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. Author manuscript; available in PMC: 2019 Sep 1.
Published in final edited form as: Cardiol Young. 2018 Dec 21;29(3):303–309. doi: 10.1017/S104795111800224X

Table 3. Final list of research priorities determined by the workshop (stage 3).

Category Research question
Associated co-morbidities What are the outcomes in children with multiple co-morbidities?
What are the common co-morbidities in children with a single ventricle heart condition?
What is the impact of syndromes or other co-morbidities on the quality of life of those with a single ventricle heart condition and their families?

Brain & neurodevelopment What factors influence neurodevelopmental outcomes in children with single ventricle heart conditions?
Are there differences in brain development in the fetus with a single ventricle heart condition and if so, what is the impact on outcomes?
What is the impact of a single ventricle heart condition on neurodevelopment?

Exercise What are the effects of regular physical exercise on well-being and long-term outcome in the Fontan circulation?
What type of exercise is most beneficial in those with a Fontan circulation?
What are the benefits of exercise rehabilitation in well and deteriorating patients with a Fontan circulation?

Fontan failure What are the best markers of deterioration in patients with a Fontan circulation?
What is the main mechanism behind failure of the Fontan circulation?
How can we prevent late multi-organ dysfunction in the Fontan circulation?

Heart function What is the cause of ventricular dysfunction in the single ventricle?
How can ventricular function be best preserved in patients with a single ventricle heart condition?
What treatments can be developed for ventricular failure in patients with a Fontan circulation?

Living with a single ventricle heart condition What are the frequencies of symptoms, limitations, physical and emotional quality of life with a Fontan circulation?
What lifestyle choices (eg. diet, exercise, occupation, physical location) best support the long-term health of patients with a single ventricle heart condition?
What are the long-term social, psychosocial and other non-clinical outcomes of children with single ventricle heart conditions?
Which models of care, virtual ward and home monitoring programmes, best support infants, children and young people with a single ventricle heart condition at home?

Management of the well-functioning Fontan circulation How can the longevity of the Fontan circulation be prolonged?
How can organ function be optimised in patients with a Fontan circulation?
What factors best determine a well-functioning Fontan circulation?
What is the optimal interval for follow-up of patients with a Fontan circulation?
What is the impact of pregnancy on patients with a Fontan circulation and how can outcomes be improved?

Surgery & perioperative care Which modifiable perioperative factors can reduce mortality for the Norwood operation?
How can perinatal risk stratification be used to identify those in whom the Norwood operation is futile?
How could technology be used to perform a biventricular repair in patients with a single ventricle heart condition?

Transplantation, mechanical support & novel therapies How can mechanical assist devices be developed to support the Fontan circulation?
What are the roles of novel therapies in the failing Fontan?
What are the alternatives to transplantation for the failing Fontan?