Prognosis

If no surgical treatment:

• 30% die in infancy
• 75% die by the age of 10 years

If definitive surgery:

• treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%)
  • however, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions
  • right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis
    • performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons

Reference:

• van der Ven JPG, van den Bosch E, Bogers AJCC and Helbing WA. Current outcomes and treatment of tetralogy of F1000Research 2019, (F1000 Faculty Rev):1530.