Options For Treating Eisenmenger Syndrome

Phlebotomy

Phlebotomy is the drawing of blood from some patients with Eisenmenger syndrome. This may seem to be counterintuitive since their blood is iron-poor, but it seems the body makes up for low iron by making the blood especially viscous, or sticky, which puts the patient at risk for symptoms such as bleeding out from their mucous membranes, retinopathy, convulsions, and coma. The procedure is also done to prepare the patient for a donation of their blood if their hematocrit level is abnormally high. The hematocrit measures the number of red blood cells against the rest of the components in the blood. When a patient with Eisenmenger syndrome is given a phlebotomy, their symptoms usually ease within a day.

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Heart-Lung Transplant

Sometimes a heart-lung transplant to replace the damaged heart and lungs is the only treatment of choice for an Eisenmenger syndrome patient. The success of the transplant depends on the particular physiology of the patient. Patients with complex cardiac anatomy do not do as well as patients with simple anatomy. Complex cardiac anatomy means the patient’s heart is different in some way from a normal heart above and beyond their Eisenmenger syndrome. For example, the patient may have a situs inversus situation where the heart is, basically, a mirror image of a normal heart. It may be on the right side of the body with the blood vessels, atria and ventricles also the opposite of what is normal. Individuals with complex cardiac anatomy also tended to need the transplant at younger ages. However, many patients do surprisingly well after a heart-lung transplant.

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