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Double Aortic Arch Clinical Presentation

  • Author: Doff B McElhinney, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 12, 2015
 

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. Note the following:

  • 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.
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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.

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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.[4] 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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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, 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, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

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.

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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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