Double Aortic Arch
Double aortic arch is one of the 2 most common forms of vascular ring, a class of congenital anomalies of the aortic arch system in which the trachea and esophagus are completely encircled by connected segments of the aortic arch and its branches. Although the double aortic arch has various forms, the common defining feature is that both the left and right aortic arches are present.
The easiest way to understand the anatomy and development of double aortic arch and other forms of vascular ring is to begin by considering the bilateral system of pharyngeal arch vessels in the early embryo.
Early in the course of embryonic morphogenesis, 6 pairs of pharyngeal arch arteries develop in conjunction with the branchial pouches. The first through sixth arches appear in a fairly sequential fashion, with left-to-right symmetry, and constitute the primitive vascular supply to the brachiocephalic structures, running from the aortic sac to the paired dorsal aortas. As normal cardiovascular morphogenesis proceeds, a patterned regression and persistence of the various arches and right-sided dorsal aorta occur, ultimately resulting in the mature configuration of the thoracic aorta and its branches. The third, fourth, and sixth arches, along with the seventh intersegmental arteries and the left dorsal aorta, are the primary contributors to the normal aortic arch and its major thoracic branches (see diagram below).
The segments of the bilateral aortic arch system that normally regress include the distal portion of the sixth arch and the right-sided dorsal aorta. Normally, the left fourth arch becomes the aortic arch, the right fourth arch contributes to the innominate artery, the distal left sixth arch becomes the ductus arteriosus, the proximal sixth arches bilaterally contribute to the proximal branch pulmonary arteries, the left dorsal aorta becomes the descending thoracic aorta, and the dorsal intersegmental arteries bilaterally become the subclavian arteries.
Vascular rings are formed when this process of regression and persistence does not occur normally, and the resulting vascular anatomy completely encircles the trachea and esophagus. (Other forms of aortic arch anomalies occur in which a vascular ring is not present.) A double aortic arch is formed when both fourth arches and both dorsal aortas remain present.
Double aortic arch has various forms. Both arches may be patent, or an atretic (but persistent) segment may be present at one of several locations in either arch. When both arches are patent, the right or left arch may be larger, or they may be similar in size. A cervical arch on either side, variable laterality of the descending thoracic aorta, coarctation of the major arch, and/or discontinuity of the central pulmonary arteries may be present. In general, the apex of the right-sided arch is more superior than the left arch, and on occasion, a cervical arch may be present on either side.
In more than 75% of patients with double aortic arch, the right arch is dominant. Among patients with a right-dominant double arch, those with a patent minor arch outnumber those with an atretic minor arch. When the minor arch is atretic, the atretic segment almost always is distal to the left subclavian artery, although atresia may also occur between the left common carotid and subclavian arteries. In approximately 20% of patients, the left arch is dominant. In these patients, the minor right arch is typically patent.
Double aortic arch usually occurs without associated cardiovascular anomalies. Ventricular septal defect and tetralogy of Fallot are probably the most common associated defects, although truncus arteriosus, transposition of the great arteries, pulmonary atresia, and complex univentricular defects sometimes occur in conjunction with a double arch.
Double aortic arch is associated with a chromosome band 22q11 deletion in approximately 20% of patients (see Causes). Band 22q11 deletion is responsible for DiGeorge, velocardiofacial, and conotruncal anomaly face syndromes, which are often referred to using the unified terms CATCH-22 syndrome or chromosome band 22q11 deletion syndrome. In patients with double aortic arch, the frequency of phenotypes satisfying the clinical criteria for these various syndromes is not known. Rather, the important point is that double aortic arch may be associated with band 22q11 deletion, which has various other possible manifestations. These include, but are not limited to, palatal abnormalities, laryngotracheal anomalies, speech and learning delay, characteristic facial features, hypocalcemia, abnormalities of T-cell–mediated immune function, and neurologic defects.
Occasionally, patients with double aortic arch may have anomalies consistent with either vertebral, anal, cardiac, tracheal, esophageal, renal, and limb (VACTERL) or posterior coloboma, heart defect, choanal atresia, retardation, genital, and ear (CHARGE) associations. Double aortic arch has also been reported in association with other chromosomal anomalies, such as trisomy 21 and other syndromes.
One of the more important noncardiac features that sometimes is found in association with double aortic arch is esophageal atresia, insofar as an undiagnosed arch anomaly may complicate repair of the esophageal atresia, which is usually recognized earlier than the double aortic arch.
Another noncardiac anomaly that may be associated with vascular rings is a congenital laryngeal web, which may present with the same symptoms and signs as a vascular ring. Accordingly, patients with persistent stridor or upper airway obstruction after repair of a vascular ring, particularly those with a chromosome 22q11 deletion, should be evaluated for the presence of a congenital laryngeal web.
By definition, vascular rings encircle the trachea and esophagus, usually causing compression of both structures. Compression of the trachea causes upper airway obstruction that impairs inspiratory and, to a lesser degree, expiratory airflow.  The extent of respiratory impairment depends on the severity of compression, which can widely vary. Significant compression of the trachea appears to be more common with double aortic arch than with other forms of vascular ring and is often more severe.
In addition to airway symptoms, patients may experience swallowing difficulties related to esophageal compression. These typically manifest as vomiting and feeding intolerance in infants and younger children and as dysphagia later in life. Swallowing dysfunction may contribute to respiratory symptoms as a result of aspiration and/or compression or irritation of the membranous portion of the trachea as a food bolus traverses the area of esophageal obstruction. Although respiratory or esophageal pathophysiology may predominate in any given patient, respiratory compromise usually is more problematic in younger patients. Patients with primarily esophageal symptomatology tend to be older at presentation.  The pathophysiology of double aortic arch does not differ in the various anatomic forms.
Long-term prognosis for patients with repaired double aortic arch is excellent; persistent respiratory symptoms are the most common adverse outcomes.
In patients with a repaired double aortic arch, lifestyle implications are minimal and most likely are related to residual symptoms or associated anomalies.
The natural history of double aortic arch is not well defined. Vascular rings were among the first congenital cardiovascular anomalies repaired surgically, and surgical management has been the standard of care for more than 50 years. Patients with significant airway compression may die as a result of respiratory compromise, but such events are rare.
Preoperative morbidity generally is limited to respiratory symptoms, feeding problems, or both. Some patients may develop recurrent respiratory infections, and some may exhibit failure to thrive as the result of a combination of increased metabolic requirements from respiratory and feeding work and relatively poor oral intake.
Complications are uncommon after repair of vascular rings.
The major postoperative symptom is persistent respiratory symptoms, including cough, dyspnea, and wheezing. Pulmonary function testing reveals persistent upper airway obstruction in some patients. Others have evidence of lower airway obstruction that usually is responsive to bronchodilator therapy. Whether the incidence of lower airway obstruction is higher in patients who have undergone repair of vascular rings than in the population at large or whether such a pathologic condition in patients with rings has any relationship to prior anatomic and functional abnormalities is not known.
Rarely, patients with unrepaired double aortic arch may develop an aortoesophageal fistula, which causes fatal hemorrhage. Another rare complication is esophageal erosion.
Based on limited data, no racial predilection is apparent.
No sex predilection has been documented in patients with double aortic arch or its various subtypes.
Double aortic arch is a developmental abnormality that is present in the fetus. The postnatal age at which the anomaly is identified may vary, although in most patients, double aortic arch is diagnosed in early infancy. Double aortic arch may also be identified on prenatal ultrasonography. 
Generally, incidence of double aortic arch and vascular rings is unknown, although vascular rings comprise an estimated 1% of cardiovascular malformations that are managed surgically. In most surgical series, 45-65% of patients undergoing repair of a vascular ring have a double aortic arch.
Umegaki T, Sumi C, Nishi K, Ikeda S, Shingu K. Airway management in an infant with double aortic arch. J Anesth. 2010 Feb. 24(1):117-20. [Medline].
Noguchi K, Hori D, Nomura Y, Tanaka H. Double aortic arch in an adult. Interact Cardiovasc Thorac Surg. 2012 Feb 28. [Medline].
Tuo G, Volpe P, Bava GL, et al. Prenatal diagnosis and outcome of isolated vascular rings. Am J Cardiol. 2009 Feb 1. 103(3):416-9. [Medline].
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. 2001 Jun 15. 37(8):2114-9. [Medline].
Moral S, Zuccarino F, Loma-Osorio P. Double aortic arch: an unreported anomaly with Kabuki syndrome. Pediatr Cardiol. 2009 Jan. 30(1):82-4. [Medline].
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. 2011 Sep. 19(3):163-6. [Medline]. [Full Text].
Chen X, Qu Y, Peng ZY, et al. Clinical value of multi-slice spiral computed tomography angiography and three-dimensional reconstruction in the diagnosis of double aortic arch. Exp Ther Med. 2014 Aug. 8(2):623-627. [Medline]. [Full Text].
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. 2009 Jul. 44(7):1328-32. [Medline].
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. 2009 Jul. 44(7):1454-7. [Medline].
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. 2002 Dec. 20(6):553-7. [Medline].
Anand R, Dooley KJ, Williams WH, Vincent RN. Follow-up of surgical correction of vascular anomalies causing tracheobronchial compression. Pediatr Cardiol. 1994 Mar-Apr. 15(2):58-61. [Medline].
Angelini A, Dimopoulos K, Frescura C, et al. Fatal aortoesophageal fistula in two cases of tight vascular ring. Cardiol Young. 2002 Mar. 12(2):172-6. [Medline].
Arciniegas E, Hakimi M, Hertzler JH, et al. Surgical management of congenital vascular rings. J Thorac Cardiovasc Surg. 1979 May. 77(5):721-7. [Medline].
Backer CL, Ilbawi MN, Idriss FS, DeLeon SY. Vascular anomalies causing tracheoesophageal compression. Review of experience in children. J Thorac Cardiovasc Surg. 1989 May. 97(5):725-31. [Medline].
Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg. 2005 Jun. 129(6):1339-47. [Medline].
Bertrand JM, Chartrand C, Lamarre A, Lapierre JG. Vascular ring: clinical and physiological assessment of pulmonary function following surgical correction. Pediatr Pulmonol. 1986 Nov-Dec. 2(6):378-83. [Medline].
Bonnard A, Auber F, Fourcade L, et al. Vascular ring abnormalities: a retrospective study of 62 cases. J Pediatr Surg. 2003 Apr. 38(4):539-43. [Medline].
Burke RP, Rosenfeld HM, Wernovsky G, Jonas RA. Video-assisted thoracoscopic vascular ring division in infants and children. J Am Coll Cardiol. 1995 Mar 15. 25(4):943-7. [Medline].
Cerillo AG, Amoretti F, Moschetti R, et al. Sixteen-row multislice computed tomography in infants with double aortic arch. Int J Cardiol. 2005 Mar 18. 99(2):191-4. [Medline].
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. 2004 Aug. 87(8):992-5. [Medline].
Chun K, Colombani PM, Dudgeon DL, Haller JA Jr. Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thorac Surg. 1992 Apr. 53(4):597-602; discussion 602-3. [Medline].
Fleenor JT, Weinberg PM, Kramer SS, Fogel M. Vascular rings and their effect on tracheal geometry. Pediatr Cardiol. 2003 Sep-Oct. 24(5):430-5. [Medline].
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].
Hartenberg MA, Salzberg AM, Krummel TM, Bush JJ. Double aortic arch associated with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 1989 May. 24(5):488-90. [Medline].
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].
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].
Kocis KC, Midgley FM, Ruckman RN. Aortic arch complex anomalies: 20-year experience with symptoms, diagnosis, associated cardiac defects, and surgical repair. Pediatr Cardiol. 1997 Mar-Apr. 18(2):127-32. [Medline].
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. 2007 Mar. 2(2):130-3. [Medline].
Koontz CS, Bhatia A, Forbess J, Wulkan ML. Video-assisted thoracoscopic division of vascular rings in pediatric patients. Am Surg. 2005 Apr. 71(4):289-91. [Medline].
Lillehei CW, Colan S. Echocardiography in the preoperative evaluation of vascular rings. J Pediatr Surg. 1992 Aug. 27(8):1118-20; discussion 1120-1. [Medline].
McElhinney DB, Jacobs I, McDonald-McGinn DM, Goldmuntz E. Chromosomal and cardiovascular anomalies associated with congenital laryngeal web. Int J Pediatr Otorhinolaryngol. 2002 Oct 21. 66(1):23-27. [Medline].
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. 2001 Dec. 108(6):E104. [Medline].
Mihaljevic T, Cannon JW, del Nido PJ. Robotically assisted division of a vascular ring in children. J Thorac Cardiovasc Surg. 2003 May. 125(5):1163-4. [Medline].
Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med. 2006 Feb. 25(2):251-7. [Medline].
Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med. 2006. 25:[Medline].
Picard E, Tal A. Tracheal compression caused by double aortic arch in two sisters. Isr J Med Sci. 1992 Nov. 28(11):799-801. [Medline].
Rimell FL, Shapiro AM, Meza MP, et al. Magnetic resonance imaging of the pediatric airway. Arch Otolaryngol Head Neck Surg. 1997 Sep. 123(9):999-1003. [Medline].
van Son JA, Julsrud PR, Hagler DJ, et al. Imaging strategies for vascular rings. Ann Thorac Surg. 1994 Mar. 57(3):604-10. [Medline].
Weinberg PM. Aortic arch anomalies. 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.
Yoo SJ, Min JY, Lee YH, et al. Fetal sonographic diagnosis of aortic arch anomalies. Ultrasound Obstet Gynecol. 2003 Nov. 22(5):535-46. [Medline].
Doff B McElhinney, MD Assistant Professor of Pediatrics, Harvard Medical School; Associate in Cardiology, Department of Cardiology, Children’s Hospital of Boston
Disclosure: Nothing to disclose.
Gil Wernovsky, MD, FACC, FAAP Professor, Department of Pediatrics, University of Pennsylvania, Children’s Hospital of Philadelphia
Disclosure: Nothing to disclose.
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.
Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine
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, Society for Cardiovascular Angiography and Interventions
Disclosure: Nothing to disclose.
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, International Society for Heart and Lung Transplantation
Disclosure: Received honoraria from Actelion for speaking and teaching.
Double Aortic Arch
Research & References of Double Aortic Arch|A&C Accounting And Tax Services