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Surgical treatment of complex congenital heart disease with modified Fontan procedure
Insights
The modified Fontan operation offers effective palliation for complex congenital heart defects. While early mortality was 16%, it decreased to 11.7% in the last 5 years, showing improved outcomes.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- The Fontan operation is a palliative procedure for complex single ventricle congenital heart anomalies.
- Historically, treatment options for these conditions were limited.
- The modified Fontan procedure has evolved to expand operability criteria.
Purpose of the Study:
- To report the outcomes of the first 500 modified Fontan operations performed at the Mayo Clinic.
- To identify preoperative and perioperative factors influencing successful outcomes.
- To assess the long-term efficacy and safety of the modified Fontan procedure.
Main Methods:
- Retrospective analysis of 500 patients who underwent a modified Fontan operation between October 1973 and mid-January 1987.
- Categorization of patients based on underlying congenital cardiac anomalies (tricuspid atresia, double inlet ventricle, other complex single ventricles).
- Statistical analysis to identify risk factors for mortality and assess outcomes.
Main Results:
- Overall hospital mortality was 16% (80 deaths), with a 11.7% mortality in the last 5 years of the study period.
- Tricuspid atresia patients had the lowest mortality (11% overall, 5.8% in the last 5 years).
- Significant risk factors included younger age at operation, elevated pulmonary artery and right atrial pressures, and prolonged cardiopulmonary bypass times.
Conclusions:
- The modified Fontan procedure provides effective palliation for complex congenital cardiac anomalies.
- Operative mortality has declined with increasing experience and expanded criteria.
- The procedure demonstrates good late results and appears to increase longevity for patients with functional single ventricle.
Abstract:
Between October 1973 and mid-January 1987, 500 patients underwent a modified Fontan operation at the Mayo Clinic. This included 163 patients with tricuspid atresia, 154 patients with double inlet ventricle, and 183 patients with other complex single ventricle malformations. There have been 80 hospital deaths (16%). In the last 5 years there have been 39 deaths (11.7%). Lowest mortality was seen in patients with tricuspid atresia (11% overall, 5.8% last 5 years) and highest mortality was seen in patients with complex lesions. Younger age at operation, elevated pulmonary artery pressure, elevated right atrial pressure following operation, and prolonged bypass times were significant risk factors. The modified Fontan procedure provides effective palliation for complex congenital cardiac anomalies which have no other satisfactory treatment. Criteria for operability have been extended beyond those originally proposed for this operation. The operative mortality continues to decline with increasing experience, late results are good, and longevity appears to be increased. The Fontan operation has been increasingly utilized as the definitive surgical palliation for patients with functional single ventricle. At the Mayo Clinic, over 600 patients have undergone a modified Fontan operation since our first case in October 1973. This presentation gives the results of the first 500 cases and describes the various preoperative and perioperative factors which may influence a successful outcome.