Eisenmenger’s syndrome overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Abdelrahman Ibrahim Abushouk, MD[2], Priyamavada Singh, MBBS [3];Kristin Feeney, B.S. [4]

Overview

Eisenmenger's syndrome or Eisenmenger's reaction is defined as the process in which a left-to-right shunt in the heart causes increased flow through the pulmonary vasculature, which leads to pulmonary hypertension, which finally causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt. This right to left shunt causes the patient to become cyanotic. Thus, Eisenmenger's syndrome is said to develop when there is a pulmonary artery disease, right-to-left heart shunting and cyanosis.

Historical Perspective

Victor Eisenmenger first described the syndrome in 1897 in a patient who presented with dyspnea and cyanosis since infancy. Since then, advances in the medical treatment of pulmonary hypertension improved the survival of Eisenmenger's syndrome.

Causes

Eisenmenger's syndrome is causes by cardiac defects that shunt blood heading to the systemic circulation back into the pulmonary circulation. These defects include ventricular septal defect, atrial septal defect, atrioventricular canal defect, patent ductus arteriosus, and truncus arteriosus.

Epidemiology and Demographics

The incidence and prevalence of Eisenmenger's syndrome has been decreasing gradually over the years. In the general population, the prevalence decreased from 24.6 to 11.9/million inhabitants in 2012. About 8% of patents with congenital heart diseases develop Eisenmenger's syndrome. The mortality rate of Eisenmenger's syndrome is about 27%. The risk of Eisenmenger's syndrome increases in patients with large cardiac defects or who live in developing countries due to poor healthcare access.

Risk Factors

The common risk factors for Eisenmenger's syndrome include genetic mutations, exposure to rubella virus, and drug and alcohol abuse during pregnancy. Less common risk factors include poor healthcare access and patients in developing countries.

Diagnosis

Laboratory Findings

The laboratory findings of patients with Eisenmenger's syndrome include erythrocytosis, iron deficiency, increased bleeding time, increased blood uric acid and conjugated bilirubin concentrations, as well as mixed respiratory and metabolic acidosis.

Electrocardiogram

Eisenmenger's patients may show some ECG findings, including right axis deviation (ventricular hypertrophy), ST changes (Right ventricular or biventricular hypertrophy), P Pulmonale (Right atrial hypertrophy), tall R wave in V₁ and deep S wave in V₆, and microvolt T-wave alternans.

X-ray

Chest X-ray of patients with Eisenmenger's syndrome may show cardiomegaly, right ventricular or biventricular enlargement, right atrial or biatrial enlargement, and pulmonary vascular plethora.

CT scan

Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The findings on CT scan may include large main pulmonary artery, ventricular septal defect, and right ventricular hypertrophy.

MRI

Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive. The following can be observed on MRI examination of Eisenmenger's syndrome patients: Magnitude and direction of the cardiac shunt, reduced systolic function of the cardiac ventricles, and brain diffusion changes on brain MRI.