What is Eisenmenger Syndrome

Eisenmenger reaction

The Eisenmenger reaction, also known as Eisenmenger syndrome or Eisenmenger complex, is a clinical picture that has developed from a primary left-right shunt (without cyanosis) through an increase in pulmonary vascular resistance to a later right-left shunt.

In the case of a shunt, there is an unusual passage of blood between blood vessels or heart chambers if there are malformations in the anatomical separation of the two halves of the heart. This can be, for example, a hole between the heart chambers (ventricular septal defect / VSD), a hole between the atria (atrial septal defect / ASD) or a hole between the heart chamber and atrium with malformed heart valves (atrio-ventricular septal defect / AV canal / AVSD).

The basis of this disease is fixed pulmonary hypertension (high lung pressure), caused by a congenital heart defect. As a result of the shunt reversal, the affected people have cyanosis and develop heart failure, among other things.

If left untreated, the clinical picture is associated with a shortening of lifespan. Depending on the timing of the shunt reversal, only young adulthood is reached on average. Ultimately, the only treatment available is a heart-lung transplant.

The Eisenmenger reaction was named after the Austrian doctor Victor Eisenmenger (1864-1932), who first described the peculiarity in 1897 using a 32-year-old man who had pulmonary hypertension (= pulmonary high pressure), a ventricular septal defect (VSD) and a had overriding aorta.