Double Aortic Arch Workup

  • Author: Doff B McElhinney, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Mar 27, 2012
 

Laboratory Studies

  • In differentiating double aortic arch from upper or lower respiratory infections, a WBC count and respiratory viral studies may be helpful. However, because patients with double aortic arch are predisposed to respiratory infections, the diagnosis of an infection does not exclude the possibility of double aortic arch.
  • As a result of the possible association between double aortic arch and band 22q11 deletion, practitioners may wish to obtain a karyotype and a fluorescence in situ hybridization (FISH) test for deletions within band 22q11. The diagnosis of band 22q11 deletions allows targeted evaluation and follow-up care for conditions frequently found in patients with this chromosomal anomaly.
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Imaging Studies

  • Chest radiography: In patients with a double aortic arch, chest radiography may indicate the presence of a vascular ring. In some patients, lateral indentation of the tracheal air column may be revealed by anteroposterior or posteroanterior projection, usually in both the more superior right arch and the more inferior left arch. On lateral chest films, posterior indentation of the trachea by the right arch may be depicted.
  • Echocardiography: In most patients, the diagnosis of double aortic arch can be made reliably based on echocardiography; helpful information is obtained with suprasternal, high parasternal, and subcostal imaging. However, because patients with a vascular ring typically present with respiratory symptoms, the diagnosis is usually made based on other imaging modes. Echocardiography is important in the evaluation of associated cardiovascular anomalies.[6] Double aortic arch may also be identified on prenatal ultrasonography.
  • MRI and CT scanning: MRI and CT scanning are the best single imaging studies for the diagnosis and characterization of vascular rings. MRI and CT provide complete information in regard to the arterial branching pattern, clearly demonstrate the locations and extent of airway and esophageal obstruction, and can be used to delineate cardiac anatomy (see image below). In addition to tomographic images, 3-dimensional reconstruction of the aorta and airways can be a useful tool for preoperative planning. Transverse MRI images in a patient with double aorTransverse MRI images in a patient with double aortic arch. Both arches are patent; the right arch is dominant. Images A-F are arranged in a caudad to cephalad order. (A) Transverse image at the level of the pulmonary valve. The ascending aorta (AAo) and descending aorta (DAo), cephalad to the junction of the left and right arches, can be seen. (B) At the level of the pulmonary artery (PA) bifurcation, the distal confluence of the left and right arches forming the single descending aorta is depicted. (C) The distal portions of the left (L) and right (R) arches can be seen posterior and to the left and right sides of the trachea. Note the anteroposterior compression of the tracheal carina (anterior to and between the arches). (D) Moving cephalad, the dominance of the right arch can be seen. (E) At the level of the proximal/transverse aortic arches, the origin of the left and right arches from the rightward ascending aorta can be seen. (F) The left and right common carotid and subclavian arteries arise from the left and right arches, respectively. The common carotid arteries are the dark round structures anterior to and to either side of the trachea. The subclavian arteries are the dark round structures posterior to and to either side of the trachea.
  • Cardiac catheterization: This is not usually indicated in patients with double aortic arch. Although angiography was once the criterion standard for the diagnosis of this anomaly, MRI is both less invasive and diagnostically superior. Angiograph findings may be somewhat confusing because of the overlapping projection of the various vascular structures. This problem may be somewhat ameliorated by the use of digital subtraction angiography or countercurrent brachial angiography, which provide sequential information that can help clarify the aortic branching pattern.
  • Barium esophagography: Frequently, the diagnosis of a vascular ring is made initially with barium esophagography. In patients with a double aortic arch, bilateral indentation of the esophagus is observed on the anteroposterior view, with the right-sided indentation superior to the left, and posterior indentation is observed on the lateral view. Although this test is not necessary to make the diagnosis of double arch, it often is obtained in the preliminary evaluation of patients with symptoms of an upper airway and/or an esophageal pathologic condition. If a ring is strongly suspected on the basis of chest radiography or echocardiography findings, barium esophagography is not indicated. Rather, if additional imaging is desired, a more thorough diagnostic study, such as MRI, should be performed.
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Other Tests

  • Electrocardiography: No characteristic ECG findings are associated with double aortic arch, and ECG findings are usually normal, except in patients with associated cardiovascular anomalies.
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Procedures

  • Diagnostic procedures are generally not necessary in the evaluation of patients with a double aortic arch.
  • Bronchoscopy may be obtained in the evaluation of a patient with a suspected airway pathologic condition but is not indicated in most patients. Pulsatile compression of the posterior and lateral walls of the trachea can be observed in patients with a double aortic arch, but the specific type of vascular ring cannot always be determined. Other imaging modalities, especially MRI and CT, permit characterization of the severity and location of tracheal obstruction while offering superior definition of vascular anatomy.
  • In some patients, depending on surgeon preference, bronchoscopy may be performed in the operating room before and after repair of the vascular ring to determine the efficacy of relief of tracheal compression. This is of particular value in young patients in whom persistent tracheomalacia is a concern.
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Histologic Findings

  • Patients with a double aortic arch do not require a histologic tissue examination.
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Contributor Information and Disclosures
Author

Doff B McElhinney, MD  Assistant Professor of Pediatrics, Harvard Medical School; Associate in Cardiology, Department of Cardiology, Children's Hospital of Boston

Doff B McElhinney, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: Nothing to disclose.

Coauthor(s)

Gil Wernovsky, MD, FACC, FAAP  Professor, Department of Pediatrics, University of Pennsylvania, Children's Hospital of Philadelphia

Gil Wernovsky, MD, FACC, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Juan Carlos Alejos, MD  Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD  Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References

  1. Umegaki T, Sumi C, Nishi K, Ikeda S, Shingu K. Airway management in an infant with double aortic arch. J Anesth. Feb 2010;24(1):117-20. [Medline].

  2. Noguchi K, Hori D, Nomura Y, Tanaka H. Double aortic arch in an adult. Interact Cardiovasc Thorac Surg. Feb 28 2012;[Medline].

  3. Tuo G, Volpe P, Bava GL, et al. Prenatal diagnosis and outcome of isolated vascular rings. Am J Cardiol. Feb 1 2009;103(3):416-9. [Medline].

  4. McElhinney DB, Clark BJ 3rd, Weinberg PM, Kenton ML, McDonald-McGinn D, Driscoll DA, et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol. Jun 15 2001;37(8):2114-9. [Medline].

  5. Moral S, Zuccarino F, Loma-Osorio P. Double aortic arch: an unreported anomaly with Kabuki syndrome. Pediatr Cardiol. Jan 2009;30(1):82-4. [Medline].

  6. Seo HS, Park YH, Lee JH, Hur SC, Ko YJ, Park SY, et al. A case of balanced type double aortic arch diagnosed incidentally by transthoracic echocardiography in an asymptomatic adult patient. J Cardiovasc Ultrasound. Sep 2011;19(3):163-6. [Medline]. [Full Text].

  7. Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW, Brown JW. Follow-up of surgical correction of aortic arch anomalies causing tracheoesophageal compression: a 38-year single institution experience. J Pediatr Surg. Jul 2009;44(7):1328-32. [Medline].

  8. Fraga JC, Calkoen EE, Gabra HO, McLaren CA, Roebuck DJ, Elliott MJ. Aortopexy for persistent tracheal obstruction after double aortic arch repair. J Pediatr Surg. Jul 2009;44(7):1454-7. [Medline].

  9. Achiron R, Rotstein Z, Heggesh J, et al. Anomalies of the fetal aortic arch: a novel sonographic approach to in-utero diagnosis. Ultrasound Obstet Gynecol. Dec 2002;20(6):553-7. [Medline].

  10. Anand R, Dooley KJ, Williams WH, Vincent RN. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatr Cardiol. Mar-Apr 1994;15(2):58-61. [Medline].

  11. Angelini A, Dimopoulos K, Frescura C, et al. Fatal aortoesophageal fistula in two cases of tight vascular ring. Cardiol Young. Mar 2002;12(2):172-6. [Medline].

  12. Arciniegas E, Hakimi M, Hertzler JH, et al. Surgical management of congenital vascular rings. J Thorac Cardiovasc Surg. May 1979;77(5):721-7. [Medline].

  13. Backer CL, Ilbawi MN, Idriss FS, DeLeon SY. Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg. May 1989;97(5):725-31. [Medline].

  14. Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg. Jun 2005;129(6):1339-47. [Medline].

  15. Bertrand JM, Chartrand C, Lamarre A, Lapierre JG. Vascular ring: clinical and physiological assessment of pulmonary function following surgical correction. Pediatr Pulmonol. Nov-Dec 1986;2(6):378-83. [Medline].

  16. Bonnard A, Auber F, Fourcade L, et al. Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg. Apr 2003;38(4):539-43. [Medline].

  17. Burke RP, Rosenfeld HM, Wernovsky G, Jonas RA. Video-assisted thoracoscopic vascular ring division in infants and children. J Am Coll Cardiol. Mar 15 1995;25(4):943-7. [Medline].

  18. Cerillo AG, Amoretti F, Moschetti R, et al. Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. Mar 18 2005;99(2):191-4. [Medline].

  19. Chaikitpinyo A, Panamonta M, Sutra S, et al. Aortoesophageal fistula: a life-threatening cause of upper gastrointestinal hemorrhage in double aortic arch, a case report. J Med Assoc Thai. Aug 2004;87(8):992-5. [Medline].

  20. Chun K, Colombani PM, Dudgeon DL, Haller JA Jr. Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thorac Surg. Apr 1992;53(4):597-602; discussion 602-3. [Medline].

  21. Fleenor JT, Weinberg PM, Kramer SS, Fogel M. Vascular rings and their effect on tracheal geometry. Pediatr Cardiol. Sep-Oct 2003;24(5):430-5. [Medline].

  22. Giavini E, Prati M, Vismara C. Morphogenesis of aortic arch malformations in rat embryos after maternal treatment with glycerol formal during pregnancy. Acta Anat (Basel). 1981;109(2):166-72. [Medline].

  23. Hartenberg MA, Salzberg AM, Krummel TM, Bush JJ. Double aortic arch associated with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. May 1989;24(5):488-90. [Medline].

  24. Hartyanszky IL, Lozsadi K, Marcsek P, et al. Congenital vascular rings: surgical management of 111 cases. Eur J Cardiothorac Surg. 1989;3(3):250-4. [Medline].

  25. Heck HA Jr, Moore HV, Lutin WA, et al. Esophageal-aortic erosion associated with double aortic arch and tracheomalacia. Experience with 2 infants. Tex Heart Inst J. 1993;20(2):126-9. [Medline].

  26. Kocis KC, Midgley FM, Ruckman RN. Aortic arch complex anomalies: 20-year experience with symptoms, diagnosis, associated cardiac defects, and surgical repair. Pediatr Cardiol. Mar-Apr 1997;18(2):127-32. [Medline].

  27. Kogon BE, Forbess JM, Wulkan ML, Kirshbom PM, Kanter KR. Video-assisted thoracoscopic surgery: is it a superior technique for the division of vascular rings in children?. Congenit Heart Dis. Mar 2007;2(2):130-3. [Medline].

  28. Koontz CS, Bhatia A, Forbess J, Wulkan ML. Video-assisted thoracoscopic division of vascular rings in pediatric patients. Am Surg. Apr 2005;71(4):289-91. [Medline].

  29. Lillehei CW, Colan S. Echocardiography in the preoperative evaluation of vascular rings. J Pediatr Surg. Aug 1992;27(8):1118-20; discussion 1120-1. [Medline].

  30. McElhinney DB, Jacobs I, McDonald-McGinn DM, Goldmuntz E. Chromosomal and cardiovascular anomalies associated with congenital laryngeal web. Int J Pediatr Otorhinolaryngol. Oct 21 2002;66(1):23-27. [Medline].

  31. McElhinney DB, McDonald-McGinn D, Zackai EH, Goldmuntz E. Cardiovascular anomalies in patients diagnosed with a chromosome 22q11 deletion beyond 6 months of age. Pediatrics. Dec 2001;108(6):E104. [Medline].

  32. Mihaljevic T, Cannon JW, del Nido PJ. Robotically assisted division of a vascular ring in children. J Thorac Cardiovasc Surg. May 2003;125(5):1163-4. [Medline].

  33. Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med. Feb 2006;25(2):251-7. [Medline].

  34. Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med. 2006;25:[Medline].

  35. Picard E, Tal A. Tracheal compression caused by double aortic arch in two sisters. Isr J Med Sci. Nov 1992;28(11):799-801. [Medline].

  36. Rimell FL, Shapiro AM, Meza MP, et al. Magnetic resonance imaging of the pediatric airway. Arch Otolaryngol Head Neck Surg. Sep 1997;123(9):999-1003. [Medline].

  37. van Son JA, Julsrud PR, Hagler DJ, et al. Imaging strategies for vascular rings. Ann Thorac Surg. Mar 1994;57(3):604-10. [Medline].

  38. Weinberg PM. Aortic arch anomalies. In: Emmanouilides G, Reimenschneider T, Allen H, eds. Moss and Adams Heart Disease in Infants, Children and Adolescents. 5th ed. Lippincott Williams & Wilkins; 1995:810-37.

  39. Yoo SJ, Min JY, Lee YH, et al. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol. Nov 2003;22(5):535-46. [Medline].

 
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Schematic diagram (left) of the primitive pharyngeal arch system shows the left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries, fourth (IV) and sixth (VI) pharyngeal arches, distal pulmonary arterial segments (PA), dorsal aortas (DA), and seventh intersegmental arteries (VII). The proximal (p) sixth arches develop into the proximal pulmonary arteries and the distal (d) sixth arches become the arterial ducts. The seventh intersegmental arteries develop into the subclavian arteries. Schematic diagram (right) shows the segments of the pharyngeal arch system that regress (shown in black) in the normal formation of the thoracic great arteries. Left pulmonary artery (LPA); ductus arteriosus (PDA); right pulmonary artery (RPA); subclavian artery (SCA).
Schematic diagram (left) depicts the segments of the pharyngeal arch system that regress (shown in black) so that the mature vascular anatomy of a double aortic arch can develop. The dominant and minor arches can vary in laterality and specific patterns of branching and segmental hypoplasia/atresia. (These variables are not specified in this diagram.) Left (L) and right (R) external carotid (EC) and internal carotid (IC) arteries; fourth (IV) and sixth (VI) pharyngeal arches; distal pulmonary arterial segments (PA); dorsal aortas (DA); seventh intersegmental arteries (VII); proximal (p) sixth arches; distal (d) sixth arches. Mature anatomy (right) of a double aortic arch with a dominant right arch and patent minor left arch. In most patients, a single left-sided ductus arteriosus or ligamentum arteriosum is present. Left pulmonary artery (LPA); ductus arteriosus (PDA); right pulmonary artery (RPA); subclavian artery (SCA).
Transverse MRI images in a patient with double aortic arch. Both arches are patent; the right arch is dominant. Images A-F are arranged in a caudad to cephalad order. (A) Transverse image at the level of the pulmonary valve. The ascending aorta (AAo) and descending aorta (DAo), cephalad to the junction of the left and right arches, can be seen. (B) At the level of the pulmonary artery (PA) bifurcation, the distal confluence of the left and right arches forming the single descending aorta is depicted. (C) The distal portions of the left (L) and right (R) arches can be seen posterior and to the left and right sides of the trachea. Note the anteroposterior compression of the tracheal carina (anterior to and between the arches). (D) Moving cephalad, the dominance of the right arch can be seen. (E) At the level of the proximal/transverse aortic arches, the origin of the left and right arches from the rightward ascending aorta can be seen. (F) The left and right common carotid and subclavian arteries arise from the left and right arches, respectively. The common carotid arteries are the dark round structures anterior to and to either side of the trachea. The subclavian arteries are the dark round structures posterior to and to either side of the trachea.
Coronal spin-echo MRI images in a patient with a double aortic arch. Both arches are patent, with the right (R) slightly larger in caliber than the left (L). Compression of the trachea (T) between the 2 arches can be seen (left). The confluence of the arches and the descending aorta (D) are shown (right).
 
 
 
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