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Eisenmenger Syndrome: JACC State-of-the-Art Review
Alexandra Arvanitaki1, Michael A Gatzoulis2, Alexander R Opotowsky3
1Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy's and St Thomas's NHS Foundation Trust, Imperial College, London, United Kingdom; First Department of Cardiology, AHEPA University Hospital, School of Medicine, Faculty of Health Sciences, Aristotle University of Thessaloniki, Thessaloniki, Greece; Department of Cardiology III - Adult Congenital and Valvular Heart Disease, University Hospital Muenster, Albert-Schweitzer-Campus 1, Muenster, Germany.
Insights
Eisenmenger syndrome (ES) remains complex despite advances in pediatric cardiology. This review covers ES pathophysiology, prognostication, and outcomes with new therapies, highlighting care gaps and future directions.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Medicine
- Pulmonary Hypertension
Background:
- Congenital heart disease (CHD) care has advanced, yet Eisenmenger syndrome (ES) persists.
- ES presents complex multisystem challenges: erythrocytosis, hemostatic issues, arrhythmias, heart failure, and early mortality.
- Multidisciplinary expert centers are crucial for optimal ES management.
Purpose of the Study:
- To review recent progress in understanding ES pathophysiology.
- To discuss advancements in ES prognostication and clinical outcomes with targeted therapies.
- To identify care uncertainties, evidence gaps, and propose initiatives for global ES burden reduction.
Main Methods:
- Literature review of recent advancements in Eisenmenger syndrome.
- Analysis of pathophysiology, prognostication, and therapeutic outcomes.
- Exploration of global health disparities and emerging technology applications in ES care.
Main Results:
- Significant progress in understanding ES pathophysiology and improved outcomes with pulmonary arterial hypertension therapies.
- Identification of critical areas of uncertainty and gaps in current evidence-based care.
- Proposal for initiatives to reduce ES disease burden and leverage technology in healthcare delivery.
Conclusions:
- Eisenmenger syndrome requires specialized, multidisciplinary care despite overall improvements in CHD management.
- Pulmonary arterial hypertension-targeted therapies have improved clinical outcomes.
- Addressing evidence gaps and utilizing emerging technologies are key to enhancing ES care globally.
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.
