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Perioperative and long-term management of Fontan patients

Published online by Cambridge University Press:  12 January 2021

May Al-Shawk Open the ORCID record for May Al-Shawk [Opens in a new window] ,
Adeolu Banjoko ,
Ariana Axiaq ,
Kiran Amin  and
Amer Harky Open the ORCID record for Amer Harky [Opens in a new window]
May Al-Shawk
Affiliation:
Institute of Medical and Biomedical Education, St George’s University of London, London, UK
Adeolu Banjoko
Affiliation:
College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
Ariana Axiaq
Affiliation:
School of Medicine, Queen’s University Belfast, Belfast, UK
Kiran Amin
Affiliation:
Institute of Medical and Biomedical Education, St George’s University of London, London, UK
Amer Harky*
Affiliation:
Department of Cardiothoracic Surgery, Liverpool Heart and Chest Hospital, Liverpool, UK Department of Cardiac Surgery, Alder Hey Children Hospital, Liverpool, UK Liverpool Centre for Cardiovascular Science, University of Liverpool and Liverpool, Heart and Chest Hospital, UK
*
Author for correspondence: Dr A. Harky, MSc, MRCS, Department of Cardiac Surgery, Alder Hey Children Hospital, E Prescot Rd, L14 5AB, Liverpool, UK. Tel: +44-0151-228-4811. E-mail: aaharky@gmail.com
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Abstract

A Fontan circulation requires a series of three-staged operations aimed to palliate patients with single-ventricle CHD. Currently, the most frequent technique is the extracardiac total cavopulmonary connection, an external conduit connecting the IVC and right pulmonary artery, bypassing the right side of the heart. Fontan candidates must meet strict criteria; they are assessed utilising both cardiac catheterisation and cardiac magnetic resonance. Postoperatively, treatment protocols prioritise antibiotic prophylaxis, diuretics, angiotensin-converting enzyme inhibitors, anticoagulation, and oxygen therapy with fluid restriction and a low-fat diet. These measures aim to reduce length of stay in the ICU and hospital by preventing acute complications such as infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury. Late complications of a Fontan procedure include circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease. The definitive management is cardiac transplantation, with promising innovations in selective embolisation of lymphatic vessels and Fontan-specific ventricular assist devices. Further research assessing current protocols in the perioperative management of Fontan patients would be beneficial for standardising current practice and improving outcomes.

Keywords

FontanperioperativecomplicationsmanagementICU
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 5 , May 2021 , pp. 775 - 785
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

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Footnotes

*

May Al-Shawk and Adeolu Banjoko are both first authors on this review with a shared first co-authorship

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