Background The Fontan operation is a palliative procedure which increases the survival of patients born with an anatomical or functional univentricular circulation. The majority of individuals who have undergone Fontan palliation are now living into adulthood. More than 80% will survive 20 years beyond surgery. However, failure of the Fontan circulation is inevitable, and over time complications develop involving multiple organ systems.
Aims To identify the unique needs of this patient group and suggest potential models of collaborative working that could improve access to and delivery of supportive or end of life care.
Methods We present a review of the available literature and two in-depth case studies which emphasise the complexity of end of life care delivery and potential opportunities for improved management.
Results The literature suggests that adults with a Fontan circulation experience a significant burden of physical symptoms and a range of psychosocial issues, which negatively impact their quality of life. Many would welcome open discussions about end of life issues and express a wish for this to be initiated early. Despite this, healthcare resource use towards the end of life is high, with hospitalisation increasing dramatically in the last month. Input from specialist palliative care services is rare. A major challenge is balancing active treatment alongside discussions about end of life. Both cases we describe benefitted from ongoing holistic assessment by the cardiology team, early introduction to specialist palliative care services, and timely admission to the hospice, at a point where ongoing active management could be integrated into a symptom focused approach.
Conclusion The population living with a Fontan circulation is increasing rapidly. Over time, many of these patients will have significant supportive care needs which are currently not being met. The cases presented suggest models of collaborative care delivery that may help to address this gap.