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

Pulmonary Artery Sling: Differential Diagnoses & Workup

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

Differential Diagnoses

Stridor
Vascular Ring, Double Aortic Arch
Vascular Ring, Right Aortic Arch

Other Problems to Be Considered

Mediastinal mass
Tracheal stenosis
Tracheomalacia

Workup

Imaging Studies

In pulmonary artery sling, the following imaging studies may be indicated:

  • Chest radiography: The lower trachea is deviated to the left and may appear compressed on its right side. Hyperinflation of the right lung may be seen because of impingement on and compression of the right main stem bronchus. The left lung also may appear hyperinflated because of obstruction at the level of the carina and the left main stem bronchus. In patients with severe obstruction, atelectasis of a single lung or single lobe may be observed. The lateral view may reveal a density anterior to the esophagus and posterior to the trachea just above the carina. This is the left pulmonary artery viewed on end.
  • Barium swallow: The barium swallow is the diagnostic procedure of choice. An anterior indentation of the esophagus on the lateral projection is diagnostic of pulmonary artery sling. The esophagus is usually displaced to the right in the frontal projection and demonstrates an oblique impression immediately posterior to the carina.
  • Echocardiography: In the usual coronal-plane suprasternal-notch images, absence of normal bifurcation of the main pulmonary artery into right and left pulmonary arteries is visible. The left pulmonary artery appears to be absent. However, examination of the right pulmonary artery reveals the left pulmonary artery arising from its posterior surface. The left pulmonary artery can be followed posterior to the trachea and anterior to the esophagus. It passes anterior to the descending aorta and enters the hilum of the left lung. Echocardiography also reveals any associated congenital heart defects.

Bronchoscopy is generally not recommended. If performed, tracheal compression is noted, and accompanying tracheomalacia, tracheal stenosis, or both is common.2 Some recommend bronchoscopic evaluation of selected surgical candidates because surgical reconstruction of the trachea or bronchi may be necessary if the airway compromise is severe.

Currently, MRI or magnetic resonance angiography, CT scanning, or a combination can be helpful in delineating the details of the anatomy, as well as in 3-dimensional reconstruction of the anatomy of the sling as it relates to the airway anatomy.

Procedures

  • Cardiac catheterization and angiography: Diagnosis can usually be noninvasively established. Pulmonary artery angiography may be advisable to delineate anatomic details prior to surgical correction. This permits diagnosis of variations in right and left pulmonary artery anatomy that may not be apparent from noninvasive studies and that can alter the surgical approach. Origin and course of the anomalous left pulmonary artery is best revealed by contrast injection into the main pulmonary artery filming in an oblique view with steep cranial angulation of 60-70 º.

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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