Cardiovascular Medicine Book

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Tetrology of Fallot

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  1. See also
    1. Congenital Heart Disease
    2. Congenital Heart Disease Causes
  2. Epidemiology
    1. Incidence: 10-15% of Congenital Heart Disease
    2. Most common cause of CHD outside infancy
  3. Pathophysiology
    1. Cyanotic Congenital Heart Disease
    2. Primary features responsible for symptoms
      1. Large Ventricular Septal Defect (VSD)
      2. Right outflow tract obstruction
    3. Other features
      1. Right Ventricular Hypertrophy
      2. Overriding aorta
  4. Associated conditions
    1. Right aortic arch (25%)
    2. Atrial Septal Defect (15%)
    3. Absent pulmonary valve
    4. Pulmonary atresia
    5. Pulmonary stenosis
    6. Atrioventricular septal defect
    7. Left superior vena cava to coronary sinus
  5. Presentation signs and symptoms: "Tet spell"
    1. Hypercyanotic, intermittent episode
    2. Occurs in early morning with awakening
    3. Symptoms
      1. Hyperpnea
      2. Irritibility
      3. Cyanosis
  6. Signs
    1. Systolic ejection murmur at left sternal border
      1. Grade I to III intensity
      2. Murmur diminishes in intensity during "Tet Spell"
    2. Single S2 Heart Sound
  7. Electrocardiogram
    1. Right Axis Deviation
    2. Right Ventricular Hypertrophy
  8. Labs
    1. Arterial Blood Gas
      1. Normal arterial pH
      2. Normal arterial pCO2
      3. Low arterial pO2
  9. Radiology
    1. Chest XRay
      1. Heart size normal or small
      2. Narrow mediastinum
    2. Echocardiogram
      1. Large Ventricular Septal Defect (VSD)
      2. Right outflow tract obstruction
      3. Right Ventricular Hypertrophy
      4. Overriding aorta
  10. Management: Conservative of "Tet Spells"
    1. Squatting maneuver or knee chest position
      1. Decreases venous return and excessive preload
    2. Oxygen supplementation
    3. Morphine Sulfate
      1. Morphine 0.1 to 0.2 mg/kg SQ
      2. Quites child and reduces Tachypnea
    4. Volume expansion
    5. Beta Blocker
      1. Acute: Propranolol 0.05 to 0.01 mg/kg IV
      2. Chronic: Propranolol 1 to 4 mg/kg/day PO
  11. Management: Surgery
    1. Subclavian to pulmonary artery shunt (Blalock-Taussig)
      1. Palliative or temporizing method
    2. Definitive, total repair
      1. Repair as newborn or at age 3 years
      2. Ventricular Septal Defect patch
      3. Right ventricular outflow tract widening
  12. Complications: Without repair (inoperable cases)
    1. Affects related to:
      1. Hypoxia
      2. Polycythemia
    2. Symptoms
      1. Headache
      2. Altered Level of Consciousness
      3. Cerebrovascular Accident
      4. Epistaxis
      5. Hemoptysis
      6. Hyperuricemia and Gout
  13. Complications: Post-surgical correction
    1. Arrhythmia
    2. Right ventricular insufficiency
      1. Right Ventricular failure
      2. Pulmonary insufficiency
    3. Left ventricular insufficiency
      1. Left Ventricular Failure
      2. Aortic Insufficiency
  14. Prognosis
    1. Survival post-surgical repair: >95%
    2. Cognitive development may be impaired
  15. References
    1. Saenz (1999) Am Fam Physician 59(7):1857
    2. Cyran (1998) PREP review lecture, October, Phoenix
    3. Merenstein (1994) Pediatrics, Lange

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