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Canadian Journal of Cardiology

Eisenmenger Syndrome: A Multisystem Disorder—Do Not Destabilize the Balanced but Fragile Physiology

Published:October 10, 2019DOI:https://doi.org/10.1016/j.cjca.2019.10.002

      Abstract

      Eisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to-right shunt triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, which ultimately results in shunt reversal. Herein, we review the changing epidemiological patterns and pathophysiology of Eisenmenger syndrome. Multiorgan disease is an integral manifestation of Eisenmenger syndrome and includes involvement of the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems. Standardized practical guidelines for the assessment, management, risk stratification, and follow-up of this very fragile and vulnerable population are discussed. Multidisciplinary care is the best clinical practice. An approach to the prevention and management of a broad spectrum of complications is provided. Relevant therapeutic questions are discussed, including anticoagulation, noncardiac surgery, physical activity, transplantation, and advanced-care planning (palliative care). Advanced pulmonary arterial hypertension therapies are indicated in patients with Eisenmenger syndrome and World Health Organization functional class II or higher symptoms to improve functional capacity, quality of life, and—less well documented—survival. Specific recommendations regarding monotherapy or combination therapy are provided according to functional class and clinical response. The ultimate challenge for all care providers remains early detection and management of intracardiac and extracardiac shunts, considering that Eisenmenger syndrome is a preventable condition.

      Résumé

      Le syndrome d'Eisenmenger est le phénotype le plus sévère et extrême de l'hypertension artérielle pulmonaire associée aux cardiopathies congénitales. Un important shunt gauche-droit systémique non restrictif engendre une maladie vasculaire pulmonaire, l'apparition d'une hypertension artérielle pulmonaire et la majoration des résistances vasculaires pulmonaires; les pressions pulmonaires atteignent un seuil systémique à l'origine du renversement du shunt. Cet article discute des bases physiopathologiques et du changement épidémiologique du syndrome d'Eisenmenger. L'atteinte multiviscérale est caractéristique du syndrome d'Eisenmenger impliquant le système cardio-vasculaire, hématologique, neurologique, respiratoire, gastro-intestinal, urinaire, immunologique, musculo-squelettique et endocrinien. Nous proposons des lignes directrices pratiques pour l’évaluation, la prise en charge, la stratification des risques et le suivi de cette population fragile et vulnérable. Les pratiques cliniques exemplaires recommandent des soins multidisciplinaires. Nous présentons aussi une approche pour la prévention et la prise en charge d'un grand éventail de complications et nous abordons des questions thérapeutiques pertinentes, notamment l'anticoagulothérapie, la chirurgie non cardiaque, l'activité physique, la greffe et la planification préalable des soins (soins palliatifs). Les traitements spécifiques de l'hypertension artérielle pulmonaire sont indiqués chez les patients atteints de syndrome d'Eisenmenger avec une classe fonctionnelle de II ou plus de l'Organisation mondiale de la Santé afin d'améliorer la capacité fonctionnelle, la qualité de vie, et bien que moins documentée la survie. Enfin, nous formulons des recommandations spécifiques concernant le recours à la monothérapie ou à l'association thérapeutique selon la classe fonctionnelle et la réponse clinique. Le diagnostic précoce et la prise en charge optimale des shunts intracardiaques et extracardiaques sont les défis ultimes des équipes traitantes médicales pour prévenir le syndrome d'Eisenmenger.
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