From the Guidelines
Management of failed Fontan circulation should prioritize heart transplantation as the definitive curative treatment option, with mechanical circulatory support as a bridge to transplantation, as indicated by the most recent evidence from 2023 1. The management of failed Fontan circulation is a complex and multifaceted process, requiring a comprehensive approach to address the underlying pathophysiology.
Key Management Strategies
- Initial management includes diuretics like furosemide (20-80 mg daily) or spironolactone (25-100 mg daily) to reduce congestion, and afterload reduction with ACE inhibitors such as enalapril (2.5-20 mg twice daily) or lisinopril (5-40 mg daily) 1.
- Pulmonary vasodilators like sildenafil (20 mg three times daily) or bosentan (62.5-125 mg twice daily) may improve pulmonary blood flow, with bosentan showing a modest but significant increase in peak oxygen consumption and exercise duration in a randomized trial 1.
- Antiarrhythmic medications or catheter ablation are often necessary for rhythm control, and anticoagulation with warfarin (target INR 2-3) may help prevent thromboembolic complications, although routine use of anticoagulation cannot be strongly recommended due to limited evidence 1.
- For protein-losing enteropathy, dietary modifications, albumin infusions, and budesonide (9 mg daily) may provide relief.
Advanced Failure Management
In advanced failure, heart transplantation or heart-liver transplantation represents definitive therapy, with mechanical circulatory support serving as a bridge to transplantation 1. Regular monitoring of liver function, coagulation parameters, and cardiac function is essential, as Fontan failure often involves multiple organ systems due to chronic passive congestion. Early recognition and intervention are critical to prevent irreversible end-organ damage and improve quality of life in these complex patients.
Surgical/Interventional Options
Surgical or catheter intervention may be alternatives to transplantation for a “failing Fontan” after weighing risks and benefits of the intended procedure, including conversion to TCPC, closure of fenestration, and treatment of AV malformations 1.
From the Research
Management Options for Failed Fontan Procedure
The management options for a failed Fontan procedure are varied and depend on the underlying cause of the failure. Some of the options include:
- Medical treatment, such as pulmonary vasodilator and/or systemic anti-inflammatory therapy with budesonide 2
- Surgical or interventional treatment for haemodynamic restrictions 2
- Heparin treatment for protein-losing enteropathy 3
- Traditional heart failure medications, such as renin-angiotensin-aldosterone system blockers and beta-blockers, diuretics, antiarrhythmics, and phosphodiesterase-5 inhibitors 4
- Mechanical circulatory support for patients with ventricular dysfunction 4
- Cardiac transplantation, which remains the definitive treatment for circulatory failure after Fontan surgery 2, 4, 5
- Novel lymphatic interventions, which hold potential for remission with good results 6
Considerations for Management
When managing a failed Fontan procedure, it is essential to consider the underlying etiology of the failure, as well as the patient's overall clinical condition. Some factors that may influence management decisions include:
- Presence of protein-losing enteropathy, which can be a challenging condition to treat 2, 3, 6
- Presence of ventricular dysfunction, which may require mechanical circulatory support 4
- Presence of haemodynamic restrictions, which may require surgical or interventional treatment 2
- Patient's overall functional status and quality of life, which can impact treatment decisions 4, 5