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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.

DOI:10.15557/JoU.2019.0010
PMID:31088014
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6750180/
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。

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本文引用的文献

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Right aortic arch with isolated left subclavian artery: a rare association with coarctation of the left pulmonary artery.右位主动脉弓伴孤立左锁骨下动脉:一种罕见的合并左肺动脉狭窄的情况。
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Isolated left subclavian artery arising from the main pulmonary artery.孤立的左锁骨下动脉起源于主肺动脉。
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Isolated Left Subclavian Artery, Complete Atrioventricular Block, and Tricuspid Atresia in a Neonate.
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Cardiol Young. 2017 Apr;27(3):613-616. doi: 10.1017/S1047951116001840. Epub 2016 Nov 7.
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Three Rare Anomalies of the Large Vessels in an Infant with Tetralogy of Fallot.一名法洛四联症婴儿的三大罕见大血管异常。
J Card Surg. 2016 Jul;31(7):461-3. doi: 10.1111/jocs.12770. Epub 2016 Jun 1.
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Anomalous origin of the left subclavian artery from the pulmonary artery.左锁骨下动脉起源于肺动脉异常。
Cardiol Young. 2014 Feb;24(1):134-5. doi: 10.1017/S1047951113000735.
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Subclavian and pulmonary artery steal phenomenon in a patient with isolated left subclavian artery and right aortic arch.孤立性左锁骨下动脉及右主动脉弓患者的锁骨下动脉及肺动脉盗血现象
J Clin Ultrasound. 2013 May;41(4):265-8. doi: 10.1002/jcu.21874. Epub 2012 Jan 12.
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Isolation of the left subclavian artery in a child with tetralogy of Fallot and right aortic arch.法洛四联症合并右位主动脉弓患儿左锁骨下动脉的分离
J Formos Med Assoc. 2005 Jun;104(6):418-20.
9
Isolation of the left subclavian artery in tetralogy of Fallot and bronchial anomalies.法洛四联症中左锁骨下动脉的分离及支气管异常
S Afr Med J. 1997 Feb;87 Suppl 1:C25-8.
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Congenital pulmonary steal phenomenon associated with tetralogy of Fallot, right aortic arch, and isolation of the left subclavian artery.法洛四联症、右位主动脉弓及左锁骨下动脉离断相关的先天性肺内分流现象
Pediatr Cardiol. 1997 Jan-Feb;18(1):57-60. doi: 10.1007/s002469900111.