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Pulmonary Artery Sling Treatment & Management

  • Author: Stuart Berger, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
 
Updated: Mar 04, 2014
 

Medical Care

In patients with pulmonary artery sling, medical care is supportive until the patient can undergo definitive surgical correction. Inpatient care in patients with pulmonary artery sling may be required for symptoms of airway obstruction and/or pneumonia.

Hypoxemia and respiratory distress should be treated with supplemental oxygen and endotracheal (ET) intubation if indicated. Treat pneumonia with appropriate antibiotics.

Stabilize the patient and make arrangements for surgery as soon as possible. Infants without airway obstruction and with minimal symptoms may not require surgical intervention. However, this scenario is the rare exception.

After surgical repair, these symptoms of airway obstruction and/or pneumonia may persist, although they should improve over time.

Consultations

Obtain consultations with the following specialists:

  • Pediatric cardiologist
  • Cardiovascular surgeon experienced in congenital heart defect surgery
  • Radiologist

Transfer

Patients hospitalized with significant stridor, in whom a vascular ring or pulmonary artery sling is suspected, should be transferred to a facility that can provide pediatric cardiology and pediatric cardiovascular surgery services.

Diet and activity

No specific dietary considerations are needed.

Normal activity is indicated after surgical repair unless significant airway obstruction persists.

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Surgical Care

Survival of symptomatic infants is unlikely without early surgical intervention. In 1954, Potts and Hollinger reported the first description of surgical repair of this lesion.[4] Surgery involves division of the anomalous left pulmonary artery and reanastomosis to the main pulmonary artery anterior to the trachea. Although this can be performed from a left thoracotomy, approach from a midline sternotomy with cardiopulmonary bypass may offer more control. The findings from one study, which presented a large series of successful patient management experiences, emphasize the advantage of cardiopulmonary bypass as the treatment of choice.[5]

Mortality varies and has been reported to be relatively high in previous studies. The major contributor to postoperative mortality is the high frequency of bronchial and tracheal abnormalities in this group of patients. Early, aggressive intervention may minimize the mortality caused by these associated lesions. If airway stenosis is severe, patients may require surgical reconstruction of the obstructed bronchi or trachea at the time of reimplantation of the anomalous left pulmonary artery.

Surgical survivors are usually free of significant symptoms at long-term follow-up. However, some degree of airway obstruction may persist in as many as 45% of patients.

Follow-up

Close follow-up care after surgery is required, particularly monitoring for persistent airway obstruction.

Although airway symptoms should improve, follow patients closely, especially during times of upper respiratory infections.

Similarly, monitor this group of patients closely for the possibility of left pulmonary artery stenosis after reimplantation of the vessel. This can be noninvasively assessed with echocardiography but rarely may require follow-up pulmonary artery angiography.

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Contributor Information and Disclosures
Author

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.

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.

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Western Society for Pediatric Research, American College of Cardiology, American Heart Association, American Pediatric Society

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Sade RM, Rosenthal A, Fellows K, et al. Pulmonary artery sling. J Thorac Cardiovasc Surg. 1975 Mar. 69(3):333-46. [Medline].

  2. Newman B, Cho YA. Left Pulmonary Artery Sling-Anatomy and Imaging. Semin Ultrasound CT MR. 2010 Apr. 31(2):158-170. [Medline].

  3. Loukanov T, Sebening C, Springer W, et al. Simultaneous management of congenital tracheal stenosis and cardiac anomalies in infants. J Thorac Cardiovasc Surg. 2005 Dec. 130(6):1537-41. [Medline].

  4. Potts W, Holinger P. Anomalous left pulmonary artery causing obstruction to the right main stem bronchus. JAMA. 1954. 155:1409.

  5. Backer CL, Russell HM, Kaushal S, Rastatter JC, Rigsby CK, Holinger LD. Pulmonary artery sling: Current results with cardiopulmonary bypass. J Thorac Cardiovasc Surg. 2012 Jan. 143(1):144-51. [Medline].

  6. Freedom R, Culham J. The Angiography of Congenital Heart Disease. 1998.

  7. Grover FL, Norton JB Jr, Webb GE, et al. Pulmonary sling. Case report and collective review. J Thorac Cardiovasc Surg. 1975 Feb. 69(2):295-300. [Medline].

  8. Morrow R, Huhta J. Aortic arch and pulmonary artery anomalies. The Science and Practice of Pediatric Cardiology. 1990. 1444-7.

  9. Niwayama G. Unusual vascular ring formed by the anomalous left pulmonary artery, with tracheal compression. Am Heart J. 1960 Mar. 59:454-61. [Medline].

  10. Semple MG, Bricker L, Shaw BN, Pilling DW. Left pulmonary artery sling presenting as unilateral echogenic lung on 20-week detailed antenatal ultrasound examination. Pediatr Radiol. 2003 Aug. 33(8):567-9. [Medline].

  11. Tesler UF, Balsara RH, Niguidula FN. Aberrant left pulmonary artery (vascular sling): report of five cases. Chest. 1974 Oct. 66(4):402-7. [Medline].

 
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Aberrant left pulmonary artery or pulmonary artery sling.
 
 
 
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