End-of-life care

New guidance on Fontan circulation focuses on care for adult survivors

The “extraordinary success” of treatment strategies for people with Fontan circulation has resulted in new guidelines being released for management of the increasing numbers of patients who are living as adults.

A focus on lifelong follow up is needed for people with single ventricle types of congenital heart disease, according to the guideline authors, because improvements in coronary care have boosted 30-year survival rates for people after surgical Fontan completion to over 80%.

“Despite this remarkable improvement in survival, ensuring these patients a normal quality and duration of life is not currently a reality,” the authors write in Circulation.

Adult patients with Fontan circulation face a risk of complications including circulatory failure, ventricular dysfunction, atrioventricular valve regurgitation, arrhythmia, protein- losing enteropathy, and plastic bronchitis., they note.

Abnormalities in body composition, bone structure, and growth are common, along with liver fibrosis renal dysfunction, as well as cognitive, neuropsychological, and behavioural deficits.

“Healthcare providers are ill-prepared to tackle these challenges, as well as specific needs such as contraception and pregnancy in female patients. The role of therapies such as cardiovascular drugs to prevent and treat complications, heart transplantation, and mechanical circulatory support remains undetermined,” their position statement says.

It proposes a surveillance “testing toolkit” for routine, systematic evaluation of both cardiovascular and other organs affected by Fontan circulation.

Gaps in knowledge and areas for future investigation of Fontan circulation are also highlighted, with the objective of laying the groundwork for creating a normal quality and duration of life for patients.

“We need more research into the basic biology of single ventricle hearts and whether the damage to other organ systems, such as kidneys, liver and brain can be mitigated or reversed,” said writing group chair Professor Jack Rychik, of the Children’s Hospital of Philadelphia.

“We are now entering a new phase in the management of patients born with single-ventricle CHD. Provided that patients undergo regular follow-up, adopt a healthy lifestyle, and are encouraged to participate in investigational clinical protocols and research, healthcare providers and patients can share an optimistic view for a brighter future.”

“Patients with Fontan circulation are going to consume an ever-increasing amount of resources as they grow in number and age into adult life. Healthcare providers, both pediatric and adult, will need to increase their understanding and knowledge of this unique cardiovascular condition in order to maintain and improve their quality of life” concluded Professor Rychik.

The guidelines writing group included Professor David Celermajer of Sydney University and Associate Professor Yves d’Udekem of the Royal Children’s Hospital, Melbourne.

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