Management of complex congenital heart disease (CHD) – single ventricle focus

Summary

A single ventricle (SV) is a congenital heart defect (CHD) which results in the heart having one functional pumping chamber instead of two - through being smaller or missing a valve. SV babies do not survive without treatment, which entails a series of operations throughout childhood. Children who complete the full sequence of surgical treatment are referred to as Fontan patients. SV patients represent 30% of all CHD patients but are more ill, as over time they develop disease in multiple organs, are more likely to die young and cost the NHS more than other CHD patients. Because of multiple health problems and interventions, they are faced with unique physical, emotional, and intellectual challenges. There are over 3000 Fontan survivors in the UK but we do not know when and how best to treat them or SV patients in general; consequently, there is variability in interventions, including prenatal counselling, and outcomes. There is currently very limited research in the UK to inform doctors about the best way to manage patients based on their individual characteristics.

We plan to analyse a large and unique dataset on all Fontan patients in the UK. The newly created dataset, LAUNCHES, combines information about diagnosis, treatment, intensive care and hospital outcomes as well as long-term survival. We wish to determine whether the care that SV patients receive differs across the UK. We will identify the main characteristics of SV patients that influence their survival and remaining healthy. We will also investigate whether different types of surgery and the timing of surgeries influences these outcomes. At the end, we will link with existing Bristol congenital research projects and with the national SV charity to inform hypothesis-based research in a future grant.

NB - SV is the generic term for this population, they are called 'Fontans' when they complete the surgical sequence of operations around age 4 years.