What is Eisenmenger Syndrome & How is it Treated?

Eisenmenger Syndrome is a rare congenital heart defect characterized by a hole between the chambers of the heart which causes the blood to abnormally circulate between the lungs and the heart. As a result of this abnormality, the increased blood flow returns to the lungs instead of going to the rest of the body causing an increase in the pressure of the arteries in the lungs such that they become excessively stiff and narrow.

This results in permanent damage to the blood vessels in the lungs. Eisenmenger Syndrome also causes deoxygenated blood to flow back through the hole in the heart leading to the deoxygenated blood circulating throughout the body. This results in various important organs of the body receiving less oxygen than is required for their normal functioning. Eisenmenger Syndrome is a potentially serious heart defect and once diagnosed the affected individual should be closely monitored and adequately medicated to prevent any serious complications of Eisenmenger Syndrome.

As stated, the root cause of Eisenmenger Syndrome is a hole between the chambers of the heart. This hole is congenital and is not acquired due to any other reason. Some of the heart defects that can cause Eisenmenger Syndrome are:

Ventricular Septal Defect: In this defect, there is a hole between the right and left ventricles of the heart and is believed to be the most common cause of Eisenmenger Syndrome.

Atrial Septal Defect: In this defect, there is a hole in the wall of the tissue which divides the right and left sides of the atria and is also one of the causes of Eisenmenger Syndrome.

Patent Ductus Arteriosus: In this type of defect, there is a hole between the pulmonary artery which carries the deoxygenated blood to the lungs and the aorta which carries oxygen rich blood to the rest of the body.

Atrioventricular Canal Defect: This is a type of defect in which there is a hole in the heart between the atria and the ventricles resulting in the some of the valves of the heart malfunctioning as a result of Eisenmenger Syndrome.

In each of the above mentioned defects, the excessive pressure with which the blood flows through the hole increases the pressure in the pulmonary artery.

As the disease condition progresses, the blood vessels in the lungs tend to get affected and may even get permanently damaged. As a result of this damage to the blood vessels, it becomes very difficult for the red blood cells to take up oxygen and carry them to the rest of the body.

Yet another cause of Eisenmenger Syndrome is the backflow of the blood such that the deoxygenated blood mixes with the oxygenated blood thus contaminating the oxygenated blood meant to be carried to the rest of the body.

As a result of this, there is decreased oxygen supply to the various important organs of the body resulting in the affected individual having cyanosis or bluish discoloration of the skin due to lack of oxygen supply. This causes an increase in the production of blood cells to make up for the lack of oxygen. Over time, if the condition is not diagnosed and treated, it will result in potentially serious complications as result of Eisenmenger Syndrome. An individual with a family history of heart defects is at an increased risk for developing a condition like Eisenmenger Syndrome.

Some of the symptoms of Eisenmenger Syndrome are:

• Cyanosis or bluish discoloration of the skin
• Rounded fingernails
• Shortness of breath with even mild activity and sometimes even at rest
• Chest pains
• Palpitations
• Syncopal spells
• Hemoptysis
• Frequent dizziness
• Numbness or tingling sensation in the upper and lower extremities
• Frequent headaches
• Swelling in the abdominal areas

In order to diagnose Eisenmenger Syndrome, the treating physician will begin by taking a detailed history of the patient including a family history of any cardiac defects within the family.

The physician will perform a thorough physical examination including asking the patient to walk for a few minutes monitoring for any signs of intolerance or symptoms of shortness of breath or difficulty. This will clearly point towards a cardiac defect.

Once a cardiac source of discomfort is established then the physician will order an EKG and electrocardiogram to look at the electrical activity of the heart which will clearly show an abnormality due to the hole present in the heart as a result of Eisenmenger Syndrome.

Additionally a chest x-ray will also be done to check on the status of the lungs along with advanced imaging studies like MRI and CT scans of the lungs to check for any abnormality such as damage to the arteries or vessels of the lungs due to Eisenmenger Syndrome.

A blood test may also be done which will clearly reveal increased levels of red blood cells which will point towards a diagnosis of Eisenmenger Syndrome. A cardiac catheterization will be done in which a catheter with a minature camera will be inserted into the heart which will clearly show the presence of a hole and confirm the diagnosis of Eisenmenger Syndrome.

The treatment of Eisenmenger Syndrome is purely symptomatic and requires coordinated efforts from various specialties. The patient will require medications to help prevent complications of Eisenmenger Syndrome.

Such patients require extremely close monitoring to check for the status of the heart and lungs. Iron deficiency anemia that develops as a result of this condition may be treated with iron supplements.

Additionally, medications may be required for prevention of edema and fluid retention which is common in cardiac defects. People with Eisenmenger Syndrome are also at an increased risk for developing bacteria infections and hence are recommended to take appropriate antibiotics before any dental or other surgical procedures.

It is also essential that individuals with Eisenmenger Syndrome stay hydrated as dehydration is quite detrimental for patients with Eisenmenger Syndrome and may tend to cause a sudden drop in blood pressure such as high altitudes to prevent any complications from Eisenmenger Syndrome.