Tetralogy of Fallot

  1. Pulmonary Stenosis
  2. RV Hypertrophy
  3. Overriding aorta
  4. VSD

“Tet Spells”

  • Agitation/irritability, hyperpnea, profound cyanosis, syncope
  • Dynamic obstruction to pulm blood flow
  • Tx: Oxygen, volume, sedation, positioning (knee-chest/squat) 
  • Other options if above fail: Bicarb, phenylephrine

Pre-operative pathophysiology

  • Physiology dependent on pulmonary blood flow
  • Minimum obstruction : L → R shunt through VSD
    • Symptoms: Pulm overcirculation, CHF
  • Severe obstruction:  R→ L shunt
    • Symptoms: Hypoxic, sat 70-80% 
  • Balanced obstruction: Enough PS to prevent over-circulation, sats ~90%    

Key Questions to ask surgeon

  1. How was the RVOT repaired? (transannular vs. valve sparing)
  2. What did the RV and PAs look like? Any abnormal anatomy?
  3. Post operative TEE findings?

Post-operative issues

  • Residual VSD – May have hemodynamic instability, high atrial pressures
  • RV Outflow tract obstruction –   Usually causes more long term issues, ventricular arrhythmias, and possible need for repeat repair
  • RV Dysfunction – Consider if incision was made in RV or pulm regurg
  • Arrhythmias – JET, ventricular ectopy, complete heart block