Complications

Congenital heart disease complications that might develop years after the initial treatment include:

• Abnormal heart rhythms (arrhythmias). Arrhythmias occur when the electrical impulses that coordinate heartbeats don't function properly, causing your heart to beat too fast, too slowly or irregularly. In some people, severe arrhythmias can cause sudden cardiac death if not treated.

• Heart infection (endocarditis). Your heart comprises four chambers and four valves, which are lined by a thin membrane called the endocardium. Endocarditis is an infection of this inner lining, which generally occurs when bacteria or other germs enter your bloodstream and lodge in your heart. Untreated, endocarditis can damage or destroy your heart valves or trigger a stroke.

  If you have an artificial (prosthetic) heart valve or your heart was repaired with prosthetic material, or if your heart defect wasn't completely repaired, your doctor might prescribe ongoing antibiotics to lower your risk of developing endocarditis.

• Stroke. Stroke occurs when the blood supply to a part of your brain is interrupted or severely reduced, depriving brain tissue of oxygen. A congenital heart defect can allow a blood clot to pass through your heart and travel to your brain.

  Certain heart arrhythmias also can increase your chance of blood clot formation leading to a stroke.

• Heart failure. Heart failure, also known as congestive heart failure, means your heart can't pump enough blood to meet your body's needs. Some types of congenital heart disease can lead to heart failure.

  Over time, certain conditions such as coronary artery disease or high blood pressure gradually sap your heart of its strength, leaving it too weak or too stiff to fill and pump efficiently.

• Pulmonary hypertension. This is a type of high blood pressure that affects the arteries in your lungs. Some congenital heart defects cause more blood to flow to the lungs, causing pressure to build and making your heart work harder. This eventually causes your heart muscle to weaken and sometimes to fail.
• Heart valve problems. In some types of congenital heart disease, the heart valves are abnormal.

Coping and support

One important thing to do if you're an adult with congenital heart disease is to become educated about your condition. Topics you should become familiar with include:

• The name and details of your heart condition and its past treatment
• How often you should be seen for follow-up care
• Information about your medications and their side effects
• How to prevent heart infections (endocarditis), if necessary
• Exercise guidelines and work restrictions
• Birth control and family planning information
• Health insurance information and coverage options
• Dental care information, including whether you need antibiotics before major dental procedures
• Symptoms of your congenital heart disease and when you should contact your doctor

Many adults with congenital heart disease lead full, long and productive lives. But it's important not to ignore your condition. Become informed about your disease; the more you know, the better you'll do.

Preparing for an appointment

If you have a congenital heart defect, make an appointment with your doctor for follow-up care, even if you haven't developed complications. You'll likely be referred to a doctor trained in diagnosing and treating heart conditions (cardiologist).

Here's some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there's anything you need to do in advance, such as restrict your diet or fast. Make a list of:

• Your symptoms, if any, including any that may seem unrelated to congenital heart disease, and when they began
• Key personal information, including a family history of heart defects and treatment you received as a child
• All medications, vitamins or other supplements you take and their doses
• Questions to ask your doctor

Take a family member or friend with you, if possible, to help you remember the information you get. For congenital heart disease, questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• What tests do I need?
• What treatments are available? Which do you recommend for me?
• Are there diet or activity restrictions I need to follow?
• How often should I be screened for complications from my heart defect?
• I have other health conditions. How can I best manage these conditions together?
• Are there brochures or other printed material that I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your doctor is likely to ask you questions, including:

• Do your symptoms come and go, or do you have them all the time?
• How severe are your symptoms?
• Does anything seem to improve your symptoms?
• What, if anything, worsens your symptoms?
• What's your lifestyle like, including your diet, tobacco use, physical activity and alcohol use?

Overview

Bicuspid aortic valve is a heart condition present at birth. That means it is a congenital heart defect.

The aortic valve is between the left lower heart chamber and the body's main artery, called the aorta. Flaps of tissue on the valve open and close with each heartbeat. The flaps are called cusps. They make sure blood flows in the correct direction.

Usually the aortic valve has three cusps. A bicuspid valve has only two cusps. Rarely, some people are born with an aortic valve that has one cusp or four cusps. A valve with one cusp is called unicuspid. A valve with four cusps is called quadricuspid.

Changes to the aortic valve can cause health conditions, including:

• Narrowing of the aortic valve, called aortic valve stenosis. The valve may not open fully. Blood flow from the heart to the body is reduced or blocked.
• Backward flow of blood, called aortic valve regurgitation. Sometimes, the bicuspid aortic valve doesn't close tightly. This causes blood to flow backward.
• Enlarged aorta, called aortopathy. An enlarged aorta increases the risk of a tear in the lining of the aorta. This tear is called an aortic dissection.

Symptoms

If the bicuspid valve causes severe aortic stenosis or severe aortic regurgitation, symptoms may include:

• Chest pain.
• Shortness of breath.
• Difficulty exercising.
• Fainting or near fainting.

Most people with bicuspid aortic valve don't have symptoms of heart valve disease until they're adults. But some infants may have severe symptoms.

When to see a doctor

If you think that you or your baby has symptoms of a heart condition, make an appointment for a health checkup right away.

Causes

Bicuspid aortic valve happens while the unborn baby, also called a fetus, is growing during pregnancy. Healthcare professionals aren't sure what causes most congenital heart defects, including bicuspid aortic valve. But genetics may play a role in causing bicuspid aortic valve.

Risk factors

A family history of early heart disease may increase the risk of developing heart valve disease such as bicuspid aortic valve. Sometimes bicuspid aortic valve runs in families, which means it is inherited.

Diagnosis

A bicuspid aortic valve may be found when tests are done for another health concern. The healthcare professional may hear a heart murmur when listening to the heart.

An echocardiogram can confirm a diagnosis of bicuspid aortic valve. This test uses sound waves to create videos of the beating heart. It shows how blood moves through the heart chambers, the heart valves and the aorta.

If you have a bicuspid aortic valve, you usually have a CT scan to check for changes in the aorta's size.

Treatment

If you have a bicuspid aortic valve, you are usually sent to a doctor trained in congenital heart disease. This type of doctor is called a congenital cardiologist.

Anyone with a bicuspid aortic valve needs regular health checkups and imaging tests. Echocardiograms can check for a narrowed or leaking aortic valve. The test also looks for changes in the size of the aorta.

Treatment for a bicuspid aortic valve depends on how severe the heart valve disease is. It may include medicines or a procedure or surgery to fix or replace the valve.

Medications

There are no medicines to repair a bicuspid aortic valve. But medicines may be used to treat symptoms caused by heart valve disease. For example, your healthcare professional may recommend blood pressure medicine.

Surgeries or other procedures

Surgery may be needed if a bicuspid aortic valve is causing:

• Aortic valve stenosis.
• Aortic valve regurgitation.
• An enlarged aorta.

Surgery is done to repair or replace the aortic valve. The type of surgery done depends on the specific heart valve condition and your symptoms.

• Aortic valve replacement. The surgeon removes the damaged valve. It's replaced with a mechanical valve or a valve made from cow, pig or human heart tissue. The tissue valve is called a biological tissue valve. Sometimes, the aortic valve is replaced with the person's own lung valve. The lung valve is replaced with a lung tissue valve from a deceased donor. This more complicated surgery is called the Ross procedure.

  Biological tissue valves break down over time. They may eventually need to be replaced. If you have a mechanical valve, you need to take blood thinners for life to prevent blood clots. Together, you and your healthcare team can talk about the benefits and risks of each valve type.

• Aortic root and ascending aorta surgery. Surgeons remove the enlarged section of the aorta located near the heart. It's replaced with a synthetic tube, called a graft, which is sewn into place. Sometimes, only the enlarged part of the aorta is removed. The aortic valve remains. The aortic valve also can be replaced or repaired during this surgery.

• Balloon valvuloplasty. This procedure can treat aortic valve stenosis in infants and children. In adults, the aortic valve tends to narrow again after the procedure. So it's usually only done if you're too ill for surgery or you're waiting for a valve replacement.

  This heart valve treatment uses a thin, flexible tube called a catheter. The catheter has a balloon on the tip. The surgeon inserts the catheter into an artery in the arm or groin. Then the catheter is guided to the aortic valve. Once in place, the balloon inflates, making the valve opening bigger. The balloon is deflated. The catheter and balloon are removed.

Lifestyle and home remedies

Anyone born with a bicuspid aortic valve needs health checkups for life. A doctor trained in heart diseases, called a cardiologist, should examine you for changes in your condition.

People with a bicuspid aortic valve are more likely to get an infection of the inner lining of the heart's chambers and valves. This infection is called infective endocarditis. Proper dental care can help lower your risk.

A bicuspid aortic valve can be passed down in families, meaning it's inherited. Parents, children and siblings of someone with a bicuspid aortic valve should have an echocardiogram to check for the condition.