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

Pulmonary Artery Sling: Treatment & Medication

Author: 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
Contributor Information and Disclosures

Updated: Aug 26, 2008

Treatment

Medical Care

In patients with pulmonary artery sling, medical care is supportive until the patient can undergo definitive surgical correction. 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.

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.3 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.

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.

Consultations

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

Diet

No specific dietary considerations are needed.

Activity

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

Medication

No specific medications directly influence this abnormality. Pneumonia requires appropriate antibiotics.

More on Pulmonary Artery Sling

Overview: Pulmonary Artery Sling
Differential Diagnoses & Workup: Pulmonary Artery Sling
Treatment & Medication: Pulmonary Artery Sling
Follow-up: Pulmonary Artery Sling
References
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References

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

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

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

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

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

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

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

  8. 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. Aug 2003;33(8):567-9. [Medline].

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

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Further Reading

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Keywords

pulmonary artery sling, vascular ring, vascular sling, emphysema, atelectasis, atrial septal defect, patent ductus arteriosus, ventricular septal defect, left superior vena cava, imperforate anus, Hirschsprung disease, biliary atresia, genitourinary defects, airway obstruction, stridor, respiratory distress, cyanosis, pneumonia

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Contributor Information and Disclosures

Author

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.

Medical Editor

Ira H Gessner, MD, Professor Emeritus, Pediatric Cardiology
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, and Society for Pediatric Research
Disclosure: Nothing to disclose.

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 broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

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 College of Cardiology, American Heart Association, American Pediatric Society, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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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