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

Vascular Ring, Right Aortic Arch: Follow-up

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

Follow-up

Further Inpatient Care

  • Routine postthoracotomy care is provided following division of a vascular ring. Patients are removed from mechanical ventilation as soon as possible, and tube thoracostomy is generally discontinued on the first postoperative day. The remainder of the inpatient stay is focused on determining and managing any residual symptoms, providing sufficient enteral nutrition, transitioning the patient to enteral analgesics, and educating the parents.

Further Outpatient Care

  • Close follow-up should be maintained after division of a vascular ring, especially in young children with persistent tracheomalacia or other respiratory symptoms. Additional imaging or bronchoscopic evaluation is not indicated unless persistent residual symptoms or other mitigating circumstances are present.

Inpatient & Outpatient Medications

  • Aside from analgesic medications, pharmacologic therapy is generally not 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.

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.

Deterrence/Prevention

  • No methods are known that prevent the development of vascular ring with a right aortic arch.

Complications

  • Complications are uncommon after repair of vascular rings.
  • The major postoperative issue 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 is generally 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 general population or whether such pathology in patients with rings has any relationship to their prior anatomic and functional abnormalities is not known.
  • Rarely, patients with tight unrepaired rings may develop aortoesophageal fistula, which may lead to life-threatening hemorrhage.

Prognosis

  • The long-term prognosis for patients with repaired vascular rings is excellent, with persistent respiratory symptoms being the most common adverse outcome.

Patient Education

  • For the early posthospital period, parents must be educated about 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.
  • No dietary or activity restrictions are indicated after repair of an isolated vascular ring.
  • In patients with a repaired vascular ring, lifestyle implications are minimal and are most likely related to residual symptoms or associated anomalies.
  • No special issues or concerns related to pregnancy in patients with repaired vascular ring exist. In patients with an unrepaired ring, pregnancy-induced physiologic changes should not be of special concern, although symptoms may be exacerbated in certain situations.

Miscellaneous

Medicolegal Pitfalls

  • The most significant potential medicolegal pitfall is failure to diagnose and properly define the anatomy of a vascular ring.
 


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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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