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Vascular Ring, Double Aortic Arch
Updated: Oct 29, 2009
Introduction
Background
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.
Embryology
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 Media file 1).
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).
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.
Anatomy
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.
Associated cardiovascular anomalies
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.
Associated syndromes and noncardiac conditions
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.
Pathophysiology
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.
Frequency
United States
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.
Mortality/Morbidity
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.
Other rare complications, such as esophageal erosion and aortoesophageal fistula, have been reported.
Race
Based on limited data, no racial predilection is apparent.
Sex
No sex predilection has been documented in patients with double aortic arch or its various subtypes.
Age
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.1
Clinical
History
Presentation of symptoms in patients with double aortic arch depends on several factors, including the severity of tracheal compression, esophageal compression, or both and whether associated anomalies are present.
- Among patients with a vascular ring, those with double aortic arch tend to present earlier than those with other anatomic variations. The classic history in a patient with double aortic arch is noisy breathing noted by the parents during the first few weeks of life.
- Young patients may have experienced episodes that often are termed apparent life-threatening events (ALTE) or death spells, in which acute apneic or severe obstructive events are accompanied by cyanosis. Patients with less severe tracheal compression may give a history of persistent respiratory symptoms without frank stridor, often treated as asthma or bronchiolitis, or a history of recurrent lower respiratory infections.
- Esophageal symptoms include emesis, choking, or dysphagia and are more common in older infants and children than in young infants.
- Occasionally, patients may reach older childhood or adulthood before developing persistent or progressive symptoms of dysphagia, respiratory symptoms, or both.
- Rarely, fetal echocardiography may reveal double aortic arch and types of vascular ring.
- In neonates with associated cardiac or noncardiac anomalies, a double aortic arch may be diagnosed incidentally during the course of evaluation.
Physical
Physical findings can vary, often in accordance with the patient's history.
- Newborns with associated anomalies may have no evidence of a vascular ring on physical examination, but this situation is the exception because most patients have readily recognizable physical signs.
- The classic sign of double aortic arch and of vascular rings in general is nonpositional stridor; however, many young infants with double aortic arch have adventitious expiratory breath sounds, as well as the characteristic inspiratory stridor. Respiratory findings typically do not improve with nebulized bronchodilator therapy and usually are more prominent with agitation or crying.
Causes
A persistent double aortic arch occurs when abnormal regression of the embryonic aortic arch segments is present, in which both the left and right aortic arches remain intact. With the different forms of double aortic arch, different segments of the embryonic aortic arch system, which normally regress, remain patent.- Factors responsible for the aberrant persistence of certain aortic arch segments have not been clearly identified, and the pathogenesis of this anomaly remains a mystery. Double aortic arch typically occurs without associated cardiovascular defects, although other lesions may be present, and accordingly, it is not usually found as part of a syndromic complex.
- In a study at the author's institution, band 22q11 deletions were found in 3 of 22 patients (14%) with double aortic arch.2 This chromosomal anomaly is associated with aortic arch anomalies in patients with other forms of conotruncal heart disease and other isolated vascular abnormalities, and band 22q11 deletion is likely to be an important etiologic factor in double aortic arch. Most such mutations arise de novo, and no recognizable inheritance pattern is present.
- Familial recurrence of double aortic arch has been reported, supporting a genetic etiology for this anomaly. Teratogen-induced double aortic arch in animal models also has been reported. The mechanisms and significance of these models have not been elucidated.
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References
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].
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].
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].
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].
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].
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].
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].
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].
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].
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].
Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg. Jun 2005;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. Nov-Dec 1986;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. Apr 2003;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. Mar 15 1995;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. Mar 18 2005;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. Aug 2004;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. Apr 1992;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. Sep-Oct 2003;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. May 1989;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. Mar-Apr 1997;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. Mar 2007;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. Apr 2005;71(4):289-91. [Medline].
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].
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].
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].
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].
Patel CR, Lane JR, Spector ML, Smith PC. Fetal echocardiographic diagnosis of vascular rings. J Ultrasound Med. Feb 2006;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. Nov 1992;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. Sep 1997;123(9):999-1003. [Medline].
van Son JA, Julsrud PR, Hagler DJ, et al. Imaging strategies for vascular rings. Ann Thorac Surg. Mar 1994;57(3):604-10. [Medline].
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.
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].
Further Reading
Keywords
vascular ring, aortic arch anomaly, double arch, isolated vascular ring, congenital heart defect, congenital aortic defect, double aortic arch, ventricular septal defect, truncus arteriosus, treatment, diagnosis
