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Canadian Journal of Cardiology

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

Published:April 28, 2022DOI:https://doi.org/10.1016/j.cjca.2022.04.017

      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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