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

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...

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Published in:Heart, lung & circulation lung & circulation, 2020-01, Vol.29 (1), p.5-39
Main Authors: Zentner, Dominica, Celermajer, David S., Gentles, Thomas, d’Udekem, Yves, Ayer, Julian, Blue, Gillian M., Bridgman, Cameron, Burchill, Luke, Cheung, Michael, Cordina, Rachael, Culnane, Evelyn, Davis, Andrew, du Plessis, Karin, Eagleson, Karen, Finucane, Kirsten, Frank, Belinda, Greenway, Sebastian, Grigg, Leeanne, Hardikar, Winita, Hornung, Tim, Hynson, Jenny, Iyengar, Ajay J., James, Paul, Justo, Robert, Kalman, Jonathan, Kasparian, Nadine, Le, Brian, Marshall, Kate, Mathew, Jacob, McGiffin, David, McGuire, Mark, Monagle, Paul, Moore, Ben, Neilsen, Julie, O’Connor, Bernadette, O’Donnell, Clare, Pflaumer, Andreas, Rice, Kathryn, Sholler, Gary, Skinner, Jonathan R., Sood, Siddharth, Ward, Juliet, Weintraub, Robert, Wilson, Tom, Wilson, William, Winlaw, David, Wood, Angela
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Language:English
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Summary: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. •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 cir
ISSN:1443-9506
1444-2892
DOI:10.1016/j.hlc.2019.09.010