|Pseudocoarctation of the aorta associated with the anomalous origin of the left vertebral artery: a case report.|
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|PMID: 18525233 Owner: NLM Status: MEDLINE|
|Pseudocoarctation of the aorta is a rare congenital anomaly of the aortic arch, and it has been described as an elongation of the aortic arch with "kinking" at the level of the ligamentum arteriosum without a pressure gradient across the lesion. The treatment for this condition is controversial. We report here on an unusual case of pseudocoarctation of the aorta associated with the anomalous origin of the left vertebral artery and we include a review of the medical literature.|
|Jae Sung Son; Ki Bae Hong; David Chan Wook Chung|
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|Type: Case Reports; Journal Article; Review|
|Title: Korean journal of radiology : official journal of the Korean Radiological Society Volume: 9 ISSN: 1229-6929 ISO Abbreviation: Korean J Radiol Publication Date: 2008 May-Jun|
|Created Date: 2008-06-05 Completed Date: 2008-11-14 Revised Date: 2013-06-05|
Medline Journal Info:
|Nlm Unique ID: 100956096 Medline TA: Korean J Radiol Country: Korea (South)|
|Languages: eng Pagination: 283-5 Citation Subset: IM|
|Department of Pediatrics, Dankook University College of Medicine, Cheonan, Choongnam, Korea. firstname.lastname@example.org|
|APA/MLA Format Download EndNote Download BibTex|
Vertebral Artery / abnormalities*
Journal ID (nlm-ta): Korean J Radiol
Journal ID (publisher-id): KJR
Publisher: The Korean Radiological Society
Copyright © 2008 The Korean Radiological Society
Received Day: 30 Month: 7 Year: 2007
Accepted Day: 27 Month: 9 Year: 2007
Print publication date: Season: May–Jun Year: 2008
Electronic publication date: Day: 20 Month: 6 Year: 2008
Volume: 9 Issue: 3
First Page: 283 Last Page: 285
PubMed Id: 18525233
|Pseudocoarctation of the Aorta Associated with the Anomalous Origin of the Left Vertebral Artery: a Case Report|
|Jae Sung Son, MDA1|
|Ki Bae Hong, MDA1|
|David Chan Wook Chung, MDA1|
|Department of Pediatrics, Dankook University College of Medicine, Cheonan, Choongnam 330-714, Korea.
Address reprint requests to: Jae Sung Son, MD, Department of Pediatrics, Dankook University College of Medicine, San-29 Anseo-Dong, Cheonan, Choongnam 330-714, Korea. Tel: (8241) 550-3912, Fax: (8241) 550-3937, email@example.com
Pseudocoarctation of the aorta was first described by Dotter and Steinberg, and Souders and coworker in 1951 (1, 2). It is usually found by detecting a cardiac murmur or an abnormal superior mediastinal mass on the chest radiography. Cardiac catheterization and angiography are diagnostic for this condition. We report here on an unusual case of pseudocoarctation of the aorta associated with the anomalous origin of the left vertebral artery.
A 6-year-old boy was admitted for cardiac murmur and a tingling sensation of the lower extremities on exercise. Physical examination revealed normal pulses in all four extremities with a blood pressure of 130/60 mmHg in the right arm, 132/70 mmHg in the left arm, and 100/74 mmHg in the lower extremities on admission. A grade 3/6 harsh pansystolic murmur of the left sternal border was heard. The chest X-ray studies revealed a normal sized heart. There was no evidence of rib notching, mediastinal widening or mass, or a cervical aortic arch. Electrocardiogram showed no abnormal findings. Two-dimentional echocardiographic examination showed a small perimembranous ventricular septal defect and a suspicious coarctation of the aorta (the blood velocity at the isthmus: 2.8 m/sec). There was no left ventricular hypertrophy, aortic stenosis/regurgitation or bicuspid aortic valve. The subsequent multichannel helical computed tomographic (CT) scan revealed the follows (Figs. 1A-C): The aortic arch was kinked and elongated and the narrowest part measured approximately 7 mm. No collateral circulation was detected. The left vertebral artery arose directly from the aortic arch and the order of these vessels was as follows; right brachiocephalic, left common carotid, left subclavian and left vertebral artery. The isthmic portion of the descending thoracic aorta was not visualized to be adjacent to the spine, but rather ventral to it, and this was surrounded by aerated lung due to the elongated and kinked aortic arch. Retrograde cardiac catheterization revealed that the trans-pseudocoarctation pressure gradient was 20 mmHg. We thought the patient's symptoms were caused by dynamic aortic narrowing during exercise, although the resting pressure gradient was not significant; we decided to perform a surgical procedure. The elongated and mildly dilated arch was resected and we performed end-to-end anastomosis. The patient was discharged without any complications. The pre-operative symptoms disappeared after surgery.
Pseudocoarctation of the aorta is a relatively rare congenital anomaly that consists of elongation and kinking of the aortic arch and narrowing of the aortic isthmus without significant obstruction (1, 2).
The exact etiology of pseudocoarctation of the aorta is not currently known. It has been proposed that the embryologic cause of pseudocoarctation of the aorta is a failure of compression of the third through the seventh segments of the dorsal aortic roots and the fourth arch segment (3). The elongation of the arch frequently produces an increased distance between the origin of the left common carotid and the left subclavian artery. This case had an elongated and kinked aortic arch, but the distance from the left common carotid to the left subclavian artery was normal. So, the presently proposed assumption does not explain the whole situation.
In our case, there was an exceptional association of a pseudocoarctation and the anomalous origin of the left vertebral artery. Embryologically, the aberrant origin of the left vertebral artery directly from the aortic arch is due to persistence of the 8th intersegmental artery (4). Vorster et al. (5) noted that the vertebral artery arose from the aortic arch in 5% of cadavers. To the best of our knowledge, there have been no previous reports of pseudocoarctation of the aorta associated with the anomalous origin of the left vertebral artery. This report will be helpful for better understanding the exact etiology of pseudocoarctation of the aorta.
Cardiac catheterization and angiography provides a definitive diagnosis for this condition. Measuring the pressure gradient by cardiac catheterization is necessary for the diagnosis of this condition. Similarly, CT is very helpful, and the following findings are considered diagnostic of this congenital anomaly according to several case reports; 1) demonstration that the abnormal mass in the mediastinum is part of the aorta; 2) an unusually aortic arch high in the mediastinum; 3) visualization of the isthmic portion of the descending thoracic aorta that's not adjacent to the spine, but rather ventral to it, and this is surrounded by aerated lung; and 4) a more caudal origin of the subclavian artery (6, 7).
A variety of congenital heart defects have been reported in association with pseudocoarctation (1, 3, 8). Pseudocoarctation is sometimes combined with aneurysm and aneurysmal dilatation beyond the lesion.
Pseudocoarctation of the aorta has been described in the literature as a "benign" entity that warrants no specific therapy, but reports on aneurysm formation and rupture in patients with pseudocoarctation have caused some changes for its treatment strategy (3, 8, 9). Surgical treatment should be recommended for all the symptomatic patients and for the pseudocoarctation of the aorta that is associated with aneurysm formation, and close follow-up is required for the asymptomatic patients who are without any associated anomalies.
The present research was financed by research funds of Dankook University in 2006.
|1.||Dotter CT,Steinberg I. AngiocardiographyYear: 1951New YorkPaul B Hoeber181184|
|2.||Souders CR,Pearson CM,Adams HD. An aortic deformity simulating mediastinal tumor: a subclinical form of coarctationDis ChestYear: 195120354514840276|
|3.||Yang BZ. Pseudocoarctation of the aorta with aneurysm formation: case reportChin Med JYear: 20051181230123216117874|
|4.||Albayram S,Gailloud P,Wasserman BA. Bilateral arch origin of the vertebral arteriesAJNR Am J NeuroradiolYear: 20022345545811901018|
|5.||Vorster W,du Plooy PT,Meiring JH. Abnormal origin of internal thoracic and vertebral arteriesClin AnatYear: 19981133379445095|
|6.||Gaupp RJ,Fagan CJ,Davis M,Epstein NE. Pseudocoarctation of the aortaJ Comput Assist TomogrYear: 198155715737263999|
|7.||Taneja K,Kawlra S,Sharma S,Rajani M. Pseudocoarctation of the aorta: complementary findings on plain film radiography, CT, DSA, and MRACardiovasc Intervent RadiolYear: 1998214394419853156|
|8.||Grigsby JL,Galbraith T,Shurmur S,Deligonul U. Pseudocoarctation of the aorta complicated by saccular aneurysm: treatment by aortic arch replacementAm Heart JYear: 19961312002028554012|
|9.||Turner AF,Swenson BE,Jacobson G,Kay JH. Kinking or buckling of the aorta. Case report with complication of aneurysm formationAm J Roentgenol Radium Ther Nucl MedYear: 196697411415|
Keywords: Pseudocoarctation, Computed tomography (CT), Vertebral artery.
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