Abstract
The Fontan procedure is a staged palliation for various complex congenital cardiac
lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome,
and double-inlet left ventricle, all of which involve a functional single-ventricle
physiology. The complexity of the patients’ original anatomy combined with the anatomic
and physiologic consequences of the Fontan circulation creates challenges. Teens and
adults living with Fontan palliation will need perioperative support for noncardiac
surgery, peripartum management for labour and delivery, interventions related to their
structural heart disease, electrophysiology procedures, pacemakers, cardioversions,
cardiac surgery, transplantation, and advanced mechanical support. This review focuses
on the anesthetic and intensive care unit (ICU) management of these patients during
their perioperative journey, with an emphasis on the continuity of preintervention
planning, referral pathways, and postintervention ICU management. Requests for recipes
and doses of medications are frequent; however, as in normal anesthesia and ICU practice,
the method of anesthesia and dosing are dependent on the presenting medical/surgical
conditions and the underlying anatomy and physiologic reserve. A patient with Fontan
palliation in their early 20s attending school full-time with a cavopulmonary connection
is likely to have more reserve than a patient in their late 40s with an atriopulmonary
Fontan at home waiting for a heart transplant. Each case will require an anesthetic
and critical care plan tailored to the situation. The critical care environment is
a natural extension of the anesthetic management of a patient, with complex considerations
for a patient with Fontan palliation.
Résumé
L’intervention de Fontan est un traitement palliatif par étapes pour diverses lésions
cardiaques congénitales complexes, dont l’atrésie tricuspide, l’atrésie pulmonaire,
le syndrome d’hypoplasie du cœur gauche et le ventricule gauche à double entrée, qui
impliquent toutes une physiologie univentriculaire fonctionnelle. La complexité de
l’anatomie originale des patients conjuguée aux conséquences ana-tomiques et physiologiques
de la circulation de Fontan pose des difficultés. Dans ce contexte, la prise en charge
des adolescents et des adultes ayant subi l’intervention de Fontan comprend plusieurs
fa-cettes : soutien périopératoire en chirurgie non cardiaque, gestion périnatale
du travail et de l’accouchement, interventions liées à la cardiopathie structurelle,
interventions d’électrophysiologie, mise en place de stimulateurs cardiaques, cardioversions,
chirurgies cardiaques, transplantations, assistance mécanique avancée. Le présent
article de synthèse porte sur la prise en charge périopératoire de ces patients dans
les unités d’anesthésie et de soins intensifs. L’accent est mis sur la continuité
de la planification préinterventionnelle, les voies d’orientation des patients au
sein des établissements de santé et la prise en charge postinterventionnelle à l’unité
de soins intensifs. Les demandes de recommandations posologiques sont fréquentes;
cependant, comme dans la pratique normale en anesthésie et en soins intensifs, la
méthode d’anesthésie et la détermination des doses dépendent du contexte médical ou
chirurgical dans lequel s’inscrit le tableau clinique ainsi que de l’anatomie sous-jacente
et de la réserve physiologique. Un patient ayant qui a subi une intervention de Fontan
au début de la vingtaine et qui fréquente un établissement d’enseignement à temps
plein en étant porteur d’une dérivation cavo-pulmonaire est susceptible d’avoir une
plus grande réserve qu’un patient dans la quarantaine avancée qui a subi une intervention
de Fontan de type anastomose atriopulmonaire et qui demeure à la maison dans l’attente
d’une transplantation cardiaque. Chaque cas nécessite un plan d’anesthésie et de soins
intensifs adapté à la situation. Les soins intensifs constituent un prolongement naturel
de la prise en charge en anesthésie. Ils sont assortis de considérations complexes
dans le cas d'un patient ayant subi l’intervention de Fontan.
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Article Info
Publication History
Published online: April 28, 2022
Accepted:
April 21,
2022
Received:
November 1,
2021
Footnotes
See page 1109 for disclosure information.
Identification
Copyright
Crown Copyright © 2022 Published by Elsevier Inc. on behalf of the Canadian Cardiovascular Society. All rights reserved.
