II. Epidemiology

  1. Symptoms develop in first few months of life

III. Pathophysiology

  1. Large vessels apply local pressure
    1. Compress trachea and Bronchial tree
    2. Compress esophagus

IV. Causes

  1. Double aortic arch
  2. Aberrant innominate artery
  3. Right aortic arch
  4. Pulmonary Sling

V. 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

VI. Radiology

  1. Chest XRay
    1. Note aortic arch location (right or left)
    2. Note tracheal caliber
  2. Barium swallow
    1. Note Esophageal indentation

VII. Diagnostics

  1. Bronchoscopy
  2. Angiography

VIII. Course

  1. Respiratory symptoms worsen with increased age, size

IX. Management

  1. Requires surgery

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Ontology: Origin of left pulmonary artery from right pulmonary artery (C0345041)

Concepts Congenital Abnormality (T019)
SnomedCT 253638007
English Origin LPA from RPA, pulmonary sling, Origin of left pulmonary artery from right pulmonary artery, Pulmonary sling, Origin of left pulmonary artery from right pulmonary artery (disorder)
Spanish origen en la arteria pulmonar derecha de la arteria pulmonar izquierda (trastorno), origen en la arteria pulmonar derecha de la arteria pulmonar izquierda