Pulmonary valve stenosis can be both a birth defect or something adults develop later on in life. It can range in severity from mild to severe cases. Often, a person may not know they have the condition until years down the road.
In a substantial amount of cases, the patient is able to go through life without any major issues. In moderate cases, some medical procedures may be required. It is common to see the doctor every few months in order to monitor the success of the procedures.
In some severe cases, surgery is required, and physical activity is restricted until the patient shows signs of improvement.
Causes of Pulmonary Valve Stenosis
Pulmonary valve stenosis (PVS) is a condition that affects the heart. The heart has the important job of maintaining proper blood flow throughout our bodies. It needs to remain strong and functioning for us to maintain good health.
The heart is a muscle that contains four chambers:
- The left atrium (top left)
- The right atrium (top right)
- The left ventricle (bottom left)
- The right ventricle (bottom right)
After its journey through the body, blood is pumped back into the heart. From there, it enters the right side. It goes from the right atrium into the right ventricle, then through the pulmonary valve. Then, it receives fresh oxygen from the lungs and continues through the body once more.
Picture the pulmonary valve as a one-way door. It has small flaps that allow blood to flow through it one way, with no way of getting back.
In people with pulmonary valve stenosis, the blood has a hard time making its way through the pulmonary valve. This is usually because the valve is too narrow.
The heart has its own way of dealing with this issue. Because it has to work extra hard to force blood through that small opening, it does what any other muscle might do when it gets overworked: become larger and thicker. Unfortunately, this can put a strain on heart function.
PVS isn’t a disease; it’s a defect that usually happens during the heart’s development. Doctors don't know the exact cause of PVS, but it isn't caused by anything a mother did or didn't do during her pregnancy.
PVS can be present on its own or alongside other heart defects. Some known causes of PVS include:
- Rubella infection of the mother during pregnancy can cause PVS in the baby
- William’s syndrome, a genetic disorder that causes a child developmental delays, can also cause PVS
Who is at risk of developing this condition? Most people who have it are born with it. Babies’ heart valves form during the first 8 weeks of pregnancy, so it’s possible that this condition could be passed genetically from parent to child.
If a mother gets rubella, also known as German measles, during pregnancy, there is a higher chance the baby will develop pulmonary valve stenosis.
The illness can also occur if a baby has Noonan syndrome. Noonan syndrome is a genetic condition that is typically passed down from parent to child. It can cause short stature, unusual facial characteristics, and heart development issues.
In adults, rheumatic fever and carcinoid syndrome can put you at risk of getting pulmonary stenosis. Both these diseases can cause damage to your heart valves.
Williams syndrome is a rare disorder few babies are born with. It’s characterized by certain facial features that get more pronounced with age and slow mental development. People born with Williams syndrome, also called Williams-Beuren syndrome, are at risk to develop PVS among other heart defects.
Symptoms of Pulmonary Valve Stenosis
In some mild cases of valve stenosis, you might not notice symptoms at all. You may go for a routine check-up, and your doctor might find it when they listen to your heart with a stethoscope.
Symptoms of severe pulmonary valve stenosis in babies may also manifest, though, in an obvious way: They may show some blue coloration (cyanosis).
Some common symptoms of moderate pulmonary valve stenosis can include:
- Feeling tired or very short of breath after a short amount of exercise
- Fast breathing and heart rate
- A bluish color around the lips and fingertips
- Chest pain
- Swelling in the hands and feet, legs, belly, or face
- General fatigue
Sometimes, symptoms only present during exercise.
Diagnosing Pulmonary Valve Stenosis
Pulmonary valve stenosis can sometimes be diagnosed before birth. A fetal echocardiogram, (also known as a fetal echo) can show pictures of what a fetus’ heart looks like while they’re still growing inside the mother.
If you suspect that you or your child has pulmonary stenosis, you might visit the doctor, and they will decide which tests to perform. Some of those tests can include:
- X-ray. Chest X-rays can show changes to or structural problems within your heart.
- Echocardiogram. Similar to an imaging test performed on an unborn baby, this test uses sound waves to create pictures of your heart. It is one of the most accurate diagnostic tools.
- Electrocardiogram (ECG). This test tracks the rhythms of the heart. It can detect areas of stress in the heart and record electrical activity.
- Cardiac catheterization. Doctors are usually able to diagnose pulmonary stenosis with other tests. A cardiac catheter is more invasive and will usually be used as a last option. A tube is inserted in the groin area and pushed up to the heart. They will check each of the four chambers of the heart for circulation issues and problematic blood pressure. A dye is often used to measure how efficiently the heart is pumping blood.
- Magnetic Resonance Imaging (MRI). MRI tests can sometimes be ordered depending on what equipment the doctor has available.
What to Expect at a Doctor’s Appointment
Before your doctor’s visit, write down any symptoms you or your child have experienced. Think of your family’s health history and make note of any concerns. Also, write down any questions you have for your doctor.
If you are taking any medications or supplements, make a list and have it readily available.
If you are seeing your general practitioner or family doctor, they will likely perform a general check-up and possibly order an imaging test. Depending on the results of the test, you could be referred to a heart specialist.
If you see a specialist, you might consider gathering some information about how this condition can affect everyday life. For instance, you may ask:
- Can you or your child participate in sporting activities or exercise? If so, which ones are safe?
- What could be a possible cause of this condition?
- Is there a certain heart rate number you should aim to stay under? Is a heart rate or blood pressure monitor necessary for you?
- What are the treatment options, and what risks and benefits does each option carry?
- Are there any additional educational resources such as websites or reading material that could be beneficial?
The doctor will have some questions for you too. They could be related to how you or your child are experiencing the symptoms in terms of time duration or what they are doing when the symptoms appear.
How Pulmonary Valve Stenosis Is Treated
In mild cases, no treatment is needed other than regular follow-ups with your doctor. If the symptoms are moderate-to-severe and are interfering with your quality of life, you might consider the following treatment options:
- Balloon valvuloplasty. An un-inflated balloon is placed on the end of a catheter and brought into the small heart valve using imaging tests as a guide. The balloon is then inflated, stretching the pulmonary valve open. This is one of the most common treatments. It’s possible that the valve can return to its original size after the treatment.
- Pulmonary valve replacement. The heart valve can be replaced through undergoing surgery. An artificial valve or donor valve is used. This procedure is performed on a case-by-case basis and depends on other factors the patient might be going through.
- Valvotomy. If there is scar tissue on the pulmonary valve causing less blood to flow through, corrective surgery can be performed to remove the scar tissue.
It’s important to remember that the valve usually will not function as normal, even after surgery. It will need to be monitored regularly, and precautions must be taken during some medical procedures.
Complications of Pulmonary Valve Stenosis
Complications can arise whether you’ve had corrective treatment or not. For example, if you undergo a valve replacement, there are several safeguards you must have in place to prevent inflammation or infection of the valve.
Endocarditis is one such risk. It’s a bacterial infection that settles into the lining of the heart. It can be especially threatening to someone who’s had heart surgery or has a heart condition.
Most people who have had valve replacement surgery will need to take antibiotics before any dental or medical procedures in order to prevent endocarditis.
Other complications of pulmonary valve stenosis can be:
- The heart muscle thickens. Because the heart has to work harder to pump blood, the walls of the heart can get thick. This can cause extra strain on the heart.
- Irregular heartbeat. Also known as arrhythmia, this can happen in people with valve stenosis. In severe cases, your doctor might recommend treatment.
- Heart failure. In severe cases, the heart could fail because it’s working extremely hard to pump blood, and not enough blood is going through it.
- Pregnancy complications. If you have pulmonary valve stenosis, it can interfere with your pregnancy. It depends on the severity of your case and how the health of your heart valve has been maintained.