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Transposition of Great Vessels

Topic updated on 07/17/17 10:25am

                                Anatomic anomaly where the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle.  This leads to pulmonary and systemic circulation existing in parallel.
  • A 24-hour newborn appears blue in all extremities, is cyanotic, and is transferred immediately to the NICU, where he is found to have a single, loud S2 murmur.
  • Anatomic anomaly where the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle
    • leads to pulmonary and systemic circulation existing in parallel 
  • Infant must have collateral arteriovenous communication to be compatable with life
    • patent ductus arteriosus (PDA) 
    • persistent foramen ovale (PFO)
    • atrial or ventricular septal defect
  • Risk factors are
    • Down's syndrome
    • Apert's syndrome
    • cri-du-chat,
    • trisomy 13/18
  • Symptoms
    • newborns are extremely cyanotic (blue skin) and are critically ill
  • Physical exam
    • cyanosis
    • tachypnea
    • progressive respiratory failure
    • early digital clubbing
    • may have no murmur, but a single, loud S2 is characteristic
      • patent ductus arteriosus can lead to characteristic machine like murmur
      • patent foramen ovale has no associated murmur
    • low O2 saturation
      • O2 sat on the right arm will demonstrate a lower preductal saturation than the postductal saturation taken on the lower extremity
  • CXR 
    • show enlarged egg-shaped heart, often referred to as "egg on a string" 
      • due to narrowing of superior mediastinum and convexity of atrial borders
  • Echocardiogram
    • is gold standard for diagnosis
  • Catheterization/MRI
    • may be needed in cases where anatomy is unclear prior to surgery
  • Large VSD, aortic coarctation, tetralogy of fallot, hypoplastic left ventricle
  • Prostaglandin E1 (PGE1)
    • used to keep PDA open until surgical repair is possible
  • Balloon atrial septostomy 
    • (Rashkind procedure) must be performed if immediate surgery is not feasible
      • allow oxygenated blood to travel between the ventricles to get to the aorta.
  • Surgical correction 
    • indicated in order for survival
    • can perform either arterial or atrial switch
Prognosis, Prevention, and Complications
  • Condition is fatal without correction unless patient has a PDA or VSD


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