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Fontan's procedure in two stages
1Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Pennsylvania 19104.
Insights
The staged Fontan operation, including hemi-Fontan and completion Fontan procedures, significantly reduces early mortality in single ventricle patients compared to the primary Fontan operation. This approach aims to improve outcomes for complex congenital heart disease.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiovascular Physiology
Background:
- 381 patients with single ventricle physiology treated since 1985.
- The Fontan operation is a palliative procedure for complex congenital heart disease.
- Modifications include staged approaches like hemi-Fontan and completion Fontan.
Purpose of the Study:
- To evaluate the effectiveness of a staged Fontan operation approach.
- To reduce early mortality in patients with single ventricle physiology.
- To minimize intermediate mortality and ventricular strain.
Main Methods:
- Comparison of early mortality between primary Fontan and staged (hemi-Fontan followed by completion Fontan) operations.
- Analysis of mortality rates for hemi-Fontan procedures.
- Systematic staged approach initiated at 6 months (hemi-Fontan) and 12-18 months (completion Fontan).
Main Results:
- Early mortality for completion Fontan (7%) was substantially lower than primary Fontan (16%, p < 0.05).
- Mortality associated with hemi-Fontan operations was 5.5% (7/127 patients) since January 1991.
- Staged approach aims to reduce ventricular volume load and mitigate adverse geometric changes.
Conclusions:
- A systematic staged Fontan operation approach significantly reduces early mortality.
- The staged approach is beneficial for patients with single ventricle physiology.
- Early intervention and staged palliation improve outcomes in complex pediatric cardiac surgery.
Abstract:
Since 1985, 381 patients with various cardiac malformations that share the central common feature of only one effective ventricle have been treated with some modification of Fontan's operation. Since 1989, the Fontan operation has been staged by associating the superior vena cava with the branch pulmonary arteries (hemi-Fontan) initially followed, some months later, by associating the inferior vena cava to the branch pulmonary arteries (completion Fontan). The difference in early mortality for a primary-Fontan operation (16%) compared with a completion-Fontan operation (7%) is substantial (p < 0.05). Since January 1991, mortality associated with the hemi-Fontan operation has been 5.5% (7 deaths in 127 patients). A systematic staged approach to Fontan's operation has been undertaken with a hemi-Fontan operation in patients who are 6 months of age and a completion Fontan operation when those patients are 12 to 18 months of age in an effort to reduce the volume load of the ventricle as early as possible, to minimize intermediate mortality from the palliated state, and to reduce the impact of rapid changes in ventricular geometry and diastolic function that can accompany either the hemi-Fontan or primary Fontan operation, but that are lethal only after a primary Fontan operation.