As blood leaves each chamber of the heart, it passes through a valve. There are four valves in your heart. They make sure that blood flows in only one direction through your heart.
Congenital valve defects are valves not formed correctly before birth. Valves may be the wrong size, have malformed leaflets or have leaflets that are not attached to the annulus correctly. This most often affects the aortic or pulmonic valve.
Bicuspid aortic valve
Bicuspid aortic valve disease is a congenital valve disease that affects the aortic valve. Instead of the normal three leaflets or cusps, the bicuspid aortic valve has only two. Without the third leaflet, the valve may be:
Normal aortic valve
Bicuspid aortic valve
- stenotic - stiff valves that can not open or close properly
- leaky - not able close tightly
This occurs more frequently in some family members. About 1/4 of patients may have some enlargement of the aorta above the valve. Bicuspid aortic valve disease affects about 2 percent of the population. Eighty-five percent of children with congenital aortic stenosis have a bicuspid aortic valve.
Symptoms
The clinical course for those with congenital bicuspid valve disease varies from patient to patient. Most children with bicuspid aortic valve have a heart murmur. Some may have no symptoms at all and others have severe symptoms beginning shortly after birth. Symptoms in the adult include:
- chest pain
- fatigue (feeling over-tired)
- shortness of breath
- dizziness
- fainting
Treatment
Treatment involves surgical repair or replacement of the aortic valve or congenital heart disease surgery. In some children and adults, a non-surgical, invasive procedure, called balloon valvotomy, may be used to treat aortic stenosis.
What is endocarditis?
Endocarditis occurs when germs (especially bacteria) enter your blood stream and attack the lining of your heart valves, causing growths on the valve, holes in the valve or scarring of the valve tissue, most often resulting in leaky heart valves.
Those with congenital valve disease:
- need lifelong medical follow-up with a heart specialist
- need to protect themselves from a infection of the heart, called endocarditis, even if the heart was repaired or replaced through congenital heart disease surgery
To protect yourself:
- Tell all doctors and dentists you have congenital heart disease. You may want to carry a card with this information.
- Call your doctor if you have symptoms of an infection (sore throat, general body achiness, fever). Colds and flus do not cause endocarditis. But infections, which may have the same symptoms, do. So, to be safe, call your doctor.
- Take good care of your teeth and gums to prevent infections. See your dentist for regular visits.
- Take antibiotics before you undergo any procedure that may cause bleeding:
- any dental work (even a basic teeth cleaning)
- invasive tests
- most major or minor surgery
Check with your doctor about the type and amount of antibiotics you should take. Click here for more information about bacterial endocarditis prevention.
There are other types of congenital heart disease which affects the pulmonic, tricuspid and mitral valve, although these are very rare and most often treated in childhood.
How to find a doctor if you have congenital valve defect
Congenital Heart Disease in Children and Adults Quality Indicator Guide
Doctors vary in quality due to differences in training and experience; hospitals differ in the number of services available. The more complex your medical problem, the greater these differences in quality become and the more they matter.
Clearly, the doctor and hospital that you choose for complex, specialized medical care will have a direct impact on how well you do. To help you make this choice, read more about our Miller Family Heart & Vascular Institute outcomes.
Miller Family Heart & Vascular Institute cardiologists and surgeons
Click here to find a Cleveland Clinic and Vascular Institute Cardiologist or Surgeon who specializes in the diagnosis and treatment of patients with congenital valve disease.
For younger patients:
For more information:
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If you need more information, click here to contact us , chat online with a nurse or call the Miller Family Heart & Vascular Institute Resource & Information Nurse at 216/445-9288 or toll-free at 866/289-6911. We would be happy to help you.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition.
