Abstract
As a result of remarkable progress in operative techniques and cardiology care during
childhood, Fontan patients continue to age and require team-based multidisciplinary
expertise to manage complications encountered in adulthood. They face particular challenges
in terms of altered hemodynamic stressors, cardiac and hepatic failure, and arrhythmias.
Arrhythmias in Fontan patients are highly prevalent and associated with underlying
anatomy, surgical technique, and postoperative sequelae. Diagnostic tools, treatments,
and device strategies for arrhythmias in Fontan patients should be adapted to the
specific anatomy, type of surgical repair, and clinical status. Great strides in our
understanding of arrhythmia mechanisms, options and techniques to obtain access to
relevant cardiac structures, and application of both old and new technologies have
contributed to improving cardiac implantable electronic device (CIED) therapies for
this unique population. In this state-of-the-art review, we discuss the various arrhythmias
encountered in Fontan patients, their diagnosis, and options for treatment and prevention,
with a focus on CIEDs. Throughout, access challenges particular to the Fontan circulation
are considered. Recently developed technologies, such as the subcutaneous implantable
cardioverter defibrillator, carry the potential to be transformative but require awareness
of Fontan-specific issues. Moreover, new leadless pacing technology represents a promising
strategy that may soon become applicable to Fontan patients with sinus-node dysfunction.
CIEDs are essential tools in managing Fontan patients, but the complex clinical scenarios
that arise in this patient population are among the most challenging for the electrophysiologist
treating patients with congenital heart disease.
Résumé
En raison des progrès remarquables accomplis en matière de techniques opératoires
et de soins cardiologiques pédiatriques, les patients qui ont subi l’intervention
de Fontan continuent d’avancer en âge et ont besoin d’une expertise multidisciplinaire
pour gérer les complications qu’ils rencontrent à l’âge adulte. Ces patients doivent
composer avec des difficultés particulières ayant à voir avec la modification des
facteurs de stress hémodynamique, l’insuffisance cardiaque et hépatique, et les arythmies.
Ces dernières sont très fréquentes chez les patients ayant subi l’intervention de
Fontan et se trouvent associées à l’anatomie sous-jacente, à la technique chirurgicale
et aux séquelles postopératoires. Les outils diagnostiques, les traitements et les
stratégies technologiques visant à remédier aux arythmies chez les patients ayant
subi l’intervention de Fontan devraient être adaptés aux ca-ractéristiques anatomiques
individuelles, au type de réparation chi-rurgicale et au tableau clinique. Les grandes
avancées sur le plan de notre compréhension des mécanismes de l’arythmie, les options
et les techniques dont nous disposons pour accéder aux structures cardiaques d’intérêt,
de même que la mise en œuvre de technologies an-ciennes et nouvelles ont contribué
à améliorer les traitements faisant appel à des dispositifs cardiaques électroniques
implantables (DCEI) dans cette population unique. Le présent article dresse un bilan
exhaustif de la question. Nous y abordons les diverses formes d’a-rythmies qui touchent
les patients ayant subi l’intervention de Fontan, leur diagnostic et les options de
traitement et de prévention, en accordant une attention toute particulière aux DCEI.
Les problèmes d’accès que pose la circulation de Fontan sont pris en compte tout au
long de notre article. Les technologies récemment mises au point, comme le défibrillateur
cardioverteur implantable sous-cutané, pourraient être porteuses de changements profonds,
mais elles nécessitent une prise de conscience des problèmes qui se rattachent à l’intervention
de Fontan. En outre, la nouvelle technologie de stimulation sans sonde se révèle une
stratégie prometteuse qui pourrait bientôt devenir applicable dans les cas de dysfonctionnement
du nœud sinusal chez les patients ayant subi l’intervention de Fontan. Les DCEI sont
des outils essentiels pour la prise en charge des patients ayant subi l’intervention
de Fontan. Cependant, les scénarios cliniques complexes observés au sein de cette
population de patients font partie de ceux qui posent le plus de difficultés aux électrophysiologistes
qui traitent les patients atteints de cardiopathies congénitales.
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Article Info
Publication History
Published online: May 16, 2022
Accepted:
April 19,
2022
Received:
January 9,
2022
Footnotes
See page 1056 for disclosure information.
Identification
Copyright
© 2022 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.