Ⅳ型肺动脉闭锁合并室间隔缺损患儿临床、手术预后及CT特点(附7例报道)

Clinical Characteristics and CT Manifestations of Type Ⅳ Pulmonary Atresia and Ventricular Septal Defect (PA/VSD) in Children: A Report of 7 Cases

目的探讨Ⅳ型肺动脉闭锁合并室间隔缺损(PA/VSD)患儿的临床、手术预后及CT特点。方法回顾性分析7例Ⅳ型PA/VSD患儿的临床及CT检查资料,记录Ⅳ型PA/VSD影像表现、侧支情况、供应肺段及合并其他畸形情况。结果心脏CT三维重组图像可清晰显示Ⅳ型PA/VSD影像表现、侧支情况、侧支供应肺段,以及合并其他畸形。其中合并房间隔缺损4例,主动脉右弓右降4例,迷走左锁骨下动脉2例,冠状动脉起源异常1例,永存左上腔静脉1例,三房心1例。结论Ⅳ型PA/VSD是一种罕见的先天性心脏病,常伴其他心脏、大血管发育异常;心脏CT三维重组图像可直观、清晰显示患者的各种体肺侧支情况、供应肺段及其合并心血管解剖异常。

Objective To investigate the clinical, surgical prognosis and CT characteristics of children with type Ⅳ pulmonary atresia with ventricular septal defect. Methods The clinical and CT examination data of 7 children with type Ⅳ PA/VSD were retrospectively analyzed, and the imaging manifestations of type Ⅳ PA/VSD,collaterals, supply in glungsegments, and other malformations were recorded. Results The three-dimensional reconstruction image of cardiothoracic CT can clearly show the imaging performance of type Ⅳ PA/VSD,the condition of collaterals, the supply of lung segments by collaterals, and other malformations.Among them, 4 cases had atrial septal defect, 4 cases of right descending aortic arch, 2 cases of vagus left subclavian artery, 1 case of coronary artery origin, 1 case of permanent left superior vena cava, and 1 case of triatrial heart. Conclusion Type Ⅳ PA/VSD is a rare congenital heart disease often accompanied by other heart and large blood vessel development abnormalities;CT three-dimensional reconstruction images can directly and clearly show the patient’s various body and pulmonary collaterals supplying lung segments and other cardiovascular abnormalities simultaneously.