Bicuspid Aortic Valve Health Article

Signs and symptoms

Many people with bicuspid aortic valve experience no symptoms and may live their entire lives unaware of the condition. However, progressive damage or infection of the valve may lead to three serious conditions: aortic stenosis, aortic regurgitation, or endocarditis.

As a person ages, calcium deposits on a bicuspid aortic valve making it stiff. Eventually, the valve may become so stiff that it does not open properly, making it more difficult for blood to leave the heart and pass into the aorta and resulting in aortic stenosis. When this blockage becomes serious enough, people may experience shortness of breath, chest pain, or fainting spells. These symptoms usually begin between the ages of 50 and 60 years old. Eventually, the blockage can become so bad that blood backs up in the heart and lungs instead of going out to supply the rest of the body with oxygen (congestive heart failure). Additionally, this condition can lead to thickening of the heart wall, which may cause abnormal heart rhythms leading to sudden death.

Aortic regurgitation results when the valve fails to close properly. People who develop this condition may become short of breath when exerting themselves. The extent of symptoms experienced by the patient depends on the severity of the aortic regurgitation.

Finally, bacteria may deposit on the malformed bicuspid aortic valve, causing endocarditis. People with endocarditis may have symptoms of lingering fevers, fatigue, weight loss, and sometimes damage to the kidneys or spots on their fingers and hands.

Other dangerous conditions associated with bicuspid aortic valve include aortic aneurysm and aortic dissection. People with aortic aneurysms usually do not experience symptoms unless the aneurysm ruptures, but people with aortic dissection experience tearing back pain. Aortic aneurysm rupture and aortic dissection are very dangerous and can rapidly lead to death if not promptly treated.

Diagnosis

Any of the symptoms of aortic stenosis, aortic regurgitation, or endocarditis should prompt a search for an underlying malformation of the aortic valve. Aortic stenosis or regurgitation is diagnosed by a combination of physical exam and cardiovascular tests and imaging. The earliest sign of aortic valve problems is a murmur (the sound of abnormal patterns of blood flow) heard with a stethoscope. When the valve has high levels of calcium deposits, a characteristic clicking sound can also be heard with the stethoscope just as the stiff valve attempts to open. Later signs include a large heart seen on x ray or by a special electrical test of the heart, called an ECG or EKG (electrocardiogram).

If these signs are present, it suggests that the aortic valve may be damaged. The next test to be performed is echocardiography, a method that uses ultrasound waves to look at the aortic valve, similar to the way in which ultrasound is used to look at a fetus during pregnancy. Often, only two cusps are seen on the aortic valve during the echocardiography, confirming a diagnosis of bicuspid aortic valve.

Endocarditis is diagnosed by demonstrating the presence of bacteria in the blood stream. This is performed by taking blood from the patient and growing

the bacteria on plates with specialized nutrients. Skilled technicians can then use different tests to identify which species of bacteria is present so that appropriate treatment can be started. The diagnosis of endocarditis is also confirmed by using echocardiography to look for bacterial growths on the aortic valve. During the echocardiography, a bicuspid valve is often seen and explains the tendency to develop endocarditis.

Treatment and management

Most people with bicuspid aortic valve will not experience any complications or symptoms and will not require treatment. However, in patients with any complication of valve damage, as previously discussed, treatment may be necessary.

In younger patients who have aortic stenosis, a procedure can be performed in which a small balloon is inserted through one of the major blood vessels and into the aortic valve. The balloon is then inflated, creating a bigger opening for blood to pass. Alternatively, an "open heart" procedure can be performed to cut the valve into a more normal configuration. These treatments are usually temporary, and later in life the patient, as well as any adult with advanced aortic stenosis, will most likely require aortic valve replacement.

Valve replacement is an "open heart" operation where the original malformed valve is removed and replaced with a new valve. This new valve can come from a human donor who has died, or from cows or pigs, or even from another part of the patient's heart. These valves function well, but may need to be replaced after 10 to 20 years, as they wear out. Another option is to use an artificial valve made of metal, plastic, or cloth. However, people who receive these artificial valves need to take blood thinners every day in order to prevent blood clots from forming on the new valve.

Patients with endocarditis need to be hospitalized and treated with high does of antibiotics given through a vein for several weeks. Damage done to the valve by the bacteria may make it necessary for a valve replacement procedure to be performed after the patient has recovered from the infection.

In any case, people who have been identified as having bicuspid aortic valve should be followed regularly by a cardiologist, with possible consultation with a cardiothoracic surgeon. The function of the bicuspid aortic valve should be followed through the use of echocardiography, and the state of the heart itself should be followed by regular electrocardiograms.

It should be noted that children with aortic stenosis may not be able to engage in vigorous physical activity without the risk of cardiac arrest and should consult their physician. In addition, all people with bicuspid aortic valve should receive antibiotics prior to any dental procedure or surgery; these procedures may allow bacteria to enter the blood stream and could result in endocarditis if antibiotics are not given beforehand.