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Evaluation and prognosis of Eisenmenger syndrome

Author
Heidi M Connolly, MD, FASE
Section Editor
John K Triedman, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC

INTRODUCTION

In 1897, Eisenmenger described a syndrome in which pulmonary vascular disease developed in patients with a nonrestrictive ventricular septal defect (figure 1A-B) [1]. 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.

The triad of systemic-to-pulmonary 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.

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 "Medical management of Eisenmenger syndrome".)

           

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Literature review current through: Nov 2016. | This topic last updated: Mon Jun 08 00:00:00 GMT+00:00 2015.
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References
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