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Attention: The terms Eisenmenger’s Disease, Eisenmenger’s Complex, Eisenmenger’s Syndrome, and Eisenmenger’s Reaction are all used interchangeably in the literature and a consensus seems to be missing amongst clinicians and scientists. It is the editors’ opinion that only the term “Eisenmenger Reaction” clearly refers to the pathologic process of shunt reversal leading to right-to-left shunt and severe pulmonary hypertension. Although we elected to use this terminology to title this medical condition, the reader shall find all information below in agreement with the literature and different terminology.

At a glance

It is a medical condition defined as a long-standing left-to-right cardiac shunt typically caused by the presence of a ventricular septal defect, atrial (atrioseptal) defect or less frequently, a large patent ductus arteriosus. These vascular conditions eventually lead to significant pulmonary hypertension (raised pulmonary vascular resistance; PVR) and eventually reversal of the intracardiac shunt into a right-to-left shunt, causing severe cyanosis. Fortunately, with the advent of echocardiography in early age, the incidence of one of these heart defects progressing later in life to Eisenmenger’s reaction has decreased considerably. In a pregnant mother, the presence of Eisenmenger’s Syndrome can cause serious complications and must be carefully managed early in the pregnancy. Maternal mortality ranges from 30 to 60%. It is often associated with clinical considerations such as fainting spells, thromboembolism, severe hypovolemia, hemoptysis, and later preeclampsia. Most maternal deaths occur either during or within the first weeks after delivery. Pregnant women with Eisenmenger Syndrome should be hospitalized after the 20th week of pregnancy or earlier if the clinical condition deteriorates.

Synonyms

Eisenmenger Disease; ES: Eisenmenger’s Syndrome; Eisenmenger’s Physiology; Tardive Cyanosis Syndrome; Eisen­menger’s Complex (see “Other conditions to be considered” below).

History

The Austrian physician Viktor E. Eisenmenger (1864-1932) was the first to describe in 1897 severe pulmonary vascular disease in a 32-year-old man with cyanosis and dyspnea that was present since infancy and secondary to an unrestricted ventricular septal defect. The patient died of massive pulmonary hemorrhage. This syndrome was named in 1958 by Dr Paul Wood in the honor of Dr Eisenmenger.

Incidence

Approximately 8% of patients with congenital heart disease and 11% of those with left-to-right intracardiac shunting develop Eisenmenger reaction. In the United Kingdom, pulmonary hypertension has a prevalence of 9.7/100,000 with a female:male ratio of 1.8:1.

Genetic inheritance

Eisenmenger reaction per se is not inherited. It is an acquired complication of a congenital cardiac lesion that leads to the development of this medical condition. There is no sexual predilection reported.

Pathophysiology

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure (PAPm) greater than or equal to 25 mm Hg at rest (Normal PAPm at rest 14±3 mm Hg) assessed by right-heart catheterization (RHC). In congenital cardiac lesions with ...

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