Elsevier

Progress in Cardiovascular Diseases

Volume 45, Issue 2, September–October 2002, Pages 129-138
Progress in Cardiovascular Diseases

Eisenmenger's syndrome: Current management☆

https://doi.org/10.1053/pcad.2002.127492Get rights and content

Abstract

Eisenmenger's syndrome describes the elevation of pulmonary arterial pressure to the systemic level caused by increased pulmonary vascular resistance with reversal or bi-directional shunting through a large intracardiac or extracardiac congenital heart defect. This article reviews the natural history and pathophysiology of Eisenmenger's syndrome untreated and medical and surgical treatment options presently available. Although there is no cure for this condition at present, recent advances in management have improved the quality of life for many patients with Eisenmenger's syndrome. Copyright 2002, Elsevier Science (USA). All rights reserved.

Progress in Cardiovascular Diseases, Vol. 45, No. 2, (September/October) 2002: pp 129-138

Section snippets

Natural history

The natural history of Eisenmenger's syndrome demonstrates a wide spectrum of variability, although overall, survival is significantly better than for patients with primary pulmonary hypertension (Fig 1).5

. Survival of 94 adults with ventricular septal defects and Eisenmenger's syndrome or primary pulmonary hypertension.(Reprinted with permission.5)

Most patients survive into the third to fourth decade of life; and although rare, there are case reports of patients living into the seventh decade.

Pathophysiology

Pulmonary vascular disease is characterized by elevated pulmonary vascular resistance, which leads to pulmonary arterial hypertension. In patients with large left to right shunts, the pulmonary vascular bed is subjected to ongoing shear stress. For a large unrestrictive defect, the pulmonary and systemic systolic arterial pressures are the same. This leads to the histopathologic changes seen in pulmonary arterial hypertension, which include pulmonary arteriolar medial hypertrophy, intimal

Clinical history/symptoms

Timely diagnosis of a large systemic to pulmonary shunt is critical in the prevention of Eisenmenger's syndrome. Defects closed within the first 2 years of life are unlikely to lead to pulmonary vascular obstructive disease.6 Infants with large unrestrictive defects usually present initially with signs of congestive heart failure, ie, failure to thrive, diaphoresis, and tachypnea, caused by increased pulmonary blood flow from left to right shunting across the congenital heart defect. After

Management

The treatment of patients with Eisenmenger's syndrome may include the use of supplemental oxygen, digitalis, diuretics, vasodilators, anticoagulants, or lung transplantation and repair of the congenital heart defect(s) or heart-lung transplantation.17

Future directions

There are several new therapeutic agents (alone as well as in combination) that are currently being evaluated or considered for clinical investigation for patients with pulmonary arterial hypertension. These include prostacyclin analogues, endothelin receptor antagonists (both dual receptor and endothelin A selective receptor), elastase inhibitors, inhaled nitric oxide, phosphodiesterase inhibitors, eg, sildenafil (phoshodiesterase type 5 inhibitor), and angiotensin-converting enzyme

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    ☆

    Address reprint requests to Erika Berman Rosenzweig, MD, Division of Pediatric Cardiology, Children's Hospital of New York, New-York Presbyterian, 3959 Broadway, 2 North, New York, NY 10032; [email protected].

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    Copyright © 2002 Elsevier Science (USA). All rights reserved.