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Eisenmenger syndrome: JACC state-of-the-art review

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary...

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Main Authors: Arvanitaki, Alexandra (Author) , Gatzoulis, Michael A. (Author) , Opotowsky, Alexander R. (Author) , Khairy, Paul (Author) , Dimopoulos, Konstantinos (Author) , Diller, Gerhard-Paul (Author) , Giannakoulas, George (Author) , Brida, Margarita (Author) , Griselli, Massimo (Author) , Grünig, Ekkehard (Author) , Montanaro, Claudia (Author) , Alexander, Peter David (Author) , Ameduri, Rebecca (Author) , Mulder, Barbara J. M. (Author) , D’Alto, Michele (Author)
Format: Article (Journal)
Language:English
Published: 21 March 2022
In: Journal of the American College of Cardiology
Year: 2022, Volume: 79, Issue: 12, Pages: 1183-1198
ISSN:1558-3597
DOI:10.1016/j.jacc.2022.01.022
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.jacc.2022.01.022
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S0735109722001887
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Author Notes:Alexandra Arvanitaki, Michael A. Gatzoulis, Alexander R. Opotowsky, Paul Khairy, Konstantinos Dimopoulos, Gerhard-Paul Diller, George Giannakoulas, Margarita Brida, Massimo Griselli, Ekkehard Grünig, Claudia Montanaro, Peter David Alexander, Rebecca Ameduri, Barbara J.M. Mulder, Michele D’Alto
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Summary:Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond.
Item Description:Gesehen am 08.08.2022
Physical Description:Online Resource
ISSN:1558-3597
DOI:10.1016/j.jacc.2022.01.022

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