TY - JOUR
T1 - Eisenmenger Syndrome
T2 - JACC State-of-the-Art Review
AU - Arvanitaki, Alexandra
AU - Gatzoulis, Michael A.
AU - Opotowsky, Alexander R.
AU - Khairy, Paul
AU - Dimopoulos, Konstantinos
AU - Diller, Gerhard-Paul
AU - Giannakoulas, George
AU - Brida, Margarita
AU - Griselli, Massimo
AU - Grünig, Ekkehard
AU - Montanaro, Claudia
AU - Alexander, Peter David
AU - Ameduri, Rebecca
AU - Mulder, Barbara J. M.
AU - D'Alto, Michele
N1 - Funding Information: 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. Publisher Copyright: © 2022 American College of Cardiology Foundation
PY - 2022/3/29
Y1 - 2022/3/29
N2 - 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.
AB - 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.
KW - Eisenmenger syndrome
KW - congenital heart disease
KW - multisystem involvement
KW - pulmonary arterial hypertension
KW - risk stratification
KW - targeted pulmonary arterial hypertension therapy
UR - http://www.scopus.com/inward/record.url?scp=85126277853&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.jacc.2022.01.022
DO - https://doi.org/10.1016/j.jacc.2022.01.022
M3 - Review article
C2 - 35331414
SN - 0735-1097
VL - 79
SP - 1183
EP - 1198
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 12
ER -