Abstract
Eisenmenger syndrome is a multisystem disorder and the most severe form of pulmonary
arterial hypertension in adult congenital heart disease. Pulmonary arterial hypertension
represents a fatal disease, characterized by increased pulmonary vascular resistance,
right heart failure, and death. Although therapeutic management has rapidly advanced
in recent years, these patients were not included in randomized controlled trials
for specific pulmonary arterial hypertension drugs, except for bosentan. However,
in clinical practice we apply treatment strategies combining drugs targeting multiple
pathobiological pathways. We present 3 patients with Eisenmenger syndrome and their
improvement after starting treatment with selexipag, an oral selective IP prostacyclin
receptor agonist.
Résumé
Le syndrome d'Eisenmenger est un trouble multisystémique et la forme la plus grave
d'hypertension artérielle pulmonaire parmi les cardiopathies congénitales chez l'adulte.
L'hypertension artérielle pulmonaire est une maladie mortelle, caractérisée par une
augmentation de la résistance vasculaire pulmonaire, une insuffisance cardiaque droite
et le décès. Malgré des progrès rapides dans la prise en charge thérapeutique ces
dernières années, ces patients n'ont pas été inclus dans des essais contrôlés randomisés
portant sur des médicaments spécifiques de l'hypertension artérielle pulmonaire, à
l'exception du bosentan. Cependant, dans la pratique clinique, nous appliquons des
stratégies de traitement combinant des médicaments ciblant plusieurs voies pathobiologiques.
Nous présentons trois patients atteints du syndrome d'Eisenmenger et l’amélioration
de leur état de santé après avoir commencé un traitement par selexipag, un agoniste
sélectif oral de l'IP, récepteur de la prostacycline.
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References
- Eisenmenger syndrome: a multisystem disorder-do not destabilize the balanced but fragile physiology.Can J Cardiol. 2019; 35: 1698-1707
- 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).Eur Heart J. 2016; 37: 67-119
- Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators.Circulation. 2006; 114: 48-54
- Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study.Eur J Heart Fail. 2019; 21: 352-359
- Selexipag in congenital heart disease-associated pulmonary arterial hypertension and Eisenmenger syndrome: first report.Am J Ther. 2018; 25: e714-e715
Article info
Publication history
Published online: February 01, 2021
Accepted:
January 22,
2021
Received:
May 2,
2020
Footnotes
See page 1288 for disclosure information.
Identification
Copyright
© 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
