Congenital Variants and Anomalies of the Aortic Arch

A review of the cross-sectional imaging modalities used in the evaluation of the aortic arch along with a presentation of aortic arch variants, anomalies, and malformations.

Course ID: Q00531 Category:
Modalities: , , ,


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Targeted CE per ARRT’s Discipline, Category, and Subcategory classification for enrollments starting after January 30, 2024:

Computed Tomography: 3.00
Procedures: 3.00
Neck and Chest: 3.00

Magnetic Resonance Imaging: 3.00
Procedures: 3.00
Body: 3.00

Registered Radiologist Assistant: 3.00
Procedures: 3.00
Neurological, Vascular, and Lymphatic Sections: 3.00


  1. Introduction
  2. Imaging
  3. Embryology and Anatomy
  4. Arch Variants and Anomalies
    1. Normal Left Aortic Arch and Variants
    2. Left Aortic Arch with Aberrant Right Subclavian Artery
    3. Left Aortic Arch with Diverticulum of Kommerell
    4. Sidedness of the Ligamentum Arteriosum or Ductus Arteriosus
    5. Left Circumflex Aorta
    6. Right Aortic Arch with Aberrant Left Subclavian Artery Arising from a Retroesophageal Diverticulum
    7. Right Aortic Arch with Aberrant Left Subclavian Artery without a Retroesophageal Diverticulum
    8. Right Aortic Arch with Aberrant Brachopcephalic Artery
    9. Right Aortic Arch with Mirror Image Branching
    10. Right Arch with Isolation of the Left Subclavian Artery
    11. Right Circumflex Aorta
    12. Double Aortic Arch
    13. Cervical Aortic Arch
    14. Persistent Fifth Aortic Arch
    15. Interrupted Aortic Arch
    16. Type A IAA
    17. Type B IAA
    18. Type C IAA
  5. Hypoplasia, Coarctation, and Pseudocoarctation
    1. Aortic Arch Hypoplasia
    2. Coarctation
    3. Pseudocoarctation
  6. Conclusion


Upon completion of this course, students will:

  1. list the features and signs that raise suspicion for a vascular ring
  2. note the importance of detailed evaluation of aortic arch anatomy
  3. list the imaging modalities used to identify and diagnose aortic arch variants and anomalies
  4. state the imaging modality used in the pediatric setting for diagnosis of aortic arch variants and anomalies
  5. name the disadvantages of CT angiography in pediatric patients
  6. identify the non-radiation imaging modality used for evaluation of the aortic arch
  7. explain when aortic arch development begins
  8. describe the two major parts of the primitive aorta
  9. indicate the number of paired primitive aortic arches that develop between the ventral and dorsal aortae
  10. state the branches of the primitive third aortic arch
  11. explain how soon the ductus arteriosus closes after birth
  12. list the three basic congenital anomalies of the aortic arch
  13. state where a normal left arch crosses the spine
  14. list the branches arising from a normal left aortic arch
  15. recall the percent of patients with a typical aortic branching pattern
  16. express the most common congenital anomaly of the aortic arch
  17. explain the percent of patients with trisomy 21 that have a left aortic arch with aberrant right subclavian artery anomaly
  18. recall the percent of adult patients with symptomatic dysphagia associated with a left aortic arch with aberrant right subclavian artery anomaly
  19. describe the origin of the Kommerell diverticulum
  20. describe the location of the arch in relationship to the esophagus in patients with a left circumflex aorta
  21. discuss the most common variation of a right aortic arch
  22. list the most common causes of a vascular ring
  23. state the aortic arch anomaly that is rarely associated with congenital heart disease
  24. state the aortic arch anomaly associated with tetralogy of Fallot
  25. list the aortic branches for patients with a right aortic arch with aberrant brachiocephalic artery anomaly
  26. state the second most common right aortic arch anomaly
  27. recall the percentage of right aortic arch with mirror image branching that is associated with congenital heart disease
  28. indicate the number of ipsilateral breaks in the aortic arch system that causes isolation of arch vessels
  29. list the branches in order coming off of the aorta for a right aortic arch with isolation of the left subclavian artery anomaly
  30. state the alternative name for a right aortic arch with a left descending aorta and left ductus arteriosus or ligamentum arteriosum
  31. choose the arch anomaly that may require an aortic uncrossing procedure for relief of symptoms
  32. describe the aortic arch anomaly that is the most common cause of a symptomatic vascular ring
  33. list the clinical manifestations of a double aortic in children and infants
  34. compare the right and left aortic arch appearance in a double aortic arch anomaly
  35. state the number of proposed theories explaining the development of cervical arches
  36. state the number of embryos that experience degeneration of a fifth aortic arch
  37. list the connections that may be made by a persistent fifth aortic arch
  38. state the number of interrupted aortic arch types
  39. choose which type of interrupted aortic arch is associated with chromosome 22q11 microdeletion
  40. describe normal tapering time for the distal aortic arch