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法洛四联症、右位主动脉弓及迪格奥尔格综合征患儿左锁骨下动脉的分离。超声心动图诊断病例研究。

Isolation of the left subclavian artery in an infant with tetralogy of Fallot, right aortic arch and DiGeorge syndrome. Echocardiographic diagnostic case study.

作者信息

Karolczak Maciej A, Mądry Wojciech, Grégoire Darren James

机构信息

Department of Cardiac and General Pediatric Surgery, Medical University of Warsaw , Warsaw , Poland.

出版信息

J Ultrason. 2019;19(76):66-70. doi: 10.15557/JoU.2019.0010.

Abstract

We present a case of a 6-month-old infant with an isolated left subclavian artery coexistent with right-sided aortic arch, tetralogy of Fallot and DiGeorge syndrome, with an emphasis on echocardiographic detection of this extremely rare anomaly. Specific difficulties related to echocardiographic visualization of abnormally coursing artery were a result of significantly limited ultrasonographic access due to the absence of thymus and a very close proximity of the left subclavian artery and left common carotid artery, mimicking a normal brachiocephalic trunk, which is usually present in patients with right-sided aortic arch. Precise analysis of the course of carotid and vertebral arteries as well as the nature and direction of flow in these vessels (particularly in the left vertebral and subclavian artery) suggested ductal rather than aortic origin of the left subclavian artery. Precise delineation of anatomical relationships between major arteries prior to surgical closure of the arterial duct was necessary to prevent potential postoperative ischemia of the left upper extremity; therefore the diagnosis was completed with CT angiography. We present a case of a 6-month-old infant with an isolated left subclavian artery coexistent with right-sided aortic arch, tetralogy of Fallot and DiGeorge syndrome, with an emphasis on echocardiographic detection of this extremely rare anomaly. Specific difficulties related to echocardiographic visualization of abnormally coursing artery were a result of significantly limited ultrasonographic access due to the absence of thymus and a very close proximity of the left subclavian artery and left common carotid artery, mimicking a normal brachiocephalic trunk, which is usually present in patients with right-sided aortic arch. Precise analysis of the course of carotid and vertebral arteries as well as the nature and direction of flow in these vessels (particularly in the left vertebral and subclavian artery) suggested ductal rather than aortic origin of the left subclavian artery. Precise delineation of anatomical relationships between major arteries prior to surgical closure of the arterial duct was necessary to prevent potential postoperative ischemia of the left upper extremity; therefore the diagnosis was completed with CT angiography.

摘要

我们报告一例6个月大的婴儿,其孤立性左锁骨下动脉与右侧主动脉弓、法洛四联症和DiGeorge综合征并存,重点是超声心动图对这种极其罕见异常的检测。由于胸腺缺失以及左锁骨下动脉与左颈总动脉非常靠近,模拟了通常见于右侧主动脉弓患者的正常头臂干,导致超声检查途径显著受限,这给异常走行动脉的超声心动图可视化带来了特殊困难。对颈动脉和椎动脉的走行以及这些血管内血流的性质和方向(特别是左椎动脉和锁骨下动脉)进行精确分析,提示左锁骨下动脉起源于动脉导管而非主动脉。在手术闭合动脉导管之前,精确描绘主要动脉之间的解剖关系对于预防术后左上肢潜在缺血是必要的;因此,通过CT血管造影完成了诊断。我们报告一例6个月大的婴儿,其孤立性左锁骨下动脉与右侧主动脉弓、法洛四联症和DiGeorge综合征并存,重点是超声心动图对这种极其罕见异常的检测。由于胸腺缺失以及左锁骨下动脉与左颈总动脉非常靠近,模拟了通常见于右侧主动脉弓患者的正常头臂干(此处英文重复,译文相同),导致超声检查途径显著受限,这给异常走行动脉的超声心动图可视化带来了特殊困难。对颈动脉和椎动脉的走行以及这些血管内血流的性质和方向(特别是左椎动脉和锁骨下动脉)进行精确分析,提示左锁骨下动脉起源于动脉导管而非主动脉。在手术闭合动脉导管之前,精确描绘主要动脉之间的解剖关系对于预防术后左上肢潜在缺血是必要的;因此,通过CT血管造影完成了诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a3af/6750180/a5e36c592d29/jou-19-76-066-g001.jpg

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