The Eisenmenger syndrome in adults
W Vongpatanasin1, M E Brickner, L D Hillis
1University of Texas Southwestern Medical Center, Dallas, USA.
Annals of Internal Medicine
|December 16, 1998
Summary
Eisenmenger syndrome, a severe congenital heart disease complication, involves elevated pulmonary vascular resistance and right-to-left shunting. Adults with Eisenmenger syndrome face risks including thromboembolic events and require specialized multidisciplinary care.
Area of Science:
- Cardiology
- Pulmonology
- Internal Medicine
Background:
- Eisenmenger syndrome is a severe complication of unrepaired congenital heart disease.
- It is characterized by elevated pulmonary vascular resistance and bidirectional or right-to-left shunting of blood.
Purpose of the Study:
- To review the pathophysiology, clinical features, natural history, prognosis, and management of Eisenmenger syndrome in adults.
- To provide guidance for practicing physicians on managing this complex condition.
Main Methods:
- Literature review of English-language articles from 1966 to the present.
- MEDLINE database search using terms: Eisenmenger, congenital heart disease, pulmonary hypertension.
- Manual extraction of descriptive and analytical data from selected articles.
Main Results:
- Most patients survive 20-30 years, but face complications like thromboembolic events, hyperviscosity syndrome, erythrocytosis, hemoptysis, gout, and renal dysfunction.
- Pregnancy and noncardiac surgery are associated with high mortality.
- Pediatric survivors require lifelong specialized care.
Conclusions:
- Primary care physicians need a thorough understanding of Eisenmenger syndrome, its complications, and management, including phlebotomy and transplantation.
- Routine follow-up at a tertiary care center is crucial.
- A multidisciplinary approach is vital for managing high-risk situations like pregnancy or noncardiac surgery.
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