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Published on: May 2, 2025
The failing Fontan: options for surgical therapy
1Washington University School of Medicine, St. Louis Children's Hospital, St. Louis, MO, USA. Huddlestonc@wush.edu
Insights
The Fontan procedure treats single ventricle anomalies but can fail early or late. Late Fontan circulation failure may require reoperation or cardiac transplantation for improved outcomes.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- The Fontan procedure is a critical intervention for single ventricle anomalies.
- Despite its success, Fontan circulation can experience acute or late failure.
- Understanding failure modes is crucial for patient management.
Purpose of the Study:
- To review the limitations and failure patterns of the Fontan procedure.
- To discuss the management strategies for early and late Fontan failure.
- To highlight the role of reoperation and cardiac transplantation.
Main Methods:
- Literature review of Fontan procedure outcomes.
- Analysis of factors contributing to early and late Fontan failure.
- Discussion of clinical presentation and management options.
Main Results:
- Early Fontan failure is linked to myocardial injury or high pulmonary vascular resistance, often with high mortality.
- Late Fontan failure can manifest as heart failure symptoms or protein-losing enteropathy.
- Various factors contribute to late failure, with many amenable to reoperation.
Conclusions:
- Identifying hemodynamic factors is key for managing late Fontan failures.
- Reoperation offers potential solutions for many late failure cases.
- Cardiac transplantation remains a likely necessity for a significant proportion of Fontan patients.
Abstract:
The Fontan procedure has been a significant contribution to the therapeutic armamentarium of congenital heart surgeons for the treatment of single ventricle anomalies. However, it has its limitations. Patients may have failure of the Fontan circulation acutely postoperatively or late during the long-term follow-up. Early failure is often related to an intraoperative myocardial injury or unsuspected elevated pulmonary vascular resistance; this has a very high mortality. Late failure of the Fontan procedure may present with symptoms typical for heart failure. However, protein-losing enteropathy is another condition related to late failure of the Fontan operation. Late failure may be related to a number of different factors, many of which are amenable to reoperation. Every effort should be made to identify which hemodynamic factors are responsible for late failures. However, cardiac transplantation will likely be necessary for the majority of patients.
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