The Cervical Aortic Arch

Moncada, Rogelio; Shannon, Michael T.; Miller, Robert G.; White, Harvey; Friedman, Jamie L.; Shuford, Wade H.

doi:10.2214/ajr.125.3.591

Cited by 55 publications

(18 citation statements)

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“…Cervical aortic arch is an elongated aortic arch that extends into the soft tissues of the neck before turning downward on itself to become the descending aorta (17). This anomaly is usually asymptomatic; however, dysphagia and respiratory symptoms may be present.…”

Section: Cervical Aortic Archmentioning

confidence: 99%

Congenital thoracic arterial anomalies in adult: CT overview

Yıldırım

Karabulut²,

Doğan³

et al. 2011

Diagn Interv Radiol

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Section: Cervical Aortic Archmentioning

confidence: 99%

Congenital thoracic arterial anomalies in adult: CT overview

Yıldırım

Karabulut²,

Doğan³

et al. 2011

Diagn Interv Radiol

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“…The cervical aortic arch is often asymptomatic or presents as a pulsatile neck mass, dysphagia, dyspnea, discrepancy of arterial blood pressure between the upper and lower extremities, cough, hoarseness, and mild stroke. The recognized cardiac anomalies associated with the cervical aortic arch are ventricular septal defect, tetralogy of Fallot, pulmonary atresia with ventricular septal defect, and doubleoutlet right ventricle without pulmonary stenosis [7]. The interesting feature in our case is the occurrence of the cervical aortic arch in the LDS.…”

Section: Discussionmentioning

confidence: 59%

Right-sided cervical aortic arch in Loeys–Dietz syndrome

Shakerian

Mandegar

Moradi

et al. 2015

Journal of Cardiology Cases

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IntroductionThe Loeys-Dietz syndrome (LDS) is a connective tissue disorder. It is an autosomal dominant disorder caused by heterozygous mutation in the gene that encodes transforming growth factor B (TGFB) receptor type 1 or 2 as well as SMAD3. The LDS is characterized by vascular findings such as arterial aneurysm or tortuosity and skeletal and craniofacial manifestation [1].Natural history is significant for aortic dissection at smaller aortic diameter and arterial aneurysms throughout the arterial tree [2]. Case reportA 13-year-old girl presented complaining of a pulsatile mass on the right side of her neck since birth, which had gradually increased in size. Her medical history was otherwise unremarkable. On physical examination, she had a short stature, there was a pulsatile swelling on the right side of the neck (Fig. 1), and the blood pressure was 110/70 mmHg with pulses equal in both upper and lower limbs. Cardiac examination was normal. Examination of her head and face revealed an abnormal cranial structure consistent with hypertelorism, retrognathia, and low-set ears, and inspection of her oral cavity showed a bifid uvula and a high arched palate. Skeleton findings included scoliosis and joint laxity. Chest X-ray showed the absence of a normal aortic knob. The echocardiographic study revealed normal cardiac structure. Aortic valve was normal and tricuspid. Computed tomography angiography revealed a right-sided cervical aortic arch (Fig. 2), reaching the upper margin of the C-7 vertebral body and descending to the left of the mid line. The first branch of the ascending aorta was the left common carotid artery; the second branch was the right common carotid artery, arising at the C-5 vertebral level; the third branch was the right subclavian artery; the next branch was the right vertebral artery at the C-7 level; and the last branch was the left subclavian artery. Genetic study with DNA sequencing was performed and revealed a c.1069 G>A/p.G357R missense mutation in TGFBR2 gene.Cardiac surgery with median sternotomy and extension to the right side of the neck over sternocleidomastoid muscle was performed. The anatomy was confirmed visually (Fig. 3). The ascending aorta and aortic arch in neck was released and aortic arch was dissected. The excess part of cervical aortic arch over right common carotid artery up right subclavian artery was resected. Aortic stump was repaired after the right carotid take-off, and then re-anastomosis of the beginning of descending aorta to the right side of ascending aorta was performed. In this way, the right subclavian artery was saved. Pathological findings were consistent with loss of elastin content and myxoid degeneration of media with subadventitial hemorrhage.The patient remains in good general condition two years after the operation. A B S T R A C TLoeys-Dietz syndrome is an autosomal dominant connective tissue disorder that is characterized by skeletal abnormalities, craniofacial malformations, and predisposition for aortic aneurysm with tortuosity. We report a case o...

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“…Discussion Left cervical aortic arch was thought to be more rare than right, but they are now of almost equal incidence (Moncada et al, 1975). The present case is certainly the most bizarre and the only one associated with an aortic aneurysm, though some cases are associated with cardiac abnormalities such as Left cervical aortic arch associated with aortic aneurysm tetralogy of Fallot and pseudotruncus arteriosus (Moncada et al, 1975). The large aneurysm may be secondary to the haemodynamic disturbance produced by the very tortuous aorta.…”

Section: Case Reportmentioning

confidence: 72%

Left cervical aortic arch associated with aortic aneurysm.

Morris¹,

Ruttley²

1978

Heart

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An aortic arch extending into the neck is a rare congenital anomaly. Left-sided cervical aortic arch was thought to be much less common than right-sided, but they are now almost equal in incidence in the published reports. This paper describes a case of left-sided cervical aortic arch in an adult, with a previously undescribed association, aneurysm of the descending thoracic aorta. Cervical aortic arch must be considered in the differential diagnosis of pulsatile cervical swellings. The embryological development of the aortic arch is discussed. This is still controversial and it is not possible to be sure of the embryological explanation ofthis congenital anomaly of the aortic arch from a study of the adult anatomy.

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The Cervical Aortic Arch

Cited by 55 publications

References 0 publications

Congenital thoracic arterial anomalies in adult: CT overview

Congenital thoracic arterial anomalies in adult: CT overview

Right-sided cervical aortic arch in Loeys–Dietz syndrome

Left cervical aortic arch associated with aortic aneurysm.

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