Eisenmenger Complex

Eisenmenger Complex is the term used to describe the resultant effects of prolonged pulmonary hypertension in patients with uncorrected congenital heart defects, which eventually results in a reversal of the shunting of blood within the heart (see Pulmonary Hypertension Topic Note), so that it goes from right to left.

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 patient 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 Polycythaemia. 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 haemoglobin (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.

• Anticoagulants
  Drugs such as Warfarin are anticoagulants, which help to stop the blood clotting. This reduces 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.
• Venesection
  This is a procedure by which some of the blood is removed from the patient, and may or may not be replaced by plasma or other fluids. By removing some of the blood which contains high levels of haemoglobin (red cells), this reduces the concentration of red cells. In some patients this can be quite effective, and the body may take several months to reproduce the number of red cells. In others the red cells are replaced far more quickly. There is the possibility of the patient going into shock as a result of the treatment, so cardiologists are cautious about its use, and the benefits need to be weighed against the possible risks with each individual.
• Oxygen
  The use of oxygen, usually by mask or nasal cannula can be effective in raising the blood saturation (oxygen level) and reducing the workload on the heart. Many patients benefit from using oxygen overnight when it does not prohibit activity, and this gives their body 'a boost' for the following day.
• Pulmonary Vasodilators
  There are some new developments for children and young adults with Eisenmengers. Drugs are undergoing trials to see whether they are effective in Down's Syndrome and Eisenmengers.

Follow up Advice

It is important for patients to avoid becoming dehydrated, and for them to be carefully monitored during any medical or dental procedures, and appropriate antibiotic cover given.

Prognosis

Whilst there is no treatment to cure the underlying problem, the treatments mentioned above may be quite effective in helping to lessen the symptoms. Although there is of course variation in patients, it is possible for someone with Down's Syndrome who has Eisenmengers Complex, to survive for many years with a quite reasonable quality of life, although probably with reduced activity levels.

"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".