Eisenmenger syndrome is a complication of a congenital heart defect. The condition occurs when the direction of blood flow changes, causing blood low in oxygen to be pumped throughout the body. When this occurs, the tissues and organs don't receive as much oxygen as they need, which can result in serious complications. Though people with Eisenmenger syndrome require close monitoring by a physician, treatment can help improve the symptoms and prognosis.
The heart has two sides, each responsible for different functions. The right side pumps blood to the lungs, where it receives oxygen. After being oxygenated, the blood travels to the left side of the heart, from which the organ pumps it into the aorta, a large vessel that circulates blood to the rest of the body. Valves open to allow blood to flow into the heart's chambers and arteries, and close to prevent blood from flowing backward through the heart.
The cause of the condition is a shunt or hole between the main blood vessels or chambers of the heart, with which an individual is born. Several heart defects can lead to Eisenmenger syndrome. In each case, the pressure of blood traveling through the shunt results in increased pressure in the pulmonary artery. With time, this damages the blood vessels in the lungs. Eisenmenger syndrome happens when blood begins to flow backward through the shunt, which causes oxygen-rich blood to mix with oxygen-poor blood, lowering the amount of oxygen being pumped to the rest of the body.
Several congenital heart defects can lead to the development of Eisenmenger syndrome:
Many symptoms of Eisenmenger syndrome develop due to a lack of oxygenated blood in the tissues and organs. Symptoms include abdominal swelling, tingling and numbness in the fingers and toes, coughing up blood, racing or skipped heartbeats (palpitations), large, rounded fingernails or toenails, quick-onset fatigue, cyanosis (bluish or gray skin, fingers, toes, and lips), chest tightness or pain, dizziness, fainting, and headaches. Someone with Eisenmenger syndrome may also experience shortness of breath during physical activities and at rest.
Possible complications of Eisenmenger syndrome include:
If a physician suspects Eisenmenger syndrome, he may order one or more tests:
There is no cure for Eisenmenger syndrome, and medication is the primary treatment. Medications that control heart rhythm, iron supplements, blood-thinning medications, and vasodilators can help improve symptoms and prevent serious complications. People with Eisenmenger syndrome may need to take antibiotics before dental or medical procedures, as well.
Doctors may recommend phlebotomy to a patient whose high red blood cell count is causing symptoms such as visual disturbances, headaches, and difficulty concentrating. In this procedure, a machine drains some blood from the body to decrease the red blood cell count. The patient typically receives intravenous (IV) fluids to help replace those lost during the procedure.
Those who do not respond to other treatment methods may eventually need a heart and lung transplant or a lung transplant and surgical repairing of the hole in the heart. Physicians do not generally recommend surgery to repair the congenital heart defect the individual has developed Eisenmenger syndrome, as the procedure is life-threatening.
People with Eisenmenger syndrome require careful monitoring by a cardiologist specializing in congenital conditions throughout their lifetime. How well the patient fairs depends on whether he has any other medical conditions and the age at which high blood pressure in the lungs first occurs. Symptoms typically get worse with time.
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