Mumun Sinha, Niraj Nirmal Pandey, Arun Sharma, Priya Jagia
Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
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|Date of Submission||30-Sep-2019|
|Date of Acceptance||01-Dec-2019|
|Date of Web Publication||12-Feb-2020|
| Abstract|| |
Isolation of the right subclavian artery (RSCA), defined as loss of continuity between the RSCA and aorta, is a rare anomaly and its association with dextrotransposition of great arteries (D-TGA) is exceedingly unusual. We present the case of a 5-day-old neonate with cyanosis from birth and a transthoracic echocardiographic diagnosis of D-TGA, where on computed tomography angiography, the RSCA was seen arising from the proximal part of the ipsilateral pulmonary artery via a patent right ductus arteriosus.
Keywords: Arterial switch operation, computed tomography angiography, isolated subclavian artery, transposition of great arteries
|How to cite this article:|
Sinha M, Pandey NN, Sharma A, Jagia P. An exceedingly rare association of an isolated right subclavian artery with transposition of great vessels. Ann Pediatr Card 2020;13:177-8
|How to cite this URL:|
Sinha M, Pandey NN, Sharma A, Jagia P. An exceedingly rare association of an isolated right subclavian artery with transposition of great vessels. Ann Pediatr Card [serial online] 2020 [cited 2020 Jun 1];13:177-8. Available from: http://www.annalspc.com/text.asp?2020/13/2/177/278216
| Clinical Summary|| |
A 5-day-old neonate with cyanosis from birth and a transthoracic echocardiographic diagnosis of dextrotransposition of great arteries (D-TGA) underwent cardiac computed tomography angiography (CTA) for further evaluation of cardiac and extracardiac anatomy. CTA revealed situs solitus with dextroversion, ventriculoarterial discordance with D-TGA, ventricular septal defect (VSD), and good-sized confluent pulmonary arteries. Interestingly, the right subclavian artery (RSCA) was seen arising from the proximal part of the ipsilateral pulmonary artery via a patent right ductus arteriosus (DA) in the presence of a left-sided aortic arch [Figure 1].
|Figure 1: Cinematic volume-rendered images reveal the origin of the right subclavian artery from the proximal part of the right pulmonary artery via a patent right ductus arteriosus (*). The aorta and main pulmonary artery are seen arising from the right and left ventricle, respectively. RCCA: Right common carotid artery; LCCA: Left common carotid artery; LSCA: Left subclavian artery; RVA: Right vertebral artery; LVA: Left vertebral artery; LPA: Left pulmonary artery|
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| Discussion|| |
Isolation of subclavian artery is defined as loss of continuity between subclavian artery and aorta, with the subclavian artery being connected to the ipsilateral pulmonary artery via a patent/restrictive/closed DA. It generally involves the left subclavian artery (LSCA) and is usually associated with intracardiac (most commonly: tetralogy of Fallot) or aortic arch anomalies. It is always seen on the side contralateral to the aortic arch. Isolation of LSCA is a very rare anomaly seen only in ~0.8% of all right aortic arches, whose incidence itself is ~0.05% in the general population., Isolation of RSCA is even rarer (seen four times less frequently as compared to isolation of LSCA) and an association with D-TGA is exceedingly unusual, making this case an exceedingly rare entity.
Its development can be explained by the embryological occlusion of the right fourth arch distal to the right common carotid artery and persistence of the right sixth arch, which forms the right-sided patent DA and connects with the seventh cervical intersegmental artery.
In the presence of a patent DA and reduced pulmonary blood flow, pulmonary and subclavian steal may cause vertebrobasilar insufficiency. When the ductus is restrictive or closed, only subclavian steal occurs with a relatively lesser risk of cerebral symptoms. In the presence of elevated pulmonary arterial pressures, due to VSD or contralateral patent DA, the direction of shunt may be reversed from the pulmonary to the subclavian artery. Although its presence may be suspected in the setting of unilateral pulmonary plethora in a cyanotic child or in the presence of cerebral symptoms, definitive diagnosis is often possible only on orthogonal imaging. Ligating the DA and surgical reimplanting the RSCA to the ipsilateral common carotid artery at the time of primary cardiac repair would be the ideal albeit a technically challenging management option. The patient in question underwent an arterial switch operation with ligation of the DA [Figure 2].
|Figure 2: Cinematic volume-rendered image of the postarterial switch computed tomography angiography shows restoration of normal ventriculoarterial connections with anterior translocation of main pulmonary artery. The ductus is not seen (dotted circle) with right subclavian artery receiving supply from the right vertebral artery. RCCA: Right common carotid artery; LCCA: Left common carotid artery; LSCA: Left subclavian artery; LVA: Left vertebral artery; LPA: Left pulmonary artery; RIMA: Right internal mammary artery; LIMA: Left internal mammary artery|
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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.
| References|| |
Sen S, Mohanty S, Kulkarni S, Rao SG. Isolated subclavian artery: A rare entity revisited. World J Pediatr Congenit Heart Surg 2016;7:744-9.
Chai OH, Han EH, Kim HT, Song CH. Right-sided aortic arch with the retroesophageal left subclavian artery as the fourth branch. Anat Cell Biol 2013;46:167-70.
Dr. Priya Jagia
Department of Cardiovascular Radiology and Endovascular Interventions, All India Institute of Medical Sciences, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]