Research Topic

Long-Term Follow-Up in Fontan Patients and Possible Treatment Strategies in Complications

About this Research Topic

The Fontan circulation has been developed as a strategy for multiple, unrepairable complex cardiac malformation. Almost 50 years later, the Fontan circulation is viewed as a medical advance with a good hemodynamic outcome in otherwise untreatable heart diseases. At the same time, complications occur due to challenges associated with a failing circuit due to chronically elevated systemic pressure and a decreased cardiac output, as there is no sub-pulmonary ventricle. As a consequence, patients are at risk of various complications, including ventricular failure, organ failure, arrhythmia, protein losing enteropathy, and plastic bronchitis. Follow-up studies of Fontan patients have taught us to focus on principles of treatment and the identification of risk factors, exercise intolerance and premature death, to improve the outcome in patients. Despite recent improvements in tackling these potential complications, we have reached the limits of classical treatment strategies and continue to face high morbidity and mortality in this patient group. Therefore, Fontan follow-up needs to provide up-to-date management to address risk factors and improve approaches to care.

This Research Topic aims to focus on complications occurring in the long-term follow-up. Research is encouraged to address issues of detecting and treating various complications and implement new tools for future care. The focus is on pathophysiology, clinical observations, treatment of complications, and investigation related to areas with limited knowledge. The evaluations of the use of further screening tools, e.g. liver function tests, advanced imaging, and novel therapies for lymphatic complications, could support advanced treatment and improve patients' life quality and expectance.

This article collection will cover various aspects of the Fontan circulation in children and adults with congenital heart disease. This includes the indications, technical aspects of the operation and circuit, associated medical treatment, possible complications, and measures to prevent an adverse outcome with the development of complications.

• Specificity of the Fontan pathophysiology/hemodynamics
• Underlying congenital heart disease and their requirements associated with the Fontan circulation
• Timing of operative treatment, surgical and technical issues
• Exercise tolerance in Fontan patients
• Psychosocial development in Fontan patients
• Imaging in Fontan patients
• Ventricular failure in Fontan patients
• High pulmonary vascular resistance and the Fontan procedure
• Failing Fontan and therapy options
• Protein losing enteropathy and plastic bronchitis
• Liver failure in Fontan patients
• Quality of life
• Transplantation after Fontan circulation.


Keywords: Congenital heart disease, univentricular heart, TCPC, pulmonary vascular resistance, ventricular dysfunction, exercise tolerance, transplantation


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

The Fontan circulation has been developed as a strategy for multiple, unrepairable complex cardiac malformation. Almost 50 years later, the Fontan circulation is viewed as a medical advance with a good hemodynamic outcome in otherwise untreatable heart diseases. At the same time, complications occur due to challenges associated with a failing circuit due to chronically elevated systemic pressure and a decreased cardiac output, as there is no sub-pulmonary ventricle. As a consequence, patients are at risk of various complications, including ventricular failure, organ failure, arrhythmia, protein losing enteropathy, and plastic bronchitis. Follow-up studies of Fontan patients have taught us to focus on principles of treatment and the identification of risk factors, exercise intolerance and premature death, to improve the outcome in patients. Despite recent improvements in tackling these potential complications, we have reached the limits of classical treatment strategies and continue to face high morbidity and mortality in this patient group. Therefore, Fontan follow-up needs to provide up-to-date management to address risk factors and improve approaches to care.

This Research Topic aims to focus on complications occurring in the long-term follow-up. Research is encouraged to address issues of detecting and treating various complications and implement new tools for future care. The focus is on pathophysiology, clinical observations, treatment of complications, and investigation related to areas with limited knowledge. The evaluations of the use of further screening tools, e.g. liver function tests, advanced imaging, and novel therapies for lymphatic complications, could support advanced treatment and improve patients' life quality and expectance.

This article collection will cover various aspects of the Fontan circulation in children and adults with congenital heart disease. This includes the indications, technical aspects of the operation and circuit, associated medical treatment, possible complications, and measures to prevent an adverse outcome with the development of complications.

• Specificity of the Fontan pathophysiology/hemodynamics
• Underlying congenital heart disease and their requirements associated with the Fontan circulation
• Timing of operative treatment, surgical and technical issues
• Exercise tolerance in Fontan patients
• Psychosocial development in Fontan patients
• Imaging in Fontan patients
• Ventricular failure in Fontan patients
• High pulmonary vascular resistance and the Fontan procedure
• Failing Fontan and therapy options
• Protein losing enteropathy and plastic bronchitis
• Liver failure in Fontan patients
• Quality of life
• Transplantation after Fontan circulation.


Keywords: Congenital heart disease, univentricular heart, TCPC, pulmonary vascular resistance, ventricular dysfunction, exercise tolerance, transplantation


Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.

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Submission Deadlines

15 January 2021 Abstract
15 May 2021 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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Topic Editors

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Submission Deadlines

15 January 2021 Abstract
15 May 2021 Manuscript

Participating Journals

Manuscripts can be submitted to this Research Topic via the following journals:

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