Options For Treating Eisenmenger Syndrome

Eisenmenger syndrome is a complication of a congenital heart problem and is usually caused when there is a hole between the two chambers of the heart. Because of this, the blood cannot circulate normally, and blood flows into the lungs that should circulate the rest of the patient's body. This damages the arteries in the lungs and increases the pressure in them to the point where the blood flow is reversed. Because of this, oxygen-starved, as opposed to oxygen-rich, blood circulates around the individual's organs. 

The person has a bluish cast to their skin, clubbed finger and toenails, chest pain, heart palpitations, and numbness and tingling in their extremities. They are also subject to fainting, fatigue, dizziness, and headaches. Eisenmenger syndrome patients also cough up blood and have abdominal swelling.

Iron Supplements


Doctors often recommend iron supplements to patients who have Eisenmenger syndrome and whose iron levels are low. People may wonder why these patients need iron supplements as opposed to other types of supplements that can support their health. The one thing about iron is it's the mineral that helps bring oxygen to the cells, which is something individuals with Eisenmenger syndrome desperately need. Iron makes up hemoglobin, a pigment in red blood cells that actually carries oxygen. The supplements, which can come in tablets or as a liquid, need to be taken under a doctor's supervision, as too much iron is toxic. This is especially true for children.



These are medications that cause the blood vessels to open, resulting in a reduction in the pressure in the arteries to the lungs, better circulation of blood, and better respiration in both lungs. Vasodilators given to patients with Eisenmenger syndrome both extend their lives and improve the quality of their lives. There are different types of medicines to ease the pulmonary hypertension that accompanies Eisenmenger syndrome. One of them is oxygen. The lack of oxygen, or hypoxemia, causes blood vessels to narrow, while oxygen therapy causes them to open. Other vasodilators are calcium channel blockers that stop the movement of calcium ions into the smooth muscle cells of the arteries and allow them to relax and open up. Another medication that helps blood vessels dilate is nitric oxide gas.


Cedars-Sinai Blog

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.

Heart-Lung Transplant

Science Factorama

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.

Avoid High Altitudes

Tourism Whistler

High altitudes are a challenge even for the fittest mountain climber, and sometimes even they need to resort to supplemental oxygen. A patient with Eisenmenger syndrome that is not under control should avoid high altitudes, and even a healthy person should consult with their doctor if they plan to go to a place over eight thousand feet above sea level. This is simply because high altitude areas have less oxygen than lower areas, and an individual with Eisenmenger syndrome already has difficulty getting enough oxygen. 

People who suffer from altitude sickness often become nauseated and dizzy. They have a headache, struggle to breathe when they are exerting themselves, have trouble falling asleep, and suffer from tachycardia. If they do not acclimate themselves to the new altitude before climbing higher and refuse to return to a lower altitude, they may have trouble breathing even when they are resting. Other symptoms are chest pain, vomiting, mental confusion, vomiting, and cyanotic skin. At even higher altitudes the lungs and brain can swell up with fluid, which is life-threatening.


    HealthPrep Staff