• See also
  1. Congenital Heart Disease
  2. Congenital Heart Disease Causes
  • Definitions
  1. Vascular Ring
    1. Developmental defect of the aortic arch system
    2. Trachea and Esophagus are completely encircled by connected segments of the aortic arch and its branches
  • Epidemiology
  1. Symptoms develop in first few months of life
  • Pathophysiology
  1. Large vessels apply local pressure compressing mediastinal structures
    1. Compress trachea and Bronchial tree
    2. Compress Esophagus
  • Causes
  1. Double aortic arch
  2. Aberrant innominate artery
  3. Right aortic arch
  4. Pulmonary Sling
  • Signs
  1. Low pitched inspiratory and expiratory gargling sounds
  2. Inspiratory Stridor
  3. Expiratory Wheezes
  4. Obstructive Sleep Apnea
  5. Feeding difficulties may occur
  6. Sounds present since birth
    1. Established over first month of life
    2. Parent may not be aware of sounds
  7. Provocative maneuvers that increase Dyspnea
    1. Neck flexion
    2. Upper Respiratory Infections
  8. Palliative maneuvers that decrease Dyspnea
    1. Neck extension
  • Radiology
  1. Chest XRay
    1. Note aortic arch location (right or left)
    2. Note tracheal caliber
  2. Barium swallow
    1. Note Esophageal indentation
  • Diagnostics
  1. Bronchoscopy
  2. Angiography
  • Course
  1. Respiratory symptoms worsen with increased age, size
  • Management
  1. Requires surgery
  • Complications
  1. Obstructive Sleep Apnea
  2. Tracheomalacia