Abstract
There is mounting recognition that some of the most urgent problems of adult congenital
heart disease (ACHD) are the prevention, diagnosis, and management of heart failure
(HF). Recent expert consensus and position statements not only emphasize a specific
and pressing need to tackle HF in ACHD (ACHD-HF) but also highlight the difficulty
of doing so given a current sparsity of data. Some of the challenges will be addressed
by this review. The authors are from 3 different centres; each centre has an established
subspeciality ACHD-HF clinic and is able to provide heart transplant, multiorgan transplant,
and mechanical support for patients with ACHD. Appropriate care of this complex population
requires multidisciplinary ACHD-HF teams evaluate all possible treatment options.
The risks and benefits of nontransplant ACHD surgery, percutaneous structural and
electrophysiological intervention, and ongoing conservative management must be considered
alongside those of transplant strategies. In our approach, advanced care planning
and palliative care coexist with the consideration of advanced therapies. An ethos
of shared decision making, guided by the patient’s values and preferences, strengthens
clinical care, but requires investment of time as well as skilled communication. In
this review, we aim to offer practical real-world advice for managing these patients,
supported by scientific data where it exists.
Résumé
Il est de plus en plus admis que la prévention, le diagnostic et la prise en charge
de l’insuffisance cardiaque (IC) font partie des problèmes les plus urgents dans le
traitement de la cardiopathie congénitale chez l’adulte (CPCA). Les consensus d’experts
et les énoncés de position formulés récemment font ressortir la nécessité particulière
et urgente de s’attaquer à l’IC dans la CPCA (IC-CPCA), mais aussi la difficulté que
pose cette tâche étant donné le peu de données dont on dispose. L’article expose certains
des défis à relever. Les auteurs sont rattachés à trois centres différents, chacun
doté d’une clinique spécialisée dans l’IC-CPCA et offrant des services de greffe cardiaque,
de greffe multiorgane et de soutien artificiel aux patients atteints de CPCA. Pour
que les patients de cette population complexe puissent recevoir des soins adéquats,
des équipes multidisciplinaires spécialisées dans l’IC-CPCA doivent évaluer toutes
les options thérapeutiques possibles. Les risques et les avantages associés à une
intervention chirurgicale autre qu’une greffe, à une intervention percutanée visant
à effectuer un remodelage structurel et électrophysiologique et à une prise en charge
prudente soutenue doivent être envisagés en parallèle avec ceux d’une greffe. En vertu
de notre approche, la planification préalable des soins avancés et les soins palliatifs
sont tout aussi importants que la prise en considération des traitements avancés.
Une culture axée sur la prise de décision partagée, qui tient compte des valeurs et
des préférences du patient, rehausse les soins cliniques, mais exige plus de temps
ainsi qu’une communication efficace. Nous tentons ici de formuler des conseils pratiques
et réalistes pour la prise en charge de ces patients, en nous fondant dans la mesure
du possible sur des données scientifiques.
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Article info
Publication history
Published online: July 26, 2019
Accepted:
July 23,
2019
Received:
May 17,
2019
Footnotes
See page 1736 for disclosure information.
Identification
Copyright
© 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
