查。结果 5例病例中 3例为右肺动脉异常起源于升主动脉 ;2例为左肺动脉异常起源于升主动脉 ,4例属近端型 ,1例远端型。结论 肺动脉异常起源于升主动脉为少见的先天性心脏病 ,病理分型有二种 ,一种右肺动脉异常起源于升主动脉 ,占肺动脉异常起源升主动脉的绝大部分 ;另一种为左肺动脉异常起源升主动脉 ,此种类型少见 ,又可根据肺动脉起源离主动脉瓣及无名动脉的距离分近端型及远端型二种亚型。二种类型的胚胎发生学的解释不尽相同。肺动脉异常起源于升主动脉以前称为半永存动脉干 ,从胚胎角度讲与永存动脉干不同 ,故目前不提倡用半永存动脉干这个术语。

Objective To discuss the pathological type and embryological mechanism of anomalous origin of a pulmonary artery from ascending aorta.Methods Five cases of anomalous origin of a pulmonary artery from ascending aorta underwent angiocardiography and MRI.Results 3 cases are anomalous origin of right pulmonary artery from ascending aorta and 2 cases are anomalous origin of left pulmonary artery from ascending aorta.Conclusion Anomalous origin of a pulmonary artery from ascending aorta is a rare congenital heart disease.There are two types:anomalous origin of right pulmonary artery from ascending aorta and anomalous origin of left pulmonary artery from ascending aorta.It could be divided into two subtypes:proximal and distal type according to the origin of the pulmonary artery in the ascending aorta.Hemitrucus is not a right term for the anomalous origin of a pulmonary artery from ascending aorta.