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The Fontan procedure: contemporary techniques have improved long-term outcomes
Yves d'Udekem1, Ajay J Iyengar, Andrew D Cochrane
1Department of Cardiac Surgery, Royal Children's Hopital, Flemington Road, Parkville, Melbourne 3052, Victoria Australia. yves.dudekem@rch.org.au
Insights
Modified Fontan procedures, including lateral tunnel and extracardiac conduits, significantly improve long-term survival and reduce arrhythmias compared to the classical atriopulmonary connection. These advancements enhance outcomes for patients with complex congenital heart disease.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease Surgery
Background:
- The Fontan procedure is a palliative surgical intervention for complex single-ventricle congenital heart defects.
- Classical atriopulmonary connection is a traditional method, while lateral tunnel and extracardiac conduit modifications represent newer techniques.
Purpose of the Study:
- To compare the long-term outcomes of patients undergoing modified Fontan procedures (lateral tunnel and extracardiac conduit) versus the classical atriopulmonary connection.
- To identify risk factors influencing mortality and long-term success after Fontan procedures.
Main Methods:
- Retrospective analysis of 305 consecutive patients who underwent a Fontan procedure between 1980 and 2000.
- Comparison of survival rates, need for reoperation or transplantation, and incidence of arrhythmias between different surgical techniques.
- Identification of independent risk factors for mortality using statistical analysis.
Main Results:
- Overall hospital mortality was 3%, with no deaths occurring after 1990. Preoperative elevated pulmonary artery pressures and common atrioventricular valve were independent risk factors for mortality.
- Twenty-year survival was 84%. Fifteen-year survival was significantly higher for lateral tunnel (94%) compared to atriopulmonary connection (81%).
- Modified Fontan procedures were associated with decreased arrhythmia occurrence and improved freedom from supraventricular tachycardia (SVT). Extracardiac conduits showed no observed deaths, SVT, or failure in follow-up.
Conclusions:
- The Fontan procedure, while palliative, has seen improved outcomes due to better patient selection and surgical modifications.
- Lateral tunnel and extracardiac conduit modifications lead to reduced arrhythmias and delayed Fontan circulation failure.
- These advancements offer better long-term results for patients requiring Fontan palliation.
Background:
To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection.
Methods And Results:
Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12+/-6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed.
Conclusions:
The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.