Investigation and Prompt Diagnosis of Patients with:

+ What is a Bicuspid Aortic Valve?

Bicuspid aortic valve is one of the most common congenital cardiac abnormalities (a condition that you are born with). It affects around 1-2 in 100 people. The aortic valve is a one way valve which lets the oxygen rich blood out of the main pumping chamber of the heart (left ventricle) and into the main artery of the body (the aorta). This valve usually has three leaflets (tricuspid) and looks like a ‘Mercedes-Benz’ sign. In bicuspid aortic valve there are two leaflets instead and the valve has a ‘fish mouth’ appearance when it opens.

As a bicuspid valve opens in a slightly different way than a normal trileaflet valve, the blood flow across the valve is often abnormal (turbulent). Usually the bicuspid valve works perfectly well for a long time, and sometimes for a whole lifetime, however, it can be prone to early wear and tear (degeneration) of the valve leaflets. This can result in a stiffening of the leaflets (stenosis) or a leaky valve (regurgitation).

+ How do people find out that they are affected?

Most people will not know that they have this condition. Sometimes it is discovered when you have an echocardiogram (ultrasound of your heart) for another reason. Most often it is discovered because your doctor has heard a heart murmur.

Will any of my family members be affected by bicuspid aortic valve?

If you have a bicuspid aortic valve there is about a 10-20% risk of your blood relatives also having a bicuspid valve or enlargement (dilatation) of the aorta. Although previously it was not thought important to screen family members, more recently doctors have started to recommend that close blood relatives have an echocardiogram (heart ultrasound) to look at their aortic valve and aorta.

+ Is Bicuspid Aortic Valve associated with any other conditions?

Bicuspid aortic valve can be associated with an enlargement (dilatation/aneurysm) of the aorta (main blood vessel leaving the heart). The aorta can be measured using echocardiography (heart ultrasound), CT or MRI. If you are found to have an enlarged aorta, your doctor may use one of these three methods to measure your aorta on a regular basis (surveillance scanning).

Less often, bicuspid valve can be associated with coarctation (narrowing) of the aorta. Coarctation is most commonly diagnosed in childhood.

+ Do I need to change my lifestyle?

If you have a bicuspid valve which is working normally and your aorta is not stretched, there are no restrictions on your lifestyle. If you have a narrowed or leaky valve, depending on the degree of the leak, your doctor may recommend that you do not take part in certain sports or exercise. If you have an enlarged (dilated) aorta, you should avoid heavy lifting as this can increase the reassure inside the aorta. Your doctor will be able to advise you on an exercise program that is best for you.

+ I have a Bicuspid Aortic Valve, is it safe for me to become pregnant?

If you have a bicuspid valve and are planning to become pregnant, you should discuss this with your cardiologist first. In most cases you will be advised that you can proceed with a normal pregnancy. However, if you have a narrowed (stenosis) or leaky (regurgitant) valve, or if you have an enlarged (dilated) aorta, this can increase the risk of complications and your pregnancy will need to be monitored carefully. In rare cases we recommend cardiac surgery before embarking on a pregnancy. Also, some of the tablets that we use to treat aortic enlargement can be harmful to your unborn baby therefore it is important to discuss plans before becoming pregnant.

+ What is the Mitral Valve?

The mitral valve is a valve situated on the left-hand side of the heart. It is a one-way valve that allows blood to move from the top chamber of the heart (left atrium) to the bottom chamber of the heart (the main pump – the left ventricle).

+ What is Mitral Regurgitation?

Mitral regurgitation is a condition where this one-way valve is not working correctly. Because the valve is unable to close tightly, a leak of blood occurs when the main pumping chamber of the heart contracts allowing blood to pass back across the valve back into the top heart chamber (left atrium).

+ What causes Mitral Regurgitation?

There are many different causes of mitral regurgitation, these include:

• Valve degeneration (wear and tear) / mitral valve prolapse
• Damage to the heart valve following heart valve infection (endocarditis)
• Congenital disorders (people born with an abnormal heart valve)
• Heart failure / following a heart attack
• Hypertrophic cardiomyopathy (a genetic condition causing thickening of the heart muscle)

+ What are the symptoms of Mitral Regurgitation?

Most people with mitral regurgitation will not experience any symptoms.

If the regurgitation (leak) is severe, then symptoms such as exertional shortness of breath, swollen ankles and tiredness can occur.

Palpitations (a feeling of your heart beating rapidly or erratically) can also be experienced by patients with mitral regurgitation.

If you experience any of the above symptoms you should inform your Healthcare Professional.

+ What tests will I need?

Most people with mitral regurgitation will have an ECG and echocardiogram. Other tests outlined below may also be performed.

• Electrocardiogram (ECG)
• Echocardiogram
• Transoesophageal Echocardiogram (TOE)
• Cardiopulmonary exercise test (CPX)
• Angiogram

+ What are the treatment options available?

Most people with mitral regurgitation will not require any treatment and will just require regular monitoring of their condition.

+ What does heart surgery involve?

A series of tests before you are referred to a surgeon will decide whether you are suitable for either a mitral valve replacement or a mitral valve repair

During a mitral valve replacement, the existing mitral valve is removed and replaced by either a mechanical (carbon) or tissue valve. Your surgeon will discuss which type of valve would be most suitable for you.

During a mitral valve repair, the surgeon will modify your existing valve to stop it from leaking.

Both these procedures require a general anaesthetic and the use of a heart lung bypass machine You will be left with a scar down the centre of the chest You will usually spend 1-2 days after the operation in intensive care and are usually discharged from hospital 7-10 days after surgery. Recovery is usually 6 weeks to 3 months. You should feel back to normal after 6 months.

+ Do I need to change my lifestyle?

As with any type of heart disease, it is important that you follow a healthy diet, keep your weight within a normal range and do not smoke Most patients with mitral regurgitation will be encouraged to take regular gentle exercise but you should check this with your doctor If you are planning pregnancy, you should discuss this with your doctor first and let them know immediately if you become pregnant

Patients with mitral regurgitation are also advised to take good care of their teeth and skin to prevent the risk of heart valve infection (which is a rare but serious condition)

• Teeth

It is important to take good care of your teeth by brushing your teeth twice a day and visiting your dentist for regular check ups (at least once per year). If you have toothache or an abscess it is important that you get treated for this quickly. Make sure you tell your dentist you have a heart condition.

• Skin

Keep your skin clean by washing regularly. Wash any cuts and grazes and keep them clean until they heal and see your GP if your skin becomes red or inflamed. Avoid cosmetic procedures (e.g tattoos, body piercing, fillers etc) that involve breaking the skin

+ What are Heart Valves?

There are four valves in the heart. They allow blood to be directed around the heart and when working normally, ensures that the blood flows in one direction. These valves open and close with every heartbeat, that’s 100,000 times a day!

+ What is the Aortic Valve?

The Aortic Valve is the main outlet valve of the heart which allows blood to exit the heart with every heartbeat. Normal aortic valves have three leaflets, but around 1 in 50 people are born with an abnormal heart valve which has two leaflets, known as a bicuspid aortic valve.

+ What is Aortic Stenosis?

Aortic stenosis is a condition where the valve becomes thickened and is less able to open well. As a result, the blood flow the valve is abnormal and means that a heart murmur can be heard when your is listened to by a stethoscope.

+ What causes Aortic Stenosis?

• The most common cause of aortic stenosis is age related wear and tear (degeneration) and as a result most people who have the condition are over the age of 60.
• In people born with an abnormal bicuspid valve, the valve can become thickened (stenosed) at a younger age, and people with a bicuspid valve can be affected at any stage in their lives
• Sometimes in addition to being narrowed, the aortic valve can also be leaky (aortic regurgitation)
• Patients with both aortic valve narrowing and leaking have a condition called mixed aortic valve disease

+ What are my treatment options?

• Aortic stenosis is a long term condition
• It can be graded into three categories mild, moderate and severe
• Regardless of the severity of aortic stenosis, if you have no symptoms then it is likely that the Heart Valve Team will keep you under review with a clinic visit and echocardiogram (echo or cardiac ultrasound) on a regular basis
• Patients with aortic stenosis are often followed up for many years without symptoms
• If the valve is severely narrowed (severe aortic stenosis) and you have symptoms then you may be referred for aortic valve surgery, which can be performed as either open heart surgery or keyhole valve replacement
• Due to the importance of symptoms in patients with aortic stenosis, you must let your Healthcare Professional know if you develop symptoms in between clinic appointments

+ Is aortic stenosis associated with any other conditions?

In patients with a bicuspid aortic valve, there can also be abnormalities of the aorta, and sometimes in addition to undergoing a regular echocardiogram you may also undergo regular CT or MRI scans.

+ What tests will I need?

Most people with mitral regurgitation will have an ECG and echocardiogram. Other tests outlined below may also be performed.

• Electrocardiogram (ECG)
• Echocardiogram
• Transoesophageal Echocardiogram (TOE)
• Cardiopulmonary exercise test (CPX)
• Angiogram

+ Lifestyle

As with any type of heart disease, it is important that you follow a healthy diet, keep your weight within a normal range and do not smoke. Most patients with aortic stenosis will be encouraged to take regular gentle exercise but you should check this with your doctor. If you are planning a pregnancy, you should discuss this with your doctor first and let them know immediately if you become pregnant. All patients will heart valve disease should visit their dentist on a regular basis to ensure good dental hygiene.

+ Symptoms

If you experience any new symptoms in between clinic appointments then it is important that let your Healthcare Professional know.

IMPORTANT SYMPTOMS TO BE AWARE OF:

• Increasing shortness of breath
• Severe or increasing ankle swelling
• Chest pain or tightness
• Blackouts/lightheadedness (especially on exertion)
• Rapid or irregular heartbeat (palpitations)
• Difficulty exercising (not being able to do as much for as long)