Aortic Arch Pattern and Embryology — #2 Retroesophageal RSCA

Aortic Arch Pattern and Embryology — #2 Retroesophageal RSCA

image

An aberrant right subclavian artery (ARSA, arteria lusoria) arises as the final branch of a left-sided aortic arch and typically courses posterior to the esophagus toward the right thoracic outlet. Reported prevalence in contemporary series ranges around 0.5–2%, and symptomatic cases cluster around dysphagia and airway complaints (“dysphagia lusoria”). Co-variants frequently include a bicarotid trunk and a Kommerell diverticulum at the aberrant origin [1, 2].

Embryologic basis. In normal development, the right subclavian artery is formed by the right 4th arch, the proximal right dorsal aorta, and the right 7th intersegmental artery. ARSA results when the right 4th arch and proximal right dorsal aorta involute, leaving the right 7th intersegmental artery attached to the left dorsal aorta; the vessel then originates distal to the left subclavian and must traverse retroesophageally to the right upper limb [1].

Anatomic course and key variants.

  • Most ARSAs pass behind the esophagus; infrequently they course between trachea and esophagus, and only rarely anterior to the trachea [2].
  • A Kommerell diverticulum—a bulbous remnant at the aberrant takeoff—may be present and is a substrate for aneurysmal degeneration or dissection [2].
  • ARSA may coexist with a bicarotid trunk or appear within right-sided arch variants; certain combinations (e.g., right arch with aberrant left subclavian) form a complete vascular ring [2].

Clinical significance. Many patients remain asymptomatic. When present, symptoms reflect esophageal and/or tracheal compression—dysphagia, feeding difficulty, stridor, cough, recurrent infections, or failure to thrive in infants. Dysphagia risk appears higher when the artery lies closer to the trachea and has a larger luminal area at the esophageal level; these CT-derived morphometrics correlate with symptoms [3]. ARSA is also enriched among fetuses and infants with chromosomal anomalies (e.g., trisomy 21, 22q11.2 deletion), warranting careful systemic evaluation when detected prenatally [4].

Imaging diagnosis.

  • Echocardiography can suggest an abnormal branching pattern in infants but lacks distal arch detail.
  • CT angiography (CTA) or MR angiography (MRA) best delineates branching sequence, vessel caliber, and the presence/size of a Kommerell diverticulum; barium esophagram may show a characteristic posterior indentation [2, 3].
  • Cross-sectional imaging is essential to guide intervention planning and to surveil suspected aneurysmal change [2, 5, 6].

Management principles.

  • Observation is appropriate for asymptomatic patients without aneurysmal change; interval cross-sectional imaging documents stability [2, 6].
  • Intervention is indicated for symptoms attributable to compression (dysphagia/airway compromise) and/or aneurysmal degeneration (often at a Kommerell diverticulum). Contemporary strategies are anatomy-specific and include:
    • Division and reimplantation/transposition of the ARSA to the right common carotid via a supraclavicular or cervical approach to eliminate the retroesophageal course while preserving right arm perfusion [7].
    • Open, hybrid, or endovascular management of a Kommerell diverticulum (e.g., resection or exclusion with carotid–subclavian revascularization and TEVAR), tailored to arch sidedness, landing zones, and comorbidities [5, 6].
  • Outcomes in modern series are favorable, with high rates of symptom relief and acceptable morbidity when procedures are performed in experienced centers [5, 6].

Operative cautions (surgeon’s view). During cervical mobilization and transposition, protect the recurrent laryngeal nerve and achieve a tension-free carotid anastomosis. In infants, plan exposure mindful of the close relationship to the trachea and esophagus, and consider whether concomitant arch work (e.g., diverticulum tailoring) is required for durable decompression [5, 7].

References

[1] Choi Y, Chung SB, Kim MS. Prevalence and anatomy of aberrant right subclavian artery evaluated by computed tomographic angiography at a single institution in Korea. J Korean Neurosurg Soc. 2019;62(2):175-182. PubMed

[2] Polguj M, Chrzanowski Ł, Kasprzak JD, Stefanczyk L. The aberrant right subclavian artery (arteria lusoria) — morphological and clinical aspects. Med Sci Monit. 2014;20:493-500. PubMed+1

[3] Krupiński M, Irzyk M, Moczulski Z, et al. CT evaluation of aberrant right subclavian artery: anatomy and clinical implications. Cardiol Young. 2019;29(2):128-132. PubMed

[4] Annetta R, Massaro G, Esposito G, et al. Aberrant right subclavian artery: embryology, prenatal diagnosis and clinical significance. J Cardiovasc Dev Dis. 2021;8(3):27. PMC

[5] van Bogerijen GHW, Patel HJ, Eliason JL, et al. Evolution in the management of aberrant subclavian arteries and related Kommerell diverticulum. Ann Thorac Surg. 2015;100(1):47-53. PubMed

[6] Erben Y, Brownstein AJ, Velasquez CA, et al. Natural history and management of Kommerell’s diverticulum in a single tertiary referral center. J Vasc Surg. 2020;71(6):2004-2011. PubMed

[7] Atay Y, Engin C, Posacıoğlu H, et al. Surgical approaches to the aberrant right subclavian artery. Tex Heart Inst J. 2006;33(4):477-481. PubMed+1