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Your heart is made up of four chambers (left atrium, right atrium, left ventricle, right ventricle) and four one-way valves to guide the flow of blood through the heart and prevent blood from moving backward. Valvular heart disease (VHD) occurs when you have damage to one of the four heart valves:
- Tricuspid valve: maintains the flow from the right atrium into the right ventricle
- Pulmonary valve: maintains the flow from the right ventricle into the pulmonary artery (the artery that takes blood to the lungs to get reoxygenated)
- Mitral valve: maintains the flow from the left atrium into the left ventricle
- Aortic valve: maintains the flow from the left ventricle into the aorta (largest artery leaving the heart)
The aortic and mitral valves are the ones most often affected by valvular heart disease.
There are three main ways that your heart valves can malfunction:
- Regurgitation (or insufficiency): This occurs when the valve does not close properly, and blood leaks backward in the heart. Sometimes this is due to the flaps of the valve (also called cusps or leaflets) bulging backward, allowing blood to leak around it; this condition is called valve “prolapse” and most often affects the mitral valve. Regurgitation can affect any of the four valves; these are referred to as tricuspid regurgitation, pulmonary regurgitation, mitral regurgitation, and aortic regurgitation.
- Stenosis: In stenosis, the valve leaflets are either fused or too stiff to open entirely, and the overall opening of the valve is narrower than normal; this makes it more difficult for the blood to get from one chamber to the next, leading to decreased blood flow. All four valves can develop stenosis; these are referred to as tricuspid stenosis, pulmonic stenosis, mitral stenosis, or aortic stenosis.
- Atresia: This condition is much less common than the others and is due to a poorly developed heart valve that does not have an opening for blood to flow through; this is a type of congenital heart defect present at birth.
How do heart valves work?
Several things happen during the span of a single heartbeat. First, blood returning from the body fills the left and right atria. The mitral and tricuspid valves that sit at the bottom of the atria open to allow blood to flow into the corresponding ventricles. As the ventricles start to contract, the mitral and tricuspid valves close to prevent blood from flowing back into the atria. When the ventricles contract, they pump blood through the aortic and pulmonic valves into the aorta and pulmonary artery, respectively. As the ventricles relax, the aortic and pulmonic valves shut to keep blood from flowing back into the ventricles; then the whole process repeats with the next heartbeat. The “lub-dub” sound you hear when listening to someone’s heartbeat is the sound of the valves closing. The “lub” is the sound of the mitral and tricuspid valves closing, and the “dub” is the sound of the pulmonic and aortic valves closing. For this reason, providers can sometimes detect valvular heart disease from changes in the heart sounds; these abnormal heart sounds are called heart murmurs.
What causes valvular heart disease?
Congenital heart valve disease is a condition where your heart valves do not develop properly before birth. In these children, one or more valves may be an abnormal size, may not open or close properly, or may have abnormally formed leaflets; pulmonary valve atresia and bicuspid aortic valves are examples of congenital heart valve disease.
A bicuspid aortic valve is one of the most common congenital heart malformations and occurs in 1–2% of people (Shah, 2018; Longobardo, 2016) . Bicuspid, means “two cusps” or leaflets; the aortic valve usually has three leaflets that come together to close the valve, but people with a bicuspid aortic valve only have two leaflets. Without that third leaflet, the aortic valve can be leaky (aortic regurgitation), or if the leaflets fuse, it can have difficulty opening (aortic stenosis). Interestingly, some people have no symptoms from this condition initially; over time, they can develop problems with their heart–like heart failure.
In contrast to congenital valve disease, acquired valvular heart disease (VHD) includes abnormalities that develop on heart valves that were previously normal. Several conditions can cause acquired VHD, including infections like rheumatic fever and endocarditis.
- Rheumatic fever occurs after an untreated streptococcal infection, like strep throat. Since the widespread use of antibiotics in the latter half of the 20th century, the incidence of rheumatic fever has decreased significantly in the United States, down to 0.04–0.06 cases per 1,000 children; it occurs mainly in children aged 5–15 (CDC, 2015). Rheumatic fever can lead to rheumatic heart disease. In the process of trying to fight off the streptococcal infection, the immune system ends up also attacking the heart valves, leading to inflammation and eventual scarring of the heart valve. This results in the valves (usually the aortic or mitral valves) becoming either stenotic or leaky.
- Endocarditis is another infectious cause of acquired valvular heart disease. Sometimes bacteria enter the bloodstream, especially after dental procedures, surgery, intravenous drug use, or severe illness. If the body cannot fight them off, they can travel in the blood to infect the heart, causing endocarditis. Infective endocarditis is a potentially life-threatening condition where the valves and the inner lining of the heart (the endocardium) get infected; as the infection attacks the valves, it can make the valves function abnormally. If you already have a valve problem, you are at an increased risk of getting endocarditis and may have to take antibiotics before having surgery or dental procedures.
- Other causes of acquired valvular heart disease include coronary artery disease, high blood pressure, heart attacks, strokes, and connective tissue diseases, like Marfan syndrome.
Mitral valve prolapse (MVP) is a type of acquired valvular heart disease that also has a genetic component; approximately 2–3% of people in the U.S. have this condition (Delling, 2014). It is rarely seen in children or adults younger than 30 years of age, but it does seem to run in families (Delling, 2014). In mitral valve prolapse, the valve flaps are floppy and bulge back into the atrium rather than forming a tight seal. If you have MVP, you are at increased risk of getting infective endocarditis. Some people with MVP need to take antibiotics before procedures; talk to your provider about whether this applies to you.
According to the American Heart Association (AHA), risk factors for valvular heart disease include (AHA-b, 2016):
- Older age: over time your heart valves thicken, become stiffer, and may not function properly.
- History of infective endocarditis or rheumatic fever.
- History of heart attack, heart failure, or previous heart valve disease (either congenital or acquired.)
- Coronary artery disease.
- Risk factors for heart disease, like high blood pressure, high cholesterol, smoking, diabetes, obesity, or family history.
Signs and symptoms of valvular heart disease
The main physical sign of valvular heart disease is an abnormal sounding heartbeat, called a heart murmur; your healthcare provider can hear it with a stethoscope during your exam. However, the presence of a heart murmur may or may not be due to heart valve disease; also, even if the murmur is due to valvular disease, you may not have any other issues or symptoms. Many people with heart valve disease have no symptoms, especially earlier in the course of the condition, or they may develop so slowly as not to be noticeable. A valve can be malfunctioning significantly with minimal symptoms; you can also have mild valvular disease with severe symptoms.
Signs and symptoms that people with valvular heart disease may experience include:
- Shortness of breath or difficulty catching your breath, especially when lying down or exerting yourself (like walking)
- Chest pain
- Feeling like your heart is fluttering, racing, or skipping beats
- Swollen feet, legs, or abdomen
- Lightheadedness or episodes of fainting
How to diagnose valvular heart disease
The diagnosis of valvular heart disease often begins with your primary healthcare provider. A physical examination is the first step in diagnosing VHD. Your provider will listen to your heart (to check for a heart murmur), listen to your lungs (to see if there is any fluid buildup), and check your feet for any swelling. You may then be referred to a cardiology specialist (a provider who specializes in diseases of the heart) for additional testing.
To help in diagnosing valvular heart disease, your cardiology provider may have you undergo a special test called an echocardiogram (sometimes referred to as an “echo”); this is the standard tool for evaluating heart valve function. Echocardiograms use sound waves to create a picture of your heart and visualize how the blood is flowing through the different chambers. It can give you information about the size of your heart, how well it is pumping blood, and if any of the valves are narrow or leaky. There are two different types of studies: transthoracic echocardiogram (TTE) and transesophageal echocardiogram (TEE). In a TTE, the instrument that makes the sound waves (transducer) is placed on your chest. Whereas in a TEE, the transducer is attached to a flexible tube and is guided down your esophagus (throat), while you are given medicine to help you relax; the TEE produces better pictures because it is closer to the heart and the sound waves don’t have to travel as far.
Depending on the results of the echocardiogram, some people may need additional testing after the echocardiogram. Further testing may include:
- Cardiac catheterization (also called angiography): This is a minimally invasive procedure where a thin tube is inserted into a blood vessel in your groin or arm and guided into your heart. Once inside, it can measure the blood pressure inside the heart, evaluate the function of the heart muscles, and check the movement of blood through the valves. Cardiac catheterization can also give you information about whether your problems are due to coronary artery disease.
- Chest X-ray: A picture of your chest is taken using X-rays to look at the size of your heart and whether there is a buildup of fluid in your lungs.
- Electrocardiogram (ECG): This test records the electrical activity of the heart and looks for abnormal heartbeats or signs of prior heart attacks.
- Cardiac MRI: A cardiac MRI uses magnetic waves to create pictures of your heart and its valves.
- Exercise stress test: You are asked to exercise (or given medicine to make your heart beat faster to simulate exercise), and pictures are taken of your heart to see how your heart functions under stress. It can provide information as to the severity of your heart valve disease.
Treatments for valvular heart disease
There are no medications to stop a leaky valve or open up a narrow one; however, there are treatments that can improve symptoms and potentially delay worsening. Some people may eventually need surgery to repair or replace their abnormal valves. The mainstays of therapy for valvular heart disease are heart-healthy lifestyle changes in addition to medications and/or surgery.
The goal of heart-healthy lifestyle changes is to treat heart conditions that can worsen your valvular heart disease. These changes include:
- Heart-healthy eating with a diet low in sodium and saturated fat and rich in fruits and vegetables
- Maintaining a healthy weight; obesity is a risk factor for not only valvular heart disease, but other forms of heart disease as well
- Minimizing stress
- Exercising several times a week
- Quit smoking
While these are excellent strategies to incorporate into your lifestyle to promote a healthy heart, many people with valvular heart disease find that this is not enough, and they need to take medication. Here are the types of medicines that your provider may prescribe for you and the reasons why (AHA-a, 2016):
Despite lifestyle changes and medications, some people’s valvular heart disease worsens to the point that they need to have surgery to repair or replace the faulty heart valve. The decision whether to repair or replace a heart valve depends on the severity of your disease, whether you need heart surgery for other reasons, and your overall health. It is preferable to repair a heart valve rather than replace it, whenever possible. Unfortunately, heart valve repair is more challenging, and not all of the valves can be repaired.
Heart valve repair can be accomplished in several different ways. Some options are to add tissue to patch up tears, remove tissue to allow the valve to close tightly, or separate leaflets that are scarred together. One type of valve repair that is helpful for valves that are stenosed (too tight) is balloon valvuloplasty. A thin tube with a balloon on the tip is guided into your heart and inflated inside the tight valve to widen the opening of the valve. This procedure seems to work best for mitral valve stenosis and not as well for adults with aortic valve stenosis.
If your valve cannot be repaired, then it needs to be replaced. Replacing a heart valve involves removing the dysfunctional valve and replacing it with either a mechanical valve or a biological one; biological valves come from a pig, cow, or human heart tissue. Sometimes an aortic valve can be replaced by inserting a new valve (via a catheter) inside the old valve; the new valve pushes the old leaflets out of the way, and the new valve takes over. This procedure is called transcatheter aortic valve replacement (TAVR).
How to prevent valvular heart disease
The best way to prevent valvular heart disease is to address the risk factors for this condition. Most of the risk factors are related to overall heart health, like high blood pressure and high cholesterol, so doing things to keep your heart healthy may also prevent heart valve disease. Things you can do to improve your heart health include:
- Low salt, low saturated fat diet that is high in fruits and vegetables.
- Exercising several times a week.
- Treat existing heart disease (like high blood pressure, high cholesterol, or heart failure) as instructed by your provider
- Quit smoking.
Valvular heart disease is a lifelong condition; however, many people live long and full lives with their valve disease. You should see your provider for regular checkups and maintain open lines of communication. Tell your healthcare provider right away if you experience any of the symptoms of valvular heart disease or if they are worsening.
- American Heart Association (AHA-a). (2016, May 31). Medications for Heart Valve Symptoms. Retrieved Nov. 22, 2019 from https://www.heart.org/en/health-topics/heart-valve-problems-and-disease/understanding-your-heart-valve-treatment-options/medications-for-heart-valve-symptoms
- American Heart Association (AHA-b). (2016, May 31). Risks for Heart Valve Problems. Retrieved Nov. 22, 2019 from https://www.heart.org/en/health-topics/heart-valve-problems-and-disease/heart-valve-disease-risks-signs-and-symptoms/risks-for-heart-valve-problems
- Centers for Disease Control and Prevention (CDC). (2015). Morbidity and Mortality Weekly Report (MMWR): Acute Rheumatic Fever and Rheumatic Heart Disease Among Children – American Samoa, 2011–2012. Retrieved on Nov. 22, 2019 from https://www.cdc.gov/mmwr/preview/mmwrhtml/mm6420a5.htm
- Longobardo, L., Jain, R., Carerj, S., Zito, C., & Khandheria, B. K. (2016). Bicuspid Aortic Valve: Unlocking the Morphogenetic Puzzle. The American Journal of Medicine, 129(8), 796–805. doi: 10.1016/j.amjmed.2016.03.009. Retrieved from https://europepmc.org/article/med/27059385
- Shah, S. Y., Higgens, A., & Desai, M. Y. (2018). Bicuspid aortic valve: Basics and beyond. Cleveland Clinic Journal of Medicine, 85(10), 779–784. Retrieved from https://www.mdedge.com/ccjm/article/175729/cardiology/bicuspid-aortic-valve-basics-and-beyond
Dr. Mike is a licensed physician and the Director, Medical Content & Education at Ro.