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Updated: Nov 21, 2025

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Published on: May 2, 2025
Perioperative and long-term management of Fontan patients
May Al-Shawk1, Adeolu Banjoko2, Ariana Axiaq3
1Institute of Medical and Biomedical Education, St George's University of London, London, UK.
Insights
The Fontan procedure, a staged surgery for single-ventricle congenital heart disease (CHD), involves extracardiac total cavopulmonary connection. Postoperative care and monitoring are crucial to prevent acute and late complications, with cardiac transplantation as a definitive treatment.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease (CHD)
- Cardiovascular Surgery
Background:
- Fontan circulation is a palliative surgical approach for single-ventricle congenital heart disease (CHD).
- The extracardiac total cavopulmonary connection is the predominant surgical technique.
- Strict patient selection criteria and comprehensive postoperative management are essential.
Purpose of the Study:
- To review the current perioperative management protocols for Fontan circulation.
- To highlight the acute and late complications associated with the Fontan procedure.
- To discuss potential future research directions for improving patient outcomes.
Main Methods:
- Assessment of Fontan candidates using cardiac catheterization and cardiac magnetic resonance.
- Implementation of postoperative treatment protocols including antibiotic prophylaxis, diuretics, ACE inhibitors, anticoagulation, and oxygen therapy.
- Management strategies involving fluid restriction and low-fat diet.
Main Results:
- Postoperative measures aim to minimize ICU and hospital stay by preventing complications like infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury.
- Late complications include Fontan circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease.
- Cardiac transplantation is the definitive treatment, with emerging innovations in lymphatic embolization and ventricular assist devices.
Conclusions:
- Current perioperative management strategies are critical for mitigating acute and late complications in Fontan patients.
- Further research is needed to standardize perioperative protocols and enhance long-term outcomes.
- Innovations in treatment offer hope for improved management of complex CHD.
Abstract:
A Fontan circulation requires a series of three-staged operations aimed to palliate patients with single-ventricle CHD. Currently, the most frequent technique is the extracardiac total cavopulmonary connection, an external conduit connecting the IVC and right pulmonary artery, bypassing the right side of the heart. Fontan candidates must meet strict criteria; they are assessed utilising both cardiac catheterisation and cardiac magnetic resonance. Postoperatively, treatment protocols prioritise antibiotic prophylaxis, diuretics, angiotensin-converting enzyme inhibitors, anticoagulation, and oxygen therapy with fluid restriction and a low-fat diet. These measures aim to reduce length of stay in the ICU and hospital by preventing acute complications such as infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury. Late complications of a Fontan procedure include circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease. The definitive management is cardiac transplantation, with promising innovations in selective embolisation of lymphatic vessels and Fontan-specific ventricular assist devices. Further research assessing current protocols in the perioperative management of Fontan patients would be beneficial for standardising current practice and improving outcomes.
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