CT血管成像诊断先天性心脏病合并内脏异位

目的评价CTA在先天性心脏病合并内脏异位诊断中的价值。方法收集接受CTA检查并经3D后处理的20例先天性心脏病合并内脏异位的患者,观察其心内及心外异常结构。结果20例患者中,左心房异构3例(3/20,15.00%),左肺两叶、右肺三叶2例,双肺两叶1例;单心室2例,房间隔缺损2例,室间隔缺损1例;大动脉异位2例,肺动脉狭窄2例,肺动脉闭锁1例;永存左上腔静脉1例;下腔静脉肝段缺如1例。右心房异构17例(17/20,85.00%),均为双肺三叶;单心室9例,右心室双出口2例,心内膜垫缺损6例,房间隔缺损10例,卵圆孔未闭1例,室间隔缺损8例;大动脉异位14例,肺动脉狭窄16例,肺动脉闭锁1例;肺静脉异位引流6例;永存左上腔7例;下腔静脉肝段缺如2例。结论 CTA可很好地显示心脏、大血管及胸腹腔内的结构,对先天性心脏病合并内脏异位的临床诊断和手术方案的制定有至关重要的作用。

CTA in diagnosis of congenital heart disease and related heterotaxy syndrome

Objective To evaluate the value of CTA in assessing congenital heart disease and related heterotaxy syndrome. Methods A total of 20 patients with congenital heart disease and heterotexy syndrome underwent CTA and 3-dimensional reconstructions. And intracardiac and extracardiac abnormalities were assessed. Results Three cases were left isomerism, two of which were bi-lobed left lungs and trilobed right lungs, and another one was bi-lobed lungs. The most common associated cardiovascular anomalies included single ventricle (n=2), atrial septal defect (n=2), ventricular septal defect (n=1), transposition of the great arteries (n=2), pulmonary artery stenosis (n=2), atresia (n=1), persistent left superior vena (n=1), no hepatic segment of inferior vena cava (n=1). The rest 17 cases were right isomerism. All of them were trilobed lungs. Associated cardiovascular anomalies included single ventricle (n=9), double outlet of right ventricle (n=2), endocardial cushion defect (n=6), atrial septal defect (n=10), patent foramen ovale (n=1), ventricular septal defect (n=8), transposition of the great arteries (n=14), pulmonary artery stenosis (n=16), atresia (n=1), pulmonary venous drainage (n=6), persistent left superior vena (n=7), no hepatic segment of inferior vena cava (n=2). Conclusion CTA can clearly show the structure of heart, great vessels, chest and abdomen which is a useful tool to accurately assess congenital heart disease and related heterotaxy syndrome for medical or surgical management.