Double Aortic Arch Follow-up

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

Further Inpatient Care

  • Routine postthoracotomy care is provided following repair of double aortic arch.
  • Patients are removed from mechanical ventilation as soon as possible, and tube thoracostomy usually is discontinued on the first postoperative day.
  • The remainder of the inpatient stay is focused on determining and managing any residual symptomatology, providing sufficient enteral nutrition, transitioning the patient to enteral analgesics, and educating the parents.
  • In patients with residual or recurrent obstruction of the airways, aortopexy may relieve the compression and associated symptoms.[8]
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Further Outpatient Care

  • Close follow-up observation should be maintained after repair of double aortic arch, especially in young children with persistent tracheomalacia or other respiratory symptoms.
  • Additional imaging or bronchoscopic evaluation is not indicated unless residual symptoms persist or other mitigating circumstances are present.
  • No dietary or activity restrictions are indicated after repair of an isolated vascular ring.
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Inpatient & Outpatient Medications

  • Aside from analgesic medications, pharmacologic therapy is not usually required after removal from mechanical ventilation. Relief from the pain of the thoracotomy incision may be achieved with age-appropriate narcotic and nonsteroidal anti-inflammatory medications.
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Transfer

  • After postoperative stabilization in the intensive care unit and removal from mechanical ventilatory support, the patient may be transferred to the regular inpatient care area for advancement of feedings and additional postoperative care.
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Deterrence/Prevention

  • No methods are known to prevent development of double aortic arch.
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Complications

  • 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.
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Prognosis

  • 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.
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Patient Education

  • For the early posthospital period, educate parents concerning the possible persistence of symptoms, the potential benefit of prone positioning in patients with tracheomalacia, signs and symptoms of aspiration, and management of the thoracotomy incision.
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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.

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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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