Table of Contents  
Year : 2015  |  Volume : 2  |  Issue : 3  |  Page : 118-121

Double Aortic Arch Surgery

1 Department of Cardiovascular and Thoracic, Grant Medical College, Mumbai, Maharashtra, India
2 Department of Cardiovascular and Thoracic, Seth G.S. Medical College, Mumbai, Maharashtra, India

Date of Web Publication8-Oct-2015

Correspondence Address:
Suraj Wasudeo Nagre
Department of Cardiovascular and Thoracic, Grant Medical College, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-0820.166936

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A 4-year-old male child was admitted with a history of difficulty in swallowing, failure to thrive and recurrent respiratory tract infections. On investigations, cardiac computed tomography was suggestive of a double aortic arch (DAA) and a virtual bronchoscopy was suggestive of tracheomalacia. After ligating the nondominant part of the DAA, the child had symptomatic relief.

Keywords: Double aortic arch, patent ductous arterious, tracheomalacia

How to cite this article:
Nagre SW, Kulkarni DV. Double Aortic Arch Surgery. Indian J Vasc Endovasc Surg 2015;2:118-21

How to cite this URL:
Nagre SW, Kulkarni DV. Double Aortic Arch Surgery. Indian J Vasc Endovasc Surg [serial online] 2015 [cited 2022 Dec 3];2:118-21. Available from:

  Introduction Top

Double aortic arch (DAA) is a relatively rare congenital cardiovascular malformation but the most common of the complete vascular rings, causing tracheo-esophageal compression. This is a case report of a DAA with balanced aortic arches, presenting with extrinsic tracheobronchial obstruction. DAA is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. The symptoms are related to the compression of the trachea, esophagus or both by the complete vascular ring. Treatment is surgical and is indicated in all symptomatic patients. In the current era, the risk of mortality or significant morbidity after the surgical division of the lesser arch is low. However, the preoperative degree of tracheomalacia has an important impact on postoperative recovery. In certain patients, it may take several months (up to 1–2 years) for the obstructive respiratory symptoms (wheezing) to disappear.

  Case Report Top

A 4-year–old, weighing 6 kg male child was admitted with complaints of noisy breathing, persistent dry cough, difficulty in swallowing with regurgitation, failure to thrive, and recurrent respiratory tract infection. He had required hospital admission for similar complaints twice. Chest X-ray showed no perceptible indentation of the tracheal shadow. Echocardiographic (ECHO) evaluation showed normal cardiac anatomy and a right aortic arch. The aberrant course of the left subclavian artery was suspected. Cardiac computed tomography (CT) [Figure 1] was suggestive of a DAA compressing the trachea and esophagus. The right aortic arch was dominant (11 mm) giving origin to right common carotid and right subclavian artery. Left aortic arch was nondominant (size: 6 mm) giving rise to left CCA and left subclavian artery [Figure 1]. Virtual bronchoscopy [Figure 2] revealed compression of the lower one-third of the trachea with only a 5 mm patent tracheal lumen and tracheomalacia [Figure 2].
Figure 1: Contrast-enhanced computed tomography scan showing double aortic arch with tracheal compression

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Figure 2: Tracheal compression because of double aortic arch

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Operative procedure

The patient underwent division of the left aortic arch through a left posterolateral thoracotomy (3rd intercostal space) approach. The right aortic arch was posterior, giving rise to the right common carotid and subclavian arteries. The left aortic arch was anterior, giving rise to the left common carotid and left subclavian arteries [Figure 3]. The patent ductus arteriosus an accidental finding was divided [Figure 4].
Figure 3: Left subclavin artery, left aortic arch and patent ductus arteriosus

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Figure 4: After ligation and division of left aortic arch and patent ductus arteriosus

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The anterior left aortic arch was clamped just proximal to its junction with the descending aorta. There was no decrease in femoral arterial blood pressure, no gradient between the left radial and the femoral arterial blood pressures, and both radial and carotid pulses were palpable. The anterior left aortic arch was divided at its junction with the descending aorta, and the cut edges were oversewn. The mediastinal tissue around the aortic arches was dissected, allowing the right aortic arch to retract behind the esophagus. Strands of tissue around the trachea and esophagus were divided to relieve any potential residual compression or fibrosis.

The child was extubated on the 1st postoperative day. He was stable and comfortable without any clinical evidence of respiratory obstruction or tracheobronchomalacia. He was transferred to the ward on the 2nd postoperative day. The remaining postoperative recovery was uneventful, with no respiratory or feeding problems. Post-procedure CT aortogram revealed a diameter of 10.5 mm for the right aortic arch; the tracheal dimensions were 4.4 mm × 7.1 mm. The patient continues to be free of respiratory compromise and feeding problems at follow-up of 1-year. ECHO revealed a peak gradient of 8 mm Hg at the junction of the left aortic arch with the descending aorta.

  Discussion Top

Complete vascular rings are aortic arch anomalies in which the trachea and esophagus are compressed by the aortic arches and their derivatives. DAA, first reported in 1737 by Hommel, is due to the presence of right and left aortic arches encircling and compressing the trachea and the esophagus. Gross performed the first successful surgical division of a vascular ring in 1945 at Boston Children's Hospital through a left anterolateral chest approach.[1] He further elucidated the surgical principles involved in the division of vascular rings.[2]

Embryologically, the ventral and dorsal aortas are connected by aortic arches which persist or involute to give rise to the normal aortic arch, its branches and minor arteries of the head. The right fourth aortic arch normally involutes at about 36–38 days in the 16-mm embryo and the left fourth aortic arch persists to give rise to the normal left aortic arch.[3] A schematic depiction with DAAs and double ductus arteriosi was described by Edwards to explain the various aortic arch anomalies due to the abnormal persistence or regression of various segments in this hypothetical DDA model.[4] The persistence of both the right and left fourth aortic arches leads to a DDA.

Anatomically, the ascending aorta arises normally, and as it exits the pericardium, it divides into two – right and left aortic arches which encircle the trachea and the esophagus and reunite posteriorly to form the descending aorta. Hypoplasia of one of the aortic arches is common with one arch, more commonly the right aortic arch, being dominant. Atresia can be uncommonly present in any of the segments of either of the aortic arches, resulting in various subtypes of DDA with atresia.[5] The posterior descending aorta formed by the union of the two aortic arches can be on the left or on the right of the thoracic vertebrae. A right dominant DDA has been more commonly observed with left descending aorta and vice versa. The descending aorta has a tendency to be more midline than normal. This abnormal position can cause compression of the airway between the pulmonary artery and the descending aorta postoperatively, leading to persistence of symptoms following surgery.[6] The ductus or ligamentum arteriosum, which is not a part of the vascular ring, runs between the left pulmonary artery, inferior to the junction between the left aortic arch and the descending aorta.[7] The aortic arches give rise to the ipsilateral common carotid and subclavian arteries and the innominate artery is absent.

Patients with DDA can be asymptomatic or present with symptoms ranging from nonspecific complaints to life-threatening respiratory distress. The symptoms of stridorous breathing, dysphagia, a “barky” chronic cough, susceptibility to bronchopneumonia, head retraction, malnutrition, onset during early infancy, and an increase of respiratory distress during feeding were described by Wolman.[1] An esophageal foreign body at the site of compression by the vascular ring is rarely the presenting feature of DDA. Life-threatening episodes of respiratory arrest and apnea have also been described.[8]

Conventional chest X-ray may show the indentation of tracheal shadow, retro tracheal opacity, and anterior tracheal bowing. Specific radiological signs have been described for barium esophagography.[9] These include bilateral persistent extrinsic compressions of the esophagus in anteroposterior view, with the dominant arch causing a deeper and superior indentation and a deep posterior indentation in lateral and oblique views. A barium swallow is diagnostic in the majority of cases. However, multidetector computed tomography and magnetic resonance imaging have become increasingly utilized in the diagnosis and evaluation of aortic arch anomalies, including DDA.[10] The assessment of the arch dominance and surrounding tissues in the mediastinum has improved with these radiological modalities, especially when the barium swallow is negative, there is innominate artery compression, or in complex cases. The four-vessel sign in the superior mediastinum can be seen due to the presence of separate subclavian and common carotid arteries on both sides. Presently angiography is rarely indicated or necessary for adequate evaluation of aortic arch anomalies. ECHO is recommended to rule out associated congenital cardiac defects.[8],[10],[11],[12]

Repair is achieved through a left posterolateral thoracotomy approach, especially those with a dominant right aortic arch.[8],[10],[11],[13] The Mayo Clinic recommends a right posterolateral thoracotomy approach in patients with left aortic arch, right-sided descending thoracic aorta, and right ductus or ligamentum arteriosum; a double arch with atresia of the right posterior segment; or when anastomosis of an aberrant right subclavian artery to the ascending aorta is performed.[14] Backer and Mavroudis [15] recommend that innominate artery compression be approached from the right-side, with the suspension of the innominate artery to the sternum. Repair through a median sternotomy is recommended when concomitant repair of intracardiac defects is performed [Table 1].[12],[14]
Table 1: A contemporary review of the literature regarding double aortic arches

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The principles of surgery are essentially the same as described by Gross.[3] The surgical repair includes adequate dissection of the aortic arches and descending aorta, division of the nondominant aortic arch, division of the ductus or ligamentum arteriosum, and the dissection and division of the mediastinal adventitial bands that may compress the trachea or esophagus.

Postoperative complications include bleeding, vocal cord paralysis, pneumonia, pneumothorax, chylothorax, feeding difficulties, and residual respiratory obstruction. Residual respiratory complaints have been noted in up to 54% of patients.[8],[11],[13] While the residual respiratory symptoms are usually due to tracheobronchomalacia, anatomic compression of the trachea by the postoperative arch and the anterior remnant of the divided arch, or by midline descending aorta, can be present.[7] Due to the presence of the dual-sidedness of the balanced aortic arches, these patients have a more midline descending aorta. This results in abnormal stacking of the structures anterior to the spine and leads to extrinsic compression of the left main bronchus between the midline descending aorta posteriorly and the pulmonary artery anteriorly.

  Conclusion Top

DDA surgery seems to be simple and safe even in unexperienced hand if operating surgeon has proper understanding of anatomy. Symptomatic recovery was fast and very good after surgery. Early diagnosis and surgery were key factor for better symptomatic recovery. Vascular rings, more commonly the DDA, are an important cause of tracheo-esophageal compression, the clinical suspicion and diagnosis of which can lead to early surgical intervention and relief or avoidance of immediate or long term respiratory complications.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Wolman IJ. Syndrome of constricting double aortic arch in infancy: Report of a case. J Pediatr 1939;14:527-33.  Back to cited text no. 1
Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 1945;233:586-90.  Back to cited text no. 2
Gross RE. Arterial malformations which cause compression of the trachea or esophagus. Circulation 1955;11:124-34.  Back to cited text no. 3
Congdon ED. Transformation of the aortic arch system during the development of the human embryo. Contrib Embryol 1922;14:47-110.  Back to cited text no. 4
Edwards JE. Anomalies of the derivatives of the aortic arch system. Med Clin North Am 1948;32:925-49.  Back to cited text no. 5
Backer CL, Mavroudis C. Congenital heart surgery nomenclature and database project: Vascular rings, tracheal stenosis, pectus excavatum. Ann Thorac Surg 2000;69 4 Suppl: S308-18.  Back to cited text no. 6
Fleck RJ, Pacharn P, Fricke BL, Ziegler MA, Cotton RT, Donnelly LF. Imaging findings in pediatric patients with persistent airway symptoms after surgery for double aortic arch. AJR Am J Roentgenol 2002;178:1275-9.  Back to cited text no. 7
Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW. Management and outcomes of double aortic arch in 81 patients. Pediatrics 2006;118:e1336-41.  Back to cited text no. 8
Noguchi K, Hori D, Nomura Y, Tanaka H. Double aortic arch in an adult. Interact Cardiovasc Thorac Surg 2012;14:900-2.  Back to cited text no. 9
Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005;129:1339-47.  Back to cited text no. 10
Shanmugam G, Macarthur K, Pollock J. Surgical repair of double aortic arch: 16-year experience. Asian Cardiovasc Thorac Ann 2005;13:4-10.  Back to cited text no. 11
Umegaki T, Sumi C, Nishi K, Ikeda S, Shingu K. Airway management in an infant with double aortic arch. J Anesth 2010;24:117-20.  Back to cited text no. 12
Chun K, Colombani PM, Dudgeon DL, Haller JA Jr. Diagnosis and management of congenital vascular rings: A 22-year experience. Ann Thorac Surg 1992;53:597-602.  Back to cited text no. 13
van Son JA, Julsrud PR, Hagler DJ, Sim EK, Pairolero PC, Puga FJ, et al. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc 1993;68:1056-63.  Back to cited text no. 14
Lowe GM, Donaldson JS and Backer CL. Vascular rings: 10-year review of imaging. Radiographics 1991;11:637-46.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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