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Canadian Journal of Cardiology

Heart Failure in Adult Congenital Heart Disease: From Advanced Therapies to End-of-Life Care

      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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