The effect of this right to left shunting is to pump blood from the right side of the heart which takes blood to the lungs, through the septal defect(s) (hole or holes in the central heart wall) and into the left side of the heart which takes blood to the body. Thus a proportion of blood bypasses the lungs and is not oxygenated, so the level of oxygen (saturation) in the blood decreases and the patient is cyanosed (blue).

Symptoms
A child or young adult with Eisenmengers can experience blue spells', dizziness and faints, breathlessness and chest pain. They can be prone to having small strokes, or can cough up blood.

In order to compensate for the low oxygen levels (saturation) in the blood, the body may produce too many red blood cells (the cells which take the oxygen around the body). This excess of red blood cells is called Polycythemia. It causes a thickening of the blood, headaches, chest, joint and muscle pain. It can also cause visual disturbance and thrombosis (clots of blood) and embolism (moving blood clots.)

Treatment
The treatment for Eisenmengers is to maintain an optimum haemoglohin (red cell) level in the blood, which may be managed in several ways, some of which will be more effective in some patients than others.

Vasodilators

Drugs such as Hydralazine and Captopril are vasodilators. They relax the blood vessels and thereby increase their size which in turn reduces the blood pressure.

Anticoagulants

Drugs such as Aspirin are anticoagulants, which help to stop the blood clotting. This induces the risk of thrombosis and embolism and also thins the blood which makes it easier for the heart to pump around.

Diuretics

Diuretics such as Frusemide are often used to assist the body in ridding itself of excess fluid which may accumulate particularly in the lungs and liver, due to the hearts inability to work efficiently.

Digoxin

This is a drug which increases the force of contraction of the heart muscle and helps it work more effectively.

"When he was sixteen, after a long consultation with the cardiologist, we declined a heart operation, having been told that he had possibly only a fifty per cent chance of survival. Life has had many ups and downs over the years, and I cannot pretend that things have always been easy. He is still ill at times, he is blue and gets breathless and tired, but he seems to enjoy life to the full. We celebrated his twenty-first birthday with a big party and disco for family and friends, and a year later he was best man at his brother's wedding, and looked extremely smart in his grey tail suit and waistcoat."