Pulmonary valve stenosis

Description

Pulmonary valve stenosis is a narrowing of the valve that is located between the lower right chamber of the heart and the pulmonary arteries. In a narrowing of the heart valve, the valve flaps can be thick or hard. This reduces the flow of blood through the valve.

Normally, the pulmonary valve disease is caused by a problem in the heart that develops before birth. A problem of the heart that are present at birth is called a congenital defect of the heart. In adults, pulmonary valve stenosis can be a complication of another disease.

Pulmonary valve stenosis ranges from mild to severe. Some people with mild pulmonary valve stenosis do not have symptoms. They may need only occasional examinations of health. Moderate and severe stenosis of the pulmonary valve may need a procedure to repair or replace the valve.

Symptoms

Pulmonary valve stenosis symptoms depend on the amount of blood flow is blocked. Some people with mild pulmonary stenosis do not have symptoms. Those with more-severe pulmonary stenosis may begin to notice symptoms during exercise.

Pulmonary valve stenosis, symptoms may include:

  • A whooshing sound called a heart murmur that can be heard with a stethoscope.
  • Fatigue.
  • Shortness of breath, especially during the activity.
  • Pain in the chest.
  • Fainting.

Infants with pulmonary valve stenosis can have the blue or the grey of the skin due to low oxygen levels.

When to see a doctor

Talk with your health care provider if you or your child has:

  • Shortness of breath.
  • Pain in the chest.
  • Fainting.

The rapid diagnosis and treatment of pulmonary valve stenosis may help reduce the risk of complications.

Causes

Pulmonary valve stenosis is usually the result of a problem of the heart present at birth. The exact cause is not clear. The pulmonary valve does not develop properly as the baby is growing in the womb.

The pulmonary valve is composed of three thin pieces of tissue called caps, also called cuspids. The leaflets open and close with each heartbeat. They make sure that the blood is moving in the right direction.

In the stenosis of the pulmonary valve, one or more of the cusps may be stiff or thick. Sometimes the cusps can join. That means they are fused together. So that the valve does not open fully. The smaller opening makes it more difficult for blood to flow out of the lower right chamber of the heart. Increases the pressure within the chamber. The increase of the pressure strains the heart. Finally, the lower right part of the heart chamber wall becomes thicker.

Risk factors

Things that may increase the risk of pulmonary valve stenosis include:

  • German measles, also called rubeola. Having rubella during pregnancy increases the risk of pulmonary valve stenosis in the baby.
  • The Noonan syndrome. This condition is caused by the alteration of DNA. This can lead to many problems with the heart, structure and function.
  • Rheumatic fever. This complication of strep throat can cause permanent damage to the heart and heart valves. Increases the risk of developing pulmonary valve stenosis later in life.
  • The carcinoid syndrome. This condition occurs when a rare cancerous tumor the release of certain chemicals in the bloodstream. That causes difficulty breathing, redness of the skin, and other symptoms. Some people with this syndrome develop carcinoid heart disease, which damages the valves of the heart.

Complications

The possible complications of the pulmonary stenosis include:

  • The infection of the lining of the heart, called infective endocarditis. People with heart valve problems, such as pulmonary stenosis, have an increased risk of developing bacterial infections that affect the inner lining of the heart.
  • Irregular heartbeats, called arrhythmias. People with pulmonary stenosis are more likely to have an irregular heartbeat. Unless the stenosis is severe, irregular heartbeat due to pulmonary stenosis is usually not life-threatening.
  • Thickening of the heart muscle. In the severe pulmonary stenosis, the lower right chamber of the heart has to pump harder to push blood into the pulmonary artery. The strain on the heart causes the muscular wall of the ventricle to thicken. The condition is called right ventricular hypertrophy.
  • Heart failure. If the right ventricle is not pumping properly, heart failure eventually develops. The symptoms of heart failure include fatigue, shortness of breath, swelling of the legs and the belly area.
  • Complications in pregnancy. The risks of complications during labor and delivery are higher for people with severe stenosis of the pulmonary valve that for those who do not.

Pulmonary valve stenosis

Diagnosis

Pulmonary valve stenosis is often diagnosed in childhood. But it may not be detected until later in life.

A doctor uses a stethoscope to listen to the heart. A whooshing sound called a heart murmur can be heard. The sound is caused by halting the flow of blood through the narrowed valve.

Tests to diagnose pulmonary valve stenosis include:

  • Electrocardiogram (ECG or EKG). This quick and painless test records the electrical signals in the heart. Sticky patches, called electrodes, are placed on the chest and sometimes the arms and legs. The wires connect the electrodes to a computer, which displays the results of the test. An electrocardiogram (ECG) may show how the heart is beating and it can reveal signs of thickening of the heart muscle.
  • The echocardiogram. An echocardiogram uses sound waves to produce images of the heart. This test shows how the heart beats and pumps blood. An echocardiogram can show the shape of the pulmonary valve. The test can show how much of the valve will close.
  • The cardiac catheterization.A thin tube, called a catheter is inserted in the groin and threaded through the blood vessels of the heart. Contrast dye flows through the catheter into the blood vessels to show up more clearly on x-rays. This part of the test is called a coronary angiography. During the test, the pressure within the heart can be measured to see how forcefully pumped the blood through the heart. A provider may determine the severity of the pulmonary stenosis by checking the pressure difference between the right lower chamber of the heart and the pulmonary artery.
  • Other imaging tests. Magnetic resonance imaging (MRI) and computed tomography (CT) scans are sometimes used to confirm the diagnosis of stenosis of the pulmonary valve.

The cardiac catheterization. A thin tube, called a catheter is inserted in the groin and threaded through the blood vessels of the heart. Contrast dye flows through the catheter into the blood vessels to show up more clearly on x-rays. This part of the test is called a coronary angiography.

During the test, the pressure within the heart can be measured to see how forcefully pumped the blood through the heart. A provider may determine the severity of the pulmonary stenosis by checking the pressure difference between the right lower chamber of the heart and the pulmonary artery.

Treatment

If you have mild pulmonary valve stenosis without symptoms, you may only need once in a while the health screenings.

If you have moderate to severe pulmonary valve stenosis, you may need a heart surgery or heart surgery. The type of procedure or surgery depends on the general health and appearance of the pulmonary valve.

Surgeries or other procedures

Pulmonary valve stenosis, treatment may include:

  • The balloon valvuloplasty.The doctor inserts a flexible tube with a balloon on the tip into an artery, usually in the groin area. X-rays help to guide the tube, called a catheter, to the narrowing of the valve in the heart. The balloon is inflated, causing the opening of the larger valve. The balloon is deflated. The catheter and balloon are removed. Valvuloplasty may improve the flow of blood through the heart and reduce the symptoms of stenosis of the pulmonary valve. But can the valve to close again. Some people need valve repair or replacement in the future.
  • The replacement of the pulmonary valve.If balloon valvuloplasty is not an option, open-heart surgery or a catheter procedure can be done to replace the pulmonary valve. If there are other problems with the heart, the surgeon can repair those during the same surgery. People who have had replacement of the pulmonary valve may need to take antibiotics before certain dental procedures or surgery to prevent endocarditis.

The balloon valvuloplasty. The doctor inserts a flexible tube with a balloon on the tip into an artery, usually in the groin area. X-rays help to guide the tube, called a catheter, to the narrowing of the valve in the heart. The balloon is inflated, causing the opening of the larger valve. The balloon is deflated. The catheter and balloon are removed.

Valvuloplasty may improve the flow of blood through the heart and reduce the symptoms of stenosis of the pulmonary valve. But can the valve to close again. Some people need valve repair or replacement in the future.

The replacement of the pulmonary valve. If balloon valvuloplasty is not an option, open-heart surgery or a catheter procedure can be done to replace the pulmonary valve. If there are other problems with the heart, the surgeon can repair those during the same surgery.

People who have had replacement of the pulmonary valve may need to take antibiotics before certain dental procedures or surgery to prevent endocarditis.

Self-care

If you have valve disease, it is important to take steps to keep your heart healthy. Certain lifestyle changes can reduce your risk of developing other types of heart disease or a heart attack.

Changes of lifestyle to speak with their health care provider include:

  • Quit smoking.
  • Eating a heart-healthy diet that includes fruits and vegetables, low-fat dairy products, whole grains, and lean meats.
  • Maintain a healthy weight.
  • Exercising on a regular basis.

Preparing for your appointment

If you or your child has the disease of the valve, you will likely be referred to a physician trained in the evaluation and treatment of diseases of the heart. This type of provider, it is called a cardiologist.

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

What you can do

  • Write down any symptoms you or your child, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including major stresses or recent illnesses.
  • List of all the medicines, vitamins, and supplements that you or your child need.
  • Write questions for the health care professional.

Prepare a list of questions can help you make the most of your time with your health care provider. For pulmonary valve stenosis, some basic questions include:

  • What is the most likely cause of my symptoms?
  • There are other possible causes?
  • What tests are needed? Do these tests require any special preparation?
  • Is stenosis of the pulmonary valve temporary or long-term?
  • What treatments are available? Which do you recommend and why?
  • What are the risks of a balloon valvuloplasty or open heart surgery?
  • Are there any activity restrictions?
  • Are there brochures or other printed material that I can take? What sites do you recommend?

Do not hesitate to ask other questions.

What to expect from your doctor

Your health care provider is likely to ask many questions, including:

  • When did symptoms begin?
  • The symptoms come and go, or not always does this happen?
  • The symptoms are worse during activity or when lying down?
  • Nothing seems to improve the symptoms?
Symptoms and treatment of Pulmonary valve stenosis