Mitral Valve Defects

Mitral Valve Defects

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What are mitral stenosis, mitral regurgitation and mitral valve prolapse?

Mitral stenosis, mitral regurgitation and mitral valve prolapse are all mitral valve diseases caused by defects of the heart's mitral valve.

The heart is a two-sided pump with four chambers. Four valves control the flow of blood through the heart. The mitral valve sits between the left atrium (upper chamber) and the left ventricle (lower chamber) and is the first valve on the left side of the heart.

A normal mitral valve is made up of two thin leaflets. This valve opens to allow blood to flow from the upper chamber to lower chamber on the left side the heart, then closes to keep blood from leaking back into the upper chamber.

Types of mitral valve defects

  • Mitral stenosis: The leaflets are fused, are too thick, or there is some other structural defect in the valve leaflets. Sometimes, mitral stenosis occurs as a result of surgery to repair an abnormal mitral valve. As a result, the valve is too narrow, and the heart has to work harder to pump blood through the valve.
  • Mitral regurgitation: The valve doesn’t close completely and allows blood to leak back (regurgitate) into the atrium (upper chamber) from the ventricle (lower chamber). This is also referred to as mitral valve insufficiency.
  • Mitral valve prolapse (MVP): When the valve is closed, the leaflets bulge abnormally up, into the atrium. Sometimes mitral valve prolapse causes mitral valve regurgitation. If the prolapse is severe, the valve leaflets can become disconnected from tendons that stretch between the leaflets and the heart muscle. This can cause the valve to malfunction.

Causes of mitral valve defects

Sometimes a baby is born with a defective mitral valve. In other cases, certain heart or health problems can lead to a weakened mitral valve during childhood or adulthood. Rheumatic fever, an acquired disease, can also cause scarring in the heart tissue resulting in mitral stenosis or regurgitation.

Symptoms and signs of mitral valve defects

Symptoms vary widely depending on the type and severity of the condition.

A newborn with severe or critical mitral stenosis may require treatment immediately. Symptoms may include:

  • Blue or purple tint to lips, skin and nails (cyanosis)
  • Difficulty breathing

In less severe cases, symptoms may include:

  • Palpitations or irregular heartbeat (arrhythmia)
  • Fatigue
  • Difficulty breathing during exercise
  • Recurrent cough and lung infections

Mitral valve regurgitation can also cause these symptoms. Sometimes symptoms don’t appear until the child is older or an adult.

When mitral stenosis or mitral regurgitation is caused by rheumatic fever, symptoms may not appear until many years after the child has the disease.

Mitral valve prolapse often has no symptoms. In some cases, mitral valve prolapse causes arrhythmias (abnormal heart rhythm).

Testing and diagnosis of mitral valve defects

Your pediatrician may hear a heart murmur (an extra heart sound) and refer your child to the Cardiac Center.

Echocardiography (sound waves create an image of the heart) will then be used to make a diagnosis. Diagnosis may also include:

  • Chest X-ray
  • Electrocardiogram (ECG): a record of the electrical activity of the heart
  • Cardiac catheterization: a thin tube is inserted into the heart through a vein and/or artery in either the leg, the neck, or through the umbilicus (“belly button” — used in newborns only).
  • Cardiac MRI

Treatment for mitral valve defects

Treatments vary widely depending on the type and severity of your child's condition. In many cases, no treatment is necessary. In other cases, your child may need to take medications.

In severe cases, treatment options include:

Balloon mitral valvuloplasty

During this cardiac catheterization procedure, an interventional cardiologist will insert a thin tube (catheter) into a vein in the leg, and then guide the tube to your child’s heart. A special technique is then used to guide the catheter across the atrial septum (the thin wall separating the two upper chambers of the heart) and into the left atrium, which allows direct access to the mitral valve. The catheter is then advanced across the mitral valve. The catheter will have a balloon on the end of it. The balloon will be briefly inflated to open up the narrow valve, then deflated and withdrawn. Sometimes, two catheters and balloons are used. After this procedure, older children will spend one night in CHOP’s dedicated post-catheterization recovery unit before returning home. They will also need to rest for the next few days, but then can resume normal activity. Newborns with critical conditions or children who are already inpatients at CHOP may stay in the hospital slightly longer, either in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU), where they will receive around-the-clock attention from a team of dedicated cardiac critical care medicine specialists, or in the Cardiac Care Unit (CCU).

Surgery to repair or replace the valve

Surgeons will separate the valve’s fused leaflets or attempt to repair the valve’s defect so that it can work properly. If the valve cannot be repaired, they will replace it with a mechanical (man-made) valve or a valve from a donated organ. In cases where a mechanical valve is used, your child will need to take a blood thinner for the rest of their life to prevent clots from forming on the valve.

  • Mechanical Valve Repair in Children and Teens

    Jonathan Chen, MD: A mechanical valve can be used to replace a damaged or diseased aortic or mitral valve. I'm Jonathan Chen, a surgeon in the Pediatric Heart Valve Center at Children's Hospital of Philadelphia. My team is one of the most experienced in the country in valve repair and replacement. To better understand mechanical valve replacement, let's first take a look at the anatomy of the heart. This is your heart. It has four chambers and four valves.

    The upper chambers are the left atrium and the right atrium. The lower chambers are the left ventricle and the right ventricle. Before entering or leaving each chamber of the heart, blood passes through a valve. The mitral valve is between the chambers on the left side of the heart. The left atrium receives oxygen-rich blood from the lungs and pumps it through the mitral valve to the left ventricle.

    The left ventricle then pumps the blood through the aortic valve and into the aorta, the largest artery in the body. Blood travels from the aorta to blood vessels throughout the body, delivering oxygen and nutrients. Heart valves are made of flaps of tissue called leaflets. The leaflets work like one way doors, opening to let blood move forward, and closing to keep blood from flowing backward. Watch how the leaflets move from another angle, opening to let blood move forward and closing to keep blood from flowing backward.

    The leaflets are attached to a strong tissue called the annulus. The annulus helps maintain the shape of the valve. If a valve is diseased or damaged, it can be surgically removed and replaced with a mechanical valve. A mechanical valve is made of metal and plastic. A thick cloth surrounds the valve. Just like your child's heart valves, the leaflets in the mechanical valve open and close to keep blood flowing through your heart. The mechanical leaflets make a soft clicking noise as they open and close. You can hear it, if you have a mechanical valve. Patients adjust to the sound and are eventually able to ignore it. You might have heard of adults who have bioprosthetic valves, made of tissue from pigs or cows.

    These valves have a high risk of becoming hard over time, in a process called calcification, causing these valves to last less than three to five years in children. During a mechanical valve replacement, your surgeon will remove the diseased or damaged valve and choose the right size replacement valve for your child.

    The surgeon will suture the mechanical valve to the annulus using the sewing ring or cloth rim of the valve. At the Valve Center at Children's Hospital of Philadelphia, our team uses cutting edge imaging to view each patient's heart before surgery. This allows us to develop a highly personalized surgical plan based on your child's unique anatomy.

    In babies and toddlers, a mechanical valve will need to be upsized as your child grows. While a teenager may only need one valve operation, a baby or toddler will likely need two or three re-operations over time. A mechanical valve has an increased risk of infection. It's important to tell your doctor if your child has a serious cut or lesion because they may need antibiotics to prevent infection from traveling to the heart.

    It is possible for scar tissue, called pannus, to grow around the mechanical valve. Too much scar tissue can make it hard for the leaflets to move, requiring a replacement valve. Patients with mechanical valves must take daily blood thinners to prevent clotting on the valve. If a blood clot detaches from the valve and travels to another area of the body, this can be life-threatening. Blood thinning medication needs careful management.

    Dosage must be monitored regularly, which can be done at home. When your child is facing heart valve surgery, we know there are difficult decisions to make. Our experts work together and with you, to make the best decisions for your child before, during, and after surgery.
Transcript Transcript

Outlook for mitral valve defects

Because of enormous strides in medicine and technology, today most children with heart conditions such as these go on to lead productive lives as adults.

Follow-up care

Through 18

If your child was born with mitral stenosis, they will require follow-up care. They will need to visit a pediatric cardiologist for evaluation and testing to ensure there is no worsening of the obstruction at the valve. Children who have had a mechanical valve surgically placed will also need routine monitoring to make sure their blood thinner is creating the right amount of thinning effect.

Depending on the severity of the condition and symptoms, children with mitral regurgitation or mitral valve prolapse may or may not require follow-up visits with a pediatric cardiologist. We will discuss follow-up care with you based on your child’s unique needs.

Into adulthood

For children who will require ongoing care from a cardiologist throughout their lives, CHOP will help transition care from a pediatric cardiologist to an adult congenital heart disease specialist.

The Philadelphia Adult Congenital Heart Center, a joint program of CHOP and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.

Why Choose Us

Our specialists are leading the way in the diagnosis, treatment, and research of congenital and acquired heart conditions.

Related specialties and programs

Cardiac Center

Philadelphia Adult Congenital Heart Center

Cardiac Catheterization Laboratory

Resources to help

Cardiac Center Resources

We know that caring for a child with a heart condition can be stressful. To help you find answers to your questions – either before or after visiting the Cardiac Center – we’ve created this list of educational health resources.
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