Eisenmenger's syndrome (or Eisenmenger's reaction) is defined as the process in which a left-to-right shunt caused by a ventricular septal defect in the heart causes increased flow through the pulmonary vasculature, causing pulmonary hypertension, which in turn, causes increased pressures in the right side of the heart and reversal of the shunt into a right-to-left shunt.
- Abnormal heart rhythm (arrhythmia)
- Bluish lips, fingers, and toes (cyanosis)
- Chest pain
- Coughing up blood
- Feeling tired
- Heart attack
- Shortness of breath
- Swelling in the joints caused by too much uric acid (gout)
Eisenmenger syndrome is caused by a defect in the heart. Most often, babies with this condition are born with a hole between the two pumping chambers -- the left and right ventricles -- of the heart (ventricular septal defect). The hole allows blood that has already picked up oxygen from the lungs to flow back into the lungs, instead of going out to the rest of the body. Other heart defects that can lead to Eisenmenger syndrome include:
- Atrioventricular canal defect
- Atrial septal defect
- Cyanotic heart disease
- Patent ductus arteriosus
- Persistent truncus arteriosus
Surgery as early as possible to correct the heart defect can prevent Eisenmenger syndrome.
Conditions needed for a person to be diagnosed with Eisenmenger's Syndrome are:
- an underlying heart defect that allows blood to pass between the left and right sides of the heart.
- pulmonary hypertension, or elevated blood pressure in the lungs
- polycythemia, an increase in the number of red blood cells
- the reversal of the shunt
How well the infant or child does depends on whether another medical condition is present, and the age at which high blood pressure develops in the lungs. Patients with this condition can live 20 to 50 years.
Older children with symptoms may have blood removed from the body (phlebotomy) to reduce the number of red blood cells, and then receive fluids to replace the lost blood (volume replacement). Children may receive oxygen, although whether it works is unclear. Children with very severe symptoms may need a heart-lung transplant.