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Serial follow-up of adults with repaired tetralogy of Fallot
1Division of Cardiology, Toronto Hospital, University of Toronto, Ontario, Canada.
Insights
Adults with repaired tetralogy of Fallot have a good prognosis and low sudden death risk. Ventricular function requires careful monitoring over time for optimal outcomes in repaired tetralogy of Fallot patients.
Area of Science:
- Cardiology
- Adult Congenital Heart Disease
Background:
- Sudden death is a concern in pediatric corrected tetralogy of Fallot.
- This risk may differ in adults with repaired tetralogy of Fallot.
Purpose of the Study:
- To assess clinical outcomes in adults with repaired tetralogy of Fallot.
- To identify risk factors affecting survival in this population.
Main Methods:
- Follow-up study of 151 adult patients with repaired tetralogy of Fallot.
- Serial testing included Holter monitoring, exercise ECG, and radionuclide angiography.
Main Results:
- Low overall mortality (0.009 death/patient-year) with no sudden cardiac deaths.
- Stable exercise capacity and decreasing arrhythmias over follow-up.
- Improved right ventricular ejection fraction, but declining left ventricular ejection fraction within normal limits.
Conclusions:
- Adults with repaired tetralogy of Fallot have a very good prognosis.
- Low risk of sudden death in this cohort.
- Long-term monitoring of ventricular function is essential.
Objectives And Background:
Sudden death has long been considered a major contributor to mortality in pediatric patients with corrected tetralogy of Fallot. However, this may not apply to the patient with repaired tetralogy of Fallot who has survived into adulthood. Consequently we followed up a cohort of such adults to establish the clinical outcome and risk factors affecting their survival.
Methods:
A baseline group of 151 adult patients with repaired tetralogy of Fallot were followed up for a mean of 3.2 years. The overall mortality rate was low (0.009 death/patient-year). Four patients died during follow-up, but only two deaths can be attributed to tetralogy of Fallot repair, and there were no sudden cardiac deaths. Clinically 94% of patients have remained in New York Heart Association functional class I. A subset of 36 patients were followed up for a mean of 6.7 years. This group had three sets of serial testing at 3-year intervals consisting of right heart catheterization at the initial study only, 24-h Holter ambulatory electrocardiographic (ECG) monitoring, exercise ECG and rest and exercise radionuclide angiography.
Results:
Exercise capacity assessed by serial exercise stress testing remained stable over the follow-up period, whereas the presence of exercise-induced arrhythmias steadily decreased. Most patients had no significant arrhythmias and had no significant change in severity of arrhythmia with time. Radionuclide angiography showed significant improvement in exercise right ventricular ejection fraction over time but a progressive decrease in left ventricular ejection fraction at both rest and exercise. However, the left ventricular ejection fraction is still within the normal limits for our laboratory.
Conclusions:
Adults with repaired tetralogy of Fallot have a very good prognosis and a low risk of sudden death. However, ventricular function may change over time and should be carefully monitored.