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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. Most complex surgery of Congenital Heart Disease repairs
    3. Pulmonary artery and aorta divided and reattached
    4. Atrial Dysrhythmias are less common with this repair method than with prior methods
  • 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]