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Medical management of Eisenmenger syndrome

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

INTRODUCTION

Centers specialized in the management of adult patients with congenital heart disease provide the optimal combination of clinical expertise, facilities for advanced diagnosis and therapy, structured care, and access to new and evolving therapies. Care of adults with congenital heart disease-related pulmonary arterial hypertension should be performed in centers that have shared expertise and training in both adult congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) [1].

The triad of systemic-to-pulmonary communication, pulmonary arterial disease, and cyanosis is called Eisenmenger syndrome. The diagnosis of Eisenmenger syndrome implies that the development of pulmonary arterial disease is a consequence of increased pulmonary blood flow, and requires exclusion of other causes of PAH.

In the past, the management of patients with Eisenmenger syndrome was primarily conservative, with an emphasis on regular informed cardiovascular follow-up. Subsequent clinical research evaluated the use of therapies designed to improve patient survival and functional capacity. Pulmonary vasodilator therapy may improve hemodynamics and quality of life in some patients. Other important aspects of management include avoidance of high-risk situations outlined below, extreme caution when undertaking noncardiac surgery, and specific attention to hematologic issues. Heart and lung transplantation, or lung transplantation with repair of the cardiac defect, is appropriate in some patients with severe disease.

Management of Eisenmenger syndrome patients includes treatment pertinent to all patients with cyanotic heart disease as well as recommendations specific to CHD-PAH and Eisenmenger syndrome. Issues specific to CHD-PAH and Eisenmenger syndrome will be reviewed here.

Evaluation and prognosis of Eisenmenger syndrome and recommendations for patients with cyanotic heart disease (including those with Eisenmenger syndrome) are discussed separately. (See "Evaluation and prognosis of Eisenmenger syndrome" and "Medical management of cyanotic congenital heart disease in adults".)

                     

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Literature review current through: Nov 2016. | This topic last updated: Tue Dec 01 00:00:00 GMT 2015.
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