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Review
. 2022 Mar 29;79(12):1183-1198.
doi: 10.1016/j.jacc.2022.01.022.

Eisenmenger Syndrome: JACC State-of-the-Art Review

Alexandra Arvanitaki  1 , Michael A Gatzoulis  2 , Alexander R Opotowsky  3 , Paul Khairy  4 , Konstantinos Dimopoulos  5 , Gerhard-Paul Diller  6 , George Giannakoulas  7 , Margarita Brida  8 , Massimo Griselli  9 , Ekkehard Grünig  10 , Claudia Montanaro  5 , Peter David Alexander  5 , Rebecca Ameduri  11 , Barbara J M Mulder  12 , Michele D'Alto  13
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Free article
Review

Eisenmenger Syndrome: JACC State-of-the-Art Review

Alexandra Arvanitaki et al. J Am Coll Cardiol. .
Free article

Erratum in

  • Correction.
    [No authors listed] [No authors listed] J Am Coll Cardiol. 2023 May 2;81(17):1746. doi: 10.1016/j.jacc.2023.03.396. J Am Coll Cardiol. 2023. PMID: 37100493 No abstract available.

Abstract

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.

Keywords: Eisenmenger syndrome; congenital heart disease; multisystem involvement; pulmonary arterial hypertension; risk stratification; targeted pulmonary arterial hypertension therapy.

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Conflict of interest statement

Funding Support and Author Disclosures Dr Arvanitaki is the recipient of the International Training and Research Fellowship EMAH Stiftung Karla Voellm. Dr Gatzoulis has received unrestricted educational grants from Actelion, Pfizer, and GlaxoSmithKline. Dr Opotowsky has received funding from Actelion, Janssen, and Johnson and Johnson, not directly related to the current work; and has received funding by the Heart Institute Research Core (HIRC) supporting research in the Cincinnati Children’s Heart Institute. Dr Khairy is supported by the André Chagnon Research Chair in Electrophysiology and Congenital Heart Disease. Dr Diller has received funding from Actelion, Daiichi-Sankyo, and Bayer, unrelated to the current work; and has received funding by the Karla Völlm Foundation, supporting research in the Department. Dr Giannakoulas has receiving honoraria and consultancy fees from Actelion Pharmaceuticals Hellas, Bayer, ELPEN, Galenica-Ferrer, GlaxoSmithKline, Pfizer, Lilly, Merck Sharp & Dohme, and United Therapeutics, not directly related to this work. Dr Grünig has received fees for lectures and/or consultations from Actelion, Bayer, GlaxoSmithKline, Janssen, Merck Sharp & Dohme, Pfizer, and United Therapeutics. Dr D’Alto has received funding from Actelion-Janssen, Merck Sharp & Dohme, GlaxoSmithKline, and Dompé, not directly related to the current work. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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