The Fontan Circulation: Problems and Solutions

The Fontan circulation has been developed as a strategy for multiple, unrepairable complex cardiac malformations in which there is only one effective ventricle. Almost 50 years after introduction, the Fontan circulation is seen as an important medical progress with good hemodynamic outcome for an otherwise untreatable heart disease. At the same time, complications occur due to challenges associated with a failing circulation due to the physiological features of chronically elevated systemic pressure and decreased cardiac output, obligatory features in the absence of a sub-pulmonary ventricle. As a consequence, patients are at risk for various complications including ventricular failure, organ failure, arrhythmias, protein losing enteropathy and plastic bronchitis. Follow-up studies of Fontan patients have taught us to focus on principles of management and the identification of risk factors to improve outcomes of patients. Despite recent improvements made in tackling these complications, we still face a high morbidity and mortality in this patient group. There is a great need for advancing health care for this unique group of patients. Analyzing these challenges in a problem and potential solution framework will help clarify for practitioners and scientists what we can do today, and what we need to develop going forward.

The research topic on the Fontan circulation 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 implementation of new tools for future care. The focus is on pathophysiology, clinical observations, treatment of complications and investigations which defines our limited knowledge and what needs to be expanded. Evaluations of the use of further screening tools in e.g. liver evaluation tests, advanced imaging, or novel therapies for lymphatic complications could support to achieve treatment advances and finally improve patient quality of life and life expectancy.

This research project will cover and discuss 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 medically treatment, possible complications and measures to prevent or reverse negative outcomes and the development of complications.

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