Evaluation and prognosis of Eisenmenger syndrome
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
The triad of systemic-to-pulmonary congenital cardiovascular communication, pulmonary arterial disease, and cyanosis is called Eisenmenger syndrome. The diagnosis of Eisenmenger syndrome implies that pulmonary arterial disease has developed as a consequence of increased pulmonary blood flow, and requires exclusion of other causes of pulmonary hypertension.
In 1897, Eisenmenger described a syndrome in which pulmonary vascular disease developed in patients with a nonrestrictive ventricular septal defect . It was subsequently shown that pulmonary vascular disease could also occur with other congenital cardiac defects in which a systemic-to-pulmonary communication is present such as atrial shunts, other ventricular shunts, and aortic shunts (table 1).
Such conditions are often associated with an initial left-to-right shunt. Increased pulmonary blood flow as a result of the shunt leads to the development of pulmonary vascular disease with increased pulmonary vascular resistance (PVR). The shunt may then reverse and become right-to-left (figure 1A-B and algorithm 1) [2,3]. At this stage, the patient typically becomes cyanotic.
With the advent of surgical correction of congenital heart disease (CHD), the prevalence of Eisenmenger syndrome has declined but is not negligible. This topic will review the general features, evaluation, and prognosis of CHD-related pulmonary arterial hypertension and Eisenmenger syndrome. The pathology and pathophysiology of pulmonary hypertension in Eisenmenger syndrome are discussed in detail separately. (See "Pathogenesis of pulmonary hypertension".)
The medical management of patients with Eisenmenger syndrome is discussed separately. (See "Management of Eisenmenger syndrome".)
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- GENERAL FEATURES
- Anatomy and physiology
- - Shunt size and defect type
- Physical examination
- EVALUATION FOR CONGENITAL HEART DISEASE RELATED PULMONARY ARTERIAL HYPERTENSION
- Initial evaluation
- Additional testing
- Additional evaluation for suspected Eisenmenger syndrome
- SUMMARY AND RECOMMENDATIONS