胎儿永存动脉干29例临床病理分析

Persistent truncus arteriosus in fetus:a clinicopathological analysis of 27 cases

目的探讨胎儿永存动脉干(PTA)的病理分型、鉴别诊断及伴发畸形。方法选取27例PTA,对其临床资料、B超、病理解剖结果进行分析,并复习相关文献进行讨论。结果根据Van Praagh分类法进行分型,29例PTA中A1型14例,A2型10例,A3型3例,A4型2例;合并心内畸形及变异16例,合并心外畸形19例。29例中有16例伴有室间隔缺损,7例单心室、单心房,4例伴有房室间隔缺损。结论永存动脉干是一种罕见心脏畸形,常常伴有心内及心外畸形。胎儿PTA类型以A1型较多见,合并心内畸形与类型有一定关系。

Objective To evaluate the value of pathological types in the diagnosis of persistent truncus arteriosus(PTA),differential diagnosis and accompanied malformations.Methods We retrospectively reviewed 27 patients with PTA,their data of clinical history,autopsy findings and pathologica diagnosis were studied,with review of literature.Results According to Van Praagh classification,these 29 PAC patients included 14 cases of type A1,10 cases of type A2,3 cases of type A3,2 cases of type A4.16 cases of PTA were accompanied with cardiaca anomalies and 19 cases were accompanied with extracardial malformations.The most common associated intracardiac malformation was ventricular septal defects in 16 cases,7 cases had arterial septal defect and single atrium,6 cases had atrioventricular septal defect.Conclusion PTA is rare cardiaca anomalies accompanied by intracardiac malformations and extracardial malformations.A1 type has high incidence in fetus.Accompanied cardiaca anomalies is certainly related to diffenent types.