eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Vascular Ring, Right Aortic Arch: Treatment & Medication

Author: Doff B McElhinney, MD, Assistant Professor of Pediatrics, Harvard Medical School; Associate in Cardiology, Department of Cardiology, Children's Hospital of Boston
Coauthor(s): Gil Wernovsky, MD, FACC, FAAP, Professor, Department of Pediatrics, University of Pennsylvania, Children's Hospital of Philadelphia
Contributor Information and Disclosures

Updated: Oct 29, 2009

Treatment

Medical Care

  • Medical care before surgical repair depends on the clinical presentation. In most cases, only supportive management is required.
  • Catheter interventions are not used in the management of vascular ring formed by a right aortic arch.

Surgical Care

  • Indications: Surgical division of the vascular ring is indicated in any patient with symptoms of airway or esophageal compression and in patients undergoing surgery for repair of associated cardiovascular or thoracic anomalies.
  • Techniques: The fundamental principle of surgical management of vascular rings is division of the ring to relieve compression of the trachea and esophagus.
    • In patients with all forms of vascular ring with a right aortic arch and a left-sided ductus arteriosus or ligamentum arteriosum, this is achieved by dividing the ductus/ligamentum through a left thoracotomy. Dissection should be carried down to the trachea and esophagus to ensure that no constricting fibrous bands remain. In patients with an aberrant subclavian artery in whom residual posterior compression of the trachea or esophagus is a concern, arteriopexy may be performed by suturing the retroesophageal diverticulum to the prevertebral fascia.
    • Video-assisted thoracoscopic division of vascular rings is performed at several centers and seems to be an effective approach in most cases, but data on outcomes are limited. Robotically assisted division of vascular rings has also been reported.
    • When associated intracardiac anomalies require surgery through a median sternotomy, division of the ductus/ligamentum is performed during the same procedure.
  • Results: In the current era, essentially no operative mortality is associated with repair of isolated vascular rings.3 Outcomes in patients with associated anomalies depend on the coexisting condition.
  • Postoperative care and precautions: Postoperative care after division of a vascular ring is similar to that for patients undergoing other cardiovascular procedures through a thoracotomy. Most patients experience immediate relief after surgery, although persistent respiratory symptoms and signs may be present, especially in very young infants with severe symptoms preoperatively. Except in patients undergoing concurrent repair of associated anomalies, cardiopulmonary bypass is not used during the repair; therefore, postoperative cardiac function is generally not a problem.
  • Complications: Complications in the early postoperative period are uncommon after division of a vascular ring.
    • The major issue is persistent respiratory symptoms, especially in neonates who are more susceptible to tracheomalacia. Occasionally, the tracheomalacia is severe. However, this tends to improve with relief of the vascular ring over time.
    • Other rare operative complications include chylothorax due to injury to the thoracic duct, diaphragmatic paresis/paralysis secondary to phrenic nerve injury, and vocal cord paresis/paralysis resulting from injury to the recurrent laryngeal nerve.

Consultations

  • Unless specific associated anomalies or problems are identified, consultations are generally not necessary. As noted above, band 22q11 deletion is present in a substantial proportion of patients with vascular rings.
  • A consultation with a geneticist is indicated in patients with other characteristic features of the band 22q11 deletion syndrome and may be appropriate in young infants, in whom typical features of the syndrome may not yet be evident.

Diet

  • No special dietary considerations are indicated in patients with a vascular ring other than those dictated by associated conditions. Postoperatively, enteral feeding is resumed as soon as possible.
  • In patients with dysphagia or emesis as a presenting symptom, adequate oral intake should be verified and feeding therapy instituted if necessary.
  • In patients with band 22q11 deletion, velopharyngeal insufficiency or cleft palate is frequently present; oral feedings should be resumed with the aid of feeding specialists.

Activity

  • Patients with vascular rings are not subject to specific restrictions on activity.
  • Prior to repair, their activity may be limited by symptoms.
  • Following repair, any persistent respiratory symptoms due to tracheomalacia should dictate activity limitations.

Medication

  • Aside from analgesia, pharmacologic therapy is not typically required in patients with a vascular ring with a right aortic arch unless associated conditions are present.

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References
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References

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Further Reading

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Keywords

vascular ring, aorta, right aortic arch, trachea, esophagus, left-sided ductus arteriosus, ventricular septal defect, DiGeorge syndrome, velocardiofacial syndrome, conotruncal anomaly face syndrome, treatment, diagnosis

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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.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
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

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

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.

CME Editor

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

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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