Year : 2019  |  Volume : 12  |  Issue : 2  |  Page : 141--143

“Double-lumen” aortic arch with “double-lumen” brachiocephalic artery

Rengarajan Rajagopal1, Pawan Kumar Garg2, Pushpinder Singh Khera2, Sanjiv Sharma1,  
1 Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
2 Department of Diagnostic and Interventional Radiology, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

Correspondence Address:
Dr. Rengarajan Rajagopal
Room No. 10A, Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi


Persistence of the embryological fifth aortic arch in postnatal life has been a subject of debate for over more than a century. We describe a patient with congenital kyphoscoliosis and variant anatomy in the aortic arch and brachiocephalic trunk which could be possibly explained by the persistence of bilateral fifth aortic arches, a pattern which has never been previously reported in literature.

How to cite this article:
Rajagopal R, Garg PK, Khera PS, Sharma S. “Double-lumen” aortic arch with “double-lumen” brachiocephalic artery.Ann Pediatr Card 2019;12:141-143

How to cite this URL:
Rajagopal R, Garg PK, Khera PS, Sharma S. “Double-lumen” aortic arch with “double-lumen” brachiocephalic artery. Ann Pediatr Card [serial online] 2019 [cited 2019 Jun 19 ];12:141-143
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The embryonic aortic arch arteries are derived from the arteries developing within the pharyngeal arches, which are six symmetrical transient paired structures undergoing a predictable sequence of evolution. Controversy still exists about the embryological fifth arch and its derivatives. The fifth arch artery has been invoked to explain otherwise enigmatic congenital cardiovascular malformations even though convincing evidence is lacking. Systemic-to-systemic pattern of bilateral persistent fifth arch arteries has previously not been reported in literature.[1]

 Case Report

An elderly short-statured gentleman with congenital cervicodorsal kyphoscoliosis was referred for preoperative evaluation of the neck vessels. Doppler examination showed an abnormally low cervical location of the carotid bifurcation on both sides with otherwise normal arteries. Computed tomography angiogram revealed a vascular channel arising from the ascending aorta at the level of origin of the brachiocephalic trunk and terminating into the proximal descending thoracic aorta. The origins of the left common carotid and the left subclavian artery were from the definitive aortic arch which was located cranial to this channel [Figure 1]a. This appearance has previously been described as “double-lumen” aorta.[2] Similar “double-lumen” appearance was also seen in the brachiocephalic artery [Figure 1]b. Both these channels showed calcification [Figure 2]. In addition, the carotid bifurcations on both sides were located at the level of the thoracic outlet, lower than usual [Figure 1]b. The pulmonary artery was unremarkable. Echocardiography showed no intracardiac defects. Multiple defects (butterfly vertebrae and vertebral fusion) were observed in the cervicodorsal vertebrae.{Figure 1}{Figure 2}


According to classical teaching, the six embryological aortic arches are said to undergo a complex process of remodeling with regression of the first, second, and fifth arch arteries by mid-embryogenesis, resulting in the characteristic adult thoracic anatomy. The right fourth arch forms the brachiocephalic artery, and the left fourth arch forms the definitive aortic arch. Although the standard textbooks of cardiac embryology describe the formation of six bilaterally symmetrical channels and their subsequent remodeling, researchers are yet to demonstrate all arteries in embryos. Persistence of the fifth aortic arch has been a subject of debate for over more than a century since the initial description of Condgon in 1922.[3],[4] A vascular structure which extended through a segment of pharyngeal mesenchyme was found in a solitary human embryo likely representing the fifth arch artery.[4] Van Praagh andVan Praagh, in 1969, first described a congenital “double-lumen” aortic arch in a male patient, attributing this pattern to persistence of the fifth arch.[2] After this description, several cases were interpreted similarly in the absence of plausible alternative explanations. However, several researchers are of the opinion that most of the vascular malformations which have been attributed so could be due to alternate developmental aberrations.[1]

Studies have demonstrated transient dorsal collateral channels connecting the fourth and sixth arches in experimental mice, but these channels do not extend through the parietal pharyngeal mesoderm.[4] The persistence of such arteries has been proposed as an alternate hypothesis for the development of vascular structures explained due to persistent fifth arch in adults, rather than due to a true persistence. However, these channels are plexiform entities which do not appear to be correlated precisely to somite number and do not resemble the solitary vascular channel parallel to the fourth and sixth arches.

The most comprehensive definition which has been proposed till date is that of an extrapericardial vessel which arises from the ascending aorta proximal to the origin of the brachiocephalic trunk though its termination may be either into the dorsal aorta or into the pulmonary arteries through the persistent ductus arteriosus.[1] While in the opinion of Gupta et al., it is the inclusion of the persistent ductus arteriosus as part of the channel which could explain the response of some these channels to prostaglandin E1 infusions, it may well be due to anomalous infiltration of prostaglandin-sensitive tissue at the aortic end of the sixth arch (later developing into the ductus arteriosus) into the fifth arch.

The “double-lumen” aortic arch and “double-lumen” brachiocephalic artery in our patient could be classified as a systemic-to-systemic “Type 4” persistent fifth arch according to the system proposed by Freedom et al.[5] This systemic-to-systemic pattern has never been reported previously with the only other reported case of “Type 4” persistent fifth arch having a right-sided “double-lumen” aortic arch coexisting with a left-sided vessel supplying an isolated left pulmonary artery.[6] The unusually low bifurcation of carotid arteries could be explained by the persistence of the ductus caroticus with involution of the third arch and nonmigration of the external carotid artery toward the carotid axis.[7] An association between cervical vertebral anomalies (fusion and butterfly vertebrae in our patient) and segmental developmental vascular anomalies has also been previously observed.

Most of these channels are diagnosed incidentally with no significant hemodynamic compromise. With coexistent severe coarctation or interruption of the fourth definitive aortic arch, however, the flow to the distal aorta is maintained by the fifth arch. In these patients, the unusually inferior position of the aortic arch with all head-and-neck vessels originating from a single connection to the ascending aorta points toward a persistent fifth aortic arch, the diagnosis of which may be otherwise difficult. The calcification seen in both vascular structures in our patient may be due to the flow turbulence due to the abnormal anatomy. Finally, the recognition of these anomalies is also of prime importance to the interventionist performing procedures related to the aortic arch and neck vessels.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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