Vascular Rings and Pulmonary Sling

  • Gaetano Domenico GargiuloEmail author
  • Francesco Petridis
  • Gabriele Egidy Assenza
  • Emanuela Angeli


Vascular rings and pulmonary slings are a group of heterogeneous congenital malformation of the aortic arch, aortic branching vessels, and pulmonary arteries. Their unifying anatomical feature is the ability to encircle the trachea and esophagus with potential for extrinsic compression. The morphological heterogeneity is paralleled by a wide spectrum of clinical presentation, ranging from asymptomatic adult with incidental diagnosis to severe respiratory distress and dysphagia in the neonatal period. These anomalies account for 1 % of congenital cardiovascular malformation and are frequently associated with genetic defects and/or intracardiac congenital anomalies. Diagnostic workup includes routine tests such as chest X-ray and echocardiogram but often requires dedicated advanced cardiovascular imaging including computerized tomography scan, magnetic resonance imaging with angiography, and in selected cases invasive angiography.

Surgical correction is mandatory in symptomatic patients, in patients with potential for progressive compression and damage to the trachea or esophagus, or in patients with indication for surgical treatment of associated cardiac defects. Surgical approach is highly dependent on defect anatomy and may include sternotomy, thoracotomy, or in specific cases video-assisted thoracoscopic surgery. Surgical mortality and morbidity are usually low, and long-term freedom from reoperation is reassuring. Complex pathology of the trachea (such as long segmental stenosis) as seen in pulmonary arterial sling may complicate surgical repair, increasing at the same time the procedure mortality and jeopardizing long-term results.


Aortic Arch Compute Tomography Angiography Subclavian Artery Hypoplastic Left Heart Syndrome Left Pulmonary Artery 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer International Publishing Switzerland 2017

Authors and Affiliations

  • Gaetano Domenico Gargiulo
    • 1
    Email author
  • Francesco Petridis
    • 1
  • Gabriele Egidy Assenza
    • 1
  • Emanuela Angeli
    • 1
  1. 1.Department of Pediatric Cardiac Surgery and GUCH UnitAzienda Ospedaliero-Universitaria S. Orsola-MalpighiBolognaItaly

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