Tetralogy of Fallot

• Tetralogy of Fallot


  A congenital heart defect characterized by four structural defects of the heart
• Tetralogy of Fallot is one of the most common complex congenital heart defects
• Tetralogy of Fallot occurs when the baby is developing in its mother's womb and some parts of the heart do not form correctly
• According to the CDC, in every 2,518 births one baby in the US will be born with tetralogy of Fallot
• Cyanotic heart defect


  A heart defect that results in decreased pulmonary blood flow and decreased oxygenation of the blood
• Normal blood flow through the heart

  Blood flows from the superior and inferior vena cava to the right atrium, through the tricuspid valve to the right ventricle, through the pulmonic valve to the pulmonary artery, to the lungs, back through the pulmonary veins to the left atrium, through the mitral valve to the left ventricle, and out through the aortic valve to the body
• Tetralogy of Fallot


  • Characterized by 4 structural defects: right ventricular hypertrophy, overriding aorta, pulmonary stenosis, and ventricular septal defect
• Right ventricular hypertrophy


  Occurs due to the resistance against the narrowed pulmonary valve and artery
• Overriding aorta


  The aorta is displaced and positioned over the ventricular septal defect, allowing deoxygenated blood to flow directly into the aorta
• Pulmonary stenosis


  Narrowing of the pulmonary valve and artery, decreasing blood flow to the lungs
• Ventricular septal defect


  A hole in the wall separating the right and left ventricles, allowing deoxygenated blood to mix with oxygenated blood
• Signs and symptoms of tetralogy of Fallot (AFFLICT)

  • Activity intolerance
  • Fingernail changes (clubbing)
  • Fatigue/fainting easily
  • Lift knee to chest position or squat
  • Inability to grow
  • Cardiac sounds (harsh systolic murmur, systolic thrill)
  • Trouble feeding and thriving
• Tet spell


  An episode of cyanosis, shortness of breath, and increased respiratory rate triggered by activity
• Nursing interventions during a Tet spell


  Stop feeding, place in knee-to-chest position, provide oxygen, calm the patient, may administer IV fluids
• Squatting or knee-to-chest position helps improve oxygen levels during a Tet spell by increasing systemic vascular resistance and decreasing the right-to-left shunt
• Prostaglandin E (Alprostadil)

  A medication that keeps the ductus arteriosus open in newborns with tetralogy of Fallot, allowing more blood flow to the lungs
• Treatment for tetralogy of Fallot

  Temporary palliative surgeries (shunt, pulmonary artery/valve procedures) followed by complete surgical repair around 6-12 months of age to correct the structural defects
• Temporary Surgery


  • Shunt/Stent
  • Open heart surgery (patch)
  • Blalock-Taussig (BT) Shunt