内脏心房异位综合征的电子束CT诊断

目的 探讨电子束CT（EBCT）在内脏心房异位综合征系统性诊断中的价值。方法 对10例内脏心房异位综合征病例进行回顾性分析。患儿年龄从7个月至17岁不等，平均9.5岁。EBCT增强单层和连续容积扫描各5例。范围自胸廓入口至中上腹。所有病例均行超声心动图和心血管造影，6例行手术。结果 （1）EBCT诊断右房异构8例，双侧形态学右房耳、右叶性对称肝、双侧动脉上支气管、双右肺及完全性肺静脉异常连接 均清晰显示，其中7例提示心内膜垫缺损、肺动脉狭窄并主动脉右弓右降，6例见右室双出口，2例见左侧膈疝。上腹均未见脾。（2）EBCT诊断左房异构2例，均见双侧形态学左房耳、左叶性对称肝、双侧动脉下支气管、双左肺、右室双出口、肺动脉狭窄、下腔静脉肝段缺如及主动脉右弓右降，1例见心内膜垫缺损。2例右上腹均见多个脾块。（3）EBCT检出异常数为116个，而心血管造影和超声分别为65和43个。6例手术所见与EBCT一致。结论 EBCT能够良好显示心脏大血管各节段的形态和连接，在对内脏心房异位综合征诊断治疗密切相关的心房形态、肺静脉异常连接的部位、肺动脉发育情况以及其他胸腹脏器异常的检出方面，具有明显优势，是1种重要的系统性诊断内脏心房异位综合征的方法。

Electron-beam CT diagnosis of the visceroatrial heterotaxy syndrome

Objetives To assess the usefulness of electron beam CT (EBCT) in diagnosis of the visceroatrial heterotaxy syndrome. Methods Ten patients with the visceroatrial heterotaxy syndrome were evaluated. The patients ranged in age from 7 months to 17 years (averaged 9.5 years). Five of the patients underwent EBCT contrast single slice mode while another 5 patients did continuous volume scan obtained from the superior aperture of thorax to the middle part of abdomen. All the patients had both angiocardiogram and echocardiogram, and 6 patients had operative outcomes. Results (1)Eight patients with right atrial isomerism, bilaterally morphologic right atrial appendages, right lobe dominant symmetric liver, bilaterally epiarterial bronchi, trilobed lungs, and total anomalous pulmonary venous connectionl were clearly detected. Endocardial cushions defect, pulmonary stenosis, right sided aortic arch and descending aorta were documented in 7 patients. Double outlet of right ventricle was imaged in 6 patients and hiatal hernia in 2 patients. Neither a spleen nor splenules were found. (2) Two patients with left atrial isomerism, bilaterally morphologic left atrial appendages, left lobe dominant symmetric liver, bilaterally hyparterial bronchi, bilobed lungs, double outlet of right ventricle, pulmonary stenosis, interruption of inferior vena cave, right sided aortic arch, and descending aorta were documented. Endocardial cushions defect was detected in 1 patient. Multiple spleens were demonstrated in the right upper abdomen in the 2 patients.(3) The number of abnormal obervasions detected by EBCT was 116,while that done by angiocardiogram and echocardiogram were 65 and 43 respectively. Conclusion EBCT is a usefu1 tool in the evaluation of patients with the syndrome of visceroatrial heterotaxia.