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The Fontan operation. Ventricular hypertrophy, age, and date of operation as risk factors
Insights
The Fontan operation offers a 63% survival rate at 6 years for various cardiac malformations. Early postoperative risks include elevated right atrial pressure and ventricular hypertrophy, but younger age is no longer a contraindication.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
Background:
- The Fontan operation is a palliative procedure for complex congenital heart defects.
- Previous palliative surgeries were common in patients undergoing the Fontan procedure.
Purpose of the Study:
- To evaluate the long-term survival and risk factors associated with the Fontan operation.
- To identify predictors of early and late mortality after the Fontan procedure.
Main Methods:
- Retrospective analysis of 102 patients who underwent the Fontan operation between 1975 and 1985.
- Actuarial survival analysis and hazard function assessment.
- Correlation of clinical parameters (age, pressures, ventricular hypertrophy) with mortality.
Main Results:
- Overall 6-year actuarial survival was 63%, with higher survival (81%) in patients with tricuspid atresia.
- Elevated right atrial pressure (>14 mm Hg) and ventricular hypertrophy were significant risk factors for early and late death.
- Younger age was a risk factor for early death, but this was mitigated by recent surgical dates.
Conclusions:
- The Fontan operation demonstrates a significant long-term survival benefit.
- Risk stratification should consider right atrial pressure and ventricular hypertrophy.
- While optimal age is young (2-4 years), older age is not an absolute contraindication.
Abstract:
One hundred two patients, aged 0.7 to 38 years, with a wide variety of cardiac malformations underwent the Fontan operation (1975 to April, 1985). Several different techniques were used. All but 17 had previously undergone one or more palliative operations. Follow-up information was obtained in all patients. Overall actuarial survival rate, with time zero being the time of the operation, was 63% at 6 years with no deaths after that in patients followed as long as out to 9.4 years; that for patients with tricuspid atresia was 81%. The hazard function (instantaneous risk) for death was highest immediately after operation and merged after about 6 months with a constant hazard extending as long as the patients were followed. Elevated post-repair right atrial pressure was correlated (p = 0.002) with the probability of death in the early phase, with the risk rising rapidly with pressures above 14 mm Hg. Hypertrophy of the ventricular main chamber was a risk factor for death in both the early (p = 0.007) and late (p = 0.008) phases of hazard, which explained in part the lesser risk of the Fontan operation in patients with tricuspid atresia. Younger age, but not older age, was a risk factor for early postoperative death, but this risk was neutralized by recent date of operation. Thus currently there is not a predicted increased risk associated with younger age at operation. In general, the Fontan operation should be done at a young age (2 to 4 years) to avoid increasing ventricular hypertrophy, but older age per se is not a contraindication to the operation.