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Transposition of the Great Vessels

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Transposition of the Great Vessels

  • Epidemiology
  1. Incidence: 5% of Congenital Heart Disease
  2. Males affected more often than females by 3:1
  • Pathophysiology
  1. Aorta and pulmonary arteries are transposed
  2. Pulmonary and systemic circulation are separate
    1. Parallel, rather than sequential
  • Associated conditions
  1. Ventricular Septal Defect (30-35%)
  2. Pulmonic stenosis (5%)
  3. Pulmonic stenosis with VSD (10%)
  4. Aortic Coarctation (5%)
  • Signs
  1. Cyanosis if ventricular septum intact
    1. Cyanosis onset in delivery room: 50%
    2. Cyanosis onset within 24 hours of life: 90%
  2. Single S2 Heart Sound
  • Imaging
  1. Chest XRay (Classic triad) - Egg on a string appearance
    1. Egg shaped cardiac silhoutte
    2. Narrow mediastinum
    3. Increased pulmonary markings
  2. Echocardiogram
  • Management
  • Initial
  1. Ductus-Dependent
    1. Keep ductus arteriosus open
    2. Administer prostaglandins (PGE1)
  2. Correct metabolic abnormalities
  3. Correct severe Hypoxia
  • Management
  • Surgical
  1. Balloon atrial septoplasty
    1. Temporizing method
  2. Arterial switch operation
    1. Procedure of choice
    2. Pulmonary artery and aorta divided and reattached
  • Prognosis
  1. Five year survival after arterial switch: 82%
  • Complications associated with surgical repair
  1. Pulmonic stenosis
  2. Aortic Stenosis
  3. Coronary Artery obstruction
  4. Ventricular dysfunction
  5. Arrhythmia
  6. Mitral Regurgitation
  • References
  1. Cyran (1998) PREP review lecture, October, Phoenix
  2. Merenstein (1994) Pediatrics, Lange
  3. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]