Cervical aortic arch with coarctation and aneurysms

Case contributed by Dr Vincent Tatco

Presentation

Patient claims to have elevated blood pressure and experienced episodes of chest pain before consult.

Patient Data

Age: 30 years old
Gender: Female

This anteroposterior chest radiograph shows a widened mediastinum with a convex soft tissue overlying the left hilum.  Calcifications are seen along the margin of the convex soft tissue density.  A soft tissue density is also observed along the left paratracheal region. 

The transverse diameter of the heart is increased which may still be attributed to magnification. 

Apparent haziness in both lower lungs are possibly due to partial volume loss secondary to lack of deep inspiration.

Patient underwent echocardiography which showed colour mosaic flow in the descending aorta at the area of the subclavian artery with an increase in gradients to as high as 58 mmHg.  Coarctation of the aorta was considered. CT angiography of the aorta was requested for further evaluation. 

The aorta arises normally from the left ventricle. Left-sided aortic arch and descending aorta are demonstrated. The aorta gives off three branches in the following order: innominate artery (brachiocephalic trunk), left common carotid artery and left subclavian artery. 

The segment of the aortic arch between the left common carotid artery and left subclavian artery is elongated and markedly tortuous with its apex positioned cephalad slightly above the level of the medial ends of the clavicles consistent with cervical aortic arch.  This segment of the aortic arch also shows multiple saccular aneurysms. The largest saccular aneurysm exhibits wall calcifications. 

There is stenosis (coarctation) of the aortic arch after the take-off of the left common carotid artery. 

The descending segment of the thoracic aorta is smaller in caliber than usual suggestive of mild hypoplasia.  

The right internal thoracic artery is prominent and connects to the right external iliac artery via the superior and inferior epigastric arteries likely serving as a collateral. 

The left brachiocephalic vein courses behind the ascending segment of the aorta before it joins the superior vena cava.

Case Discussion

This is a case of cervical aortic arch complicated by aortic coarctation and aneurysms.  There is left-sided aortic arch with normal sequence of brachiocephalic branching (innominate, left common carotid and left subclavian artery), an excessively long and markedly tortuous aortic arch segment between the left common carotid and left subclavian arteries, and mildly hypoplastic ipsilateral descending aorta consistent with type D cervical aortic arch described by Haughton and colleagues 1

The redundant segment of the aortic arch in this case also exhibits multiple saccular aneurysms which is seen in approximately 20% of cases of cervical aortic arch predominantly occurring in female patients 2,3

The case is also complicated by stenosis or coarctation involving the redundant transverse segment of the aortic arch. The right internal thoracic artery, via its superior epigastric branches anastomoses with the inferior epigastric arteries, provided an important collateral in this case.

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Case Information

rID: 44035
Case created: 4th Apr 2016
Last edited: 2nd Jun 2016
Systems: Vascular, Chest
Inclusion in quiz mode: Included

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