Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement

Dominica Zentner, David S. Celermajer, Thomas Gentles, Yves d'Udekem, Julian Ayer, Gillian M. Blue, Cameron Bridgman, Luke Burchill, Michael Cheung, Rachael Cordina, Evelyn Culnane, Andrew Davis, Karin du Plessis, Karen Eagleson, Kirsten Finucane, Belinda Frank, Sebastian Greenway, Leeanne Grigg, Winita Hardikar, Tim HornungJenny Hynson, Ajay J. Iyengar, Paul James, Robert Justo, Jonathan Kalman, Nadine Kasparian, Brian Le, Kate Marshall, Jacob Mathew, David McGiffin, Mark McGuire, Paul Monagle, Ben Moore, Julie Neilsen, Bernadette O'Connor, Clare O'Donnell, Andreas Pflaumer, Kathryn Rice, Gary Sholler, Jonathan R. Skinner, Siddharth Sood, Juliet Ward, Robert Weintraub, Tom Wilson, William Wilson, David Winlaw, Angela Wood

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The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. Keypoints: • Lifelong quality medical care with access to multidisciplinary services, is of prime importance. Care includes regular tests for surveillance of health status. • Transition from paediatric to adult care is an active process that should commence during early adolescence and continue until successful engagement with adult congenital cardiology care. • Children and adults with a Fontan circulation often have reduced peak exercise capacity (on average, 60–65% of predicted values). Increasingly, evidence suggests exercise training may improve exercise capacity and cardiovascular function. • People with a Fontan circulation have higher rates of anxiety and behavioural disorders, and there needs to be a low threshold for the provision of mental health care. • Pregnancy has increased maternal and fetal risks, and pre-conception multidisciplinary assessment and counselling is essential. • Atrial arrhythmias are common, often late after Fontan surgical repair and due to intra-atrial re-entry or “flutter” mediated by atrial stretch and scarring. Some anti-arrhythmic agents, most classically the type IC drugs, may allow haemodynamically unstable, life-threatening 1:1 AV conduction. • Anticoagulation with warfarin is routine care in patients with atrial arrhythmias. • In patients with recurrent atrial arrhythmias, catheter ablation or surgical conversion may be considered. • The Fontan circulation is an ideal substrate for thrombus formation and may result in intracardiac or intravascular thrombosis, ischaemic stroke, or other embolic phenomena. Antiplatelet and anticoagulant agents are commonly prescribed for thromboprophylaxis in patients with a Fontan circulation. Evidence suggests that treatment with one of these agents is advantageous, but there is no consensus on which is optimal. Despite treatment, symptomatic thromboembolic events are associated with significant mortality. • Heart failure is the leading cause of morbidity and mortality. Diuretics provide symptomatic relief, however standard heart failure medical therapy is not of proven benefit. • Though not well understood, there is increasing concern regarding progressive liver disease with a long-term risk of hepatocellular carcinoma. • Despite early higher mortality post heart transplant, these individuals have better long-term survival outcomes compared with many other heart transplant recipients.

Original languageEnglish
Pages (from-to)5-39
Number of pages35
JournalHeart Lung and Circulation
Issue number1
Publication statusPublished - Jan 2020

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