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Long-term management of Fontan patients: The importance of a multidisciplinary approach

The Fontan operation is a palliative procedure that leads to increased survival of patients with a functional single ventricle (SV). Starting from 1967 when the first operation was performed by Francis Fontan, more and more patients have reached adulthood. Furthermore, it is expected that in the nex...

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Autores principales: Martino, Diletta, Rizzardi, Caterina, Vigezzi, Serena, Guariento, Chiara, Sturniolo, Giulia, Tesser, Francesca, Salvo, Giovanni di
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9452819/
https://www.ncbi.nlm.nih.gov/pubmed/36090574
http://dx.doi.org/10.3389/fped.2022.886208
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author Martino, Diletta
Rizzardi, Caterina
Vigezzi, Serena
Guariento, Chiara
Sturniolo, Giulia
Tesser, Francesca
Salvo, Giovanni di
author_facet Martino, Diletta
Rizzardi, Caterina
Vigezzi, Serena
Guariento, Chiara
Sturniolo, Giulia
Tesser, Francesca
Salvo, Giovanni di
author_sort Martino, Diletta
collection PubMed
description The Fontan operation is a palliative procedure that leads to increased survival of patients with a functional single ventricle (SV). Starting from 1967 when the first operation was performed by Francis Fontan, more and more patients have reached adulthood. Furthermore, it is expected that in the next 20 years, the population with Fontan circulation will reach 150,000 subjects. The absence of right ventricular propulsion and the inability to improve cardiac output because of the low cardiac reserve are the main issues with the Fontan circulation; however, potential complications may also involve multiple organ systems, such as the liver, lungs, brain, bones, and the lymphatic system. As these patients were initially managed mainly by pediatric cardiologists, it was important to assure the appropriate transition to adult care with the involvement of a multidisciplinary team, including adult congenital cardiologists and multiple subspecialists, many of whom are neither yet familiar with the pathophysiology nor the end-organ consequences of the Fontan circulation. Therefore, the aim of our work was to collect all the best available evidence on Fontan's complications management to provide “simple and immediate” information sources for practitioners looking for state of the art evidence to guide their decision-making and work practices. Moreover, we suggest a model of follow-up of patients with Fontan based on a patient-centered multidisciplinary approach.
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spelling pubmed-94528192022-09-09 Long-term management of Fontan patients: The importance of a multidisciplinary approach Martino, Diletta Rizzardi, Caterina Vigezzi, Serena Guariento, Chiara Sturniolo, Giulia Tesser, Francesca Salvo, Giovanni di Front Pediatr Pediatrics The Fontan operation is a palliative procedure that leads to increased survival of patients with a functional single ventricle (SV). Starting from 1967 when the first operation was performed by Francis Fontan, more and more patients have reached adulthood. Furthermore, it is expected that in the next 20 years, the population with Fontan circulation will reach 150,000 subjects. The absence of right ventricular propulsion and the inability to improve cardiac output because of the low cardiac reserve are the main issues with the Fontan circulation; however, potential complications may also involve multiple organ systems, such as the liver, lungs, brain, bones, and the lymphatic system. As these patients were initially managed mainly by pediatric cardiologists, it was important to assure the appropriate transition to adult care with the involvement of a multidisciplinary team, including adult congenital cardiologists and multiple subspecialists, many of whom are neither yet familiar with the pathophysiology nor the end-organ consequences of the Fontan circulation. Therefore, the aim of our work was to collect all the best available evidence on Fontan's complications management to provide “simple and immediate” information sources for practitioners looking for state of the art evidence to guide their decision-making and work practices. Moreover, we suggest a model of follow-up of patients with Fontan based on a patient-centered multidisciplinary approach. Frontiers Media S.A. 2022-08-25 /pmc/articles/PMC9452819/ /pubmed/36090574 http://dx.doi.org/10.3389/fped.2022.886208 Text en Copyright © 2022 Martino, Rizzardi, Vigezzi, Guariento, Sturniolo, Tesser and Salvo. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Martino, Diletta
Rizzardi, Caterina
Vigezzi, Serena
Guariento, Chiara
Sturniolo, Giulia
Tesser, Francesca
Salvo, Giovanni di
Long-term management of Fontan patients: The importance of a multidisciplinary approach
title Long-term management of Fontan patients: The importance of a multidisciplinary approach
title_full Long-term management of Fontan patients: The importance of a multidisciplinary approach
title_fullStr Long-term management of Fontan patients: The importance of a multidisciplinary approach
title_full_unstemmed Long-term management of Fontan patients: The importance of a multidisciplinary approach
title_short Long-term management of Fontan patients: The importance of a multidisciplinary approach
title_sort long-term management of fontan patients: the importance of a multidisciplinary approach
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9452819/
https://www.ncbi.nlm.nih.gov/pubmed/36090574
http://dx.doi.org/10.3389/fped.2022.886208
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