The Present and Future
JACC State-of-the-Art Review
Eisenmenger Syndrome: JACC State-of-the-Art Review

https://doi.org/10.1016/j.jacc.2022.01.022Get rights and content
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Highlights

  • ES is commonly associated with secondary erythrocytosis, thrombosis, bleeding, arrhythmias, and heart failure, requiring multidisciplinary care.

  • Treatment of pulmonary arterial hypertension and timing of transplantation should be guided by prognostic tools.

  • Timely diagnosis, improved multidisciplinary care infrastructure, and access to specialists in congenital heart disease may improve outcomes for patients with ES.

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.

Key Words

congenital heart disease
Eisenmenger syndrome
multisystem involvement
pulmonary arterial hypertension
risk stratification
targeted pulmonary arterial hypertension therapy

Abbreviations and Acronyms

HIC
high-income countries
LMIC
low- and middle-income countries
RCT
randomized controlled trials
SaO2
arterial oxygen saturation

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Drs Arvanitaki and Gatzoulis are joint first authors.