Pulmonary Valve Stenosis

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What is pulmonary valve stenosis?

Pulmonary valve stenosis—or narrowing—is a rare type of heart valve disease. The heart has four valves that act as one-way gates to keep blood flowing in one direction through the heart and out to the body. The pulmonary valve controls blood flow out of the right ventricle into the pulmonary artery. Blood flows through this valve on the way to the lungs to pick up oxygen, before returning to the left side of the heart. Stenosis of the pulmonary valve occurs when the valve does not open wide enough to allow efficient blood flow. Pulmonic stenosis is another name for this condition.

In most cases, pulmonary valve stenosis is congenital, meaning it is present at birth. The defect develops very early in pregnancy, during the first eight weeks. It is usually a defect of the valve itself. The valve’s cusps, or leaflets, may be too thick to open properly or they may be fused together and unable to open like they should. In either case, the opening through the middle of the valve is too narrow. Sometimes, pulmonary valve stenosis develops as a complication of other diseases, such as rheumatic fever.

The reason this pulmonary valve birth defect develops is unclear. It is possible that genes may play a role because it can run in families. But most cases are the result of chance or a random defect. Other heart defects may also be present.

Doctors may find congenital pulmonary valve stenosis during pregnancy. However, symptoms of a stenotic pulmonary valve usually develop after the baby is born. Pulmonary valve stenosis causes mild to severe symptoms, depending on how much it affects blood flow. Mild symptoms may include feeling tired or getting short of breath during activity. In severe cases, babies can turn blue from lack of oxygenated blood. Doctors may also hear a pulmonary valve stenosis murmur in the heart. It is the result of turbulent (as opposed to smooth) blood flow through the valve.

Pulmonary valve stenosis treatment may or may not be necessary, depending on the symptoms. Mild cases may never worsen or require treatment. However, moderate to severe cases usually need surgery to correct the defect and improve blood flow. This treatment is highly successful and can prevent complications, such as heart failure.

When symptoms are severe, doctors may find pulmonary valve stenosis shortly after birth. Most cases are diagnosed during childhood. In very mild cases, it may not become apparent until adulthood. See your doctor promptly if you notice symptoms like shortness of breath, fatigue, or fast heart rate.

What are the symptoms of pulmonary valve stenosis?

Pulmonary valve stenosis symptoms can range from mild to severe. Mild problems usually do not worsen over time and may never require treatment. Children with moderate to severe cases can get worse with time.

Common symptoms of pulmonary valve stenosis

Common pulmonary valve stenosis symptoms include:

  • Cyanosis, which is a blue discoloration of the lips and fingers due to lack of oxygenated blood
  • Fast heart rate
  • Fatigue

Contact your child’s doctor right away for any of these symptoms. Prompt diagnosis of pulmonary valve stenosis can help prevent complications.

What causes pulmonary valve stenosis?

Pulmonary valve stenosis is usually a birth defect that occurs in the first eight weeks of development. Doctors do not know why the defect happens. In most cases, it is a random occurrence. However, it can run in families, which implies that genes may play a role in these cases. Stenosis of the pulmonary valve can occur alone or along with other heart birth defects.

The defect most often involves the valve itself. It causes the valve’s leaflets to thicken or sometimes fuse together. This prevents the valve from opening all the way, narrowing the pathway for blood to flow. In some cases, the defect involves the areas just before or after the valve.

Sometimes, pulmonary valve stenosis occurs as a complication of another disease that affects the heart.

What are the risk factors for pulmonary valve stenosis?

For the pulmonary valve birth defect, there are no known risk factors. It usually occurs by chance. In a few cases, it may be related to inherited genes.

For acquired pulmonary valve stenosis later in life, diseases that increase the risk of developing it include:

  • Carcinoid syndrome, which results from the growth of carcinoid tumors in the digestive system
  • Noonan syndrome, which is a genetic disorder that can affect the heart
  • Rheumatic fever, which is a complication of a streptococcal infection (strep throat, scarlet fever)

Reducing your risk of pulmonary valve stenosis

Because doctors do not understand why pulmonary valve stenosis occurs in most cases, it is not possible to definitively prevent it. It is not related to anything a mother does or does not do during pregnancy. However, studies have shown that women who consume daily folic acid before and during pregnancy are less likely to deliver children with congenital heart abnormalities.

Regular prenatal care during pregnancy is vital to the health and well-being of both mother and baby. It may be possible to find pulmonary valve stenosis during pregnancy, in some cases. This allows doctors to address the problem immediately after birth.

You can reduce your risk of rheumatic fever by treating streptococcal infections with a prescribed course of antibiotics.

What are the diet and nutrition tips for pulmonary valve stenosis?

People with heart problems need to be conscious about their diet. Eating healthy can help prevent additional heart problems. Encouraging your child to develop healthy eating habits will be a benefit to their future health. Heart-smart foods include:

  • Fresh fruits and vegetables
  • Heart-healthy oils, such as canola oil and olive oil
  • Lean meats, poultry and fish
  • Low-fat dairy products
  • Nuts, legumes and beans
  • Whole grain breads, pastas and cereals

Ask your child’s doctor about dietary goals to ensure your child gets the nutrition necessary for proper growth and development.

How do doctors diagnose pulmonary valve stenosis?

Sometimes, doctors find pulmonary valve stenosis before birth using a fetal echocardiogram. This exam uses sound waves to make moving pictures of the baby’s heart. It can help diagnose heart defects during development.

Other times, doctors do not diagnose the problem until after birth. In severe cases, symptoms may be present at birth that makes doctors suspicious of a potential heart problem. Milder cases may not be apparent until later in childhood or even adulthood. A heart murmur on a physical exam is often a clue. If your child has symptoms, your doctor may ask several questions about them including:

  • When did you first notice symptoms?
  • When do the symptoms occur?
  • Do the symptoms happen all the time or do they come and go?
  • What, if anything, seems to make the symptoms better or worse?

Heart tests

If your doctor suspects pulmonary valve stenosis, additional testing may be necessary including:

  • Imaging exams, including CT (computed tomography) and MRI (magnetic resonance imaging)
  • Cardiac catheterization, which is an invasive procedure that uses a dye and X-rays to visualize the heart. Doctors may recommend this if the diagnosis is unclear or if your child is a candidate for balloon valvuloplasty. This treatment is catheter-based and doctors can perform it in the same procedure.

What are the treatments for pulmonary valve stenosis?

Pulmonary valve stenosis treatment may or may not be necessary. Mild cases that do not interfere with daily living usually do not get worse and may never need treatment. Doctors may only recommend regular follow up to monitor the valve.

When the blockage of blood flow is more severe, it may worsen with time and usually requires treatment. Doctors can measure the pressure on either side of the stenotic valve to help determine how severe the problem is. Surgery can correct pulmonary valve stenosis including:

  • Balloon valvuloplasty, which is a catheter-based procedure. It involves threading a catheter through an incision in the leg or groin to the heart. Once in place, the doctor inflates a balloon to widen the valve opening and then removes the balloon.
  • Open heart surgery, which involves opening the chest to operate on the pulmonary valve. Doctors may repair the valve or replace it, depending on your condition.

Self-care for pulmonary valve stenosis

Anyone with heart problems can benefit from a heart-healthy lifestyle. While it will not treat pulmonary valve stenosis, it can help prevent other heart problems, which could make the problem worse. Living heart healthy includes:

  • Eating a heart-healthy diet, including whole grains, low-fat dairy, lean meats, and fresh fruits and vegetables
  • Getting regular physical activity
  • Maintaining a healthy weight

Ask your healthcare provider for guidance before making significant changes to your diet or exercise program.

What are the potential complications of pulmonary valve stenosis?

Pulmonary valve stenosis can result in complications including:

  • Endocarditis, which is an infection of the lining of the heart
  • Heart failure
  • Right ventricular hypertrophy, which is enlargement of the right lower heart chamber

What is the prognosis for pulmonary valve stenosis?

People with mild pulmonary valve stenosis typically have a very good outlook. Mild disease usually does not get worse.

Treatment for people with moderate to severe disease is highly successful. For people who have pulmonary valve replacement surgery, it is possible to need another replacement in the future. It is also possible for valve problems to come back due to scar tissue and other issues.

Anyone with pulmonary valve disease will need to see a cardiologist for their entire lives. This is necessary to monitor the disease and the success of treatment.

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2021 Apr 21
THIS TOOL DOES NOT PROVIDE MEDICAL ADVICE. It is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis or treatment. Never ignore professional medical advice in seeking treatment because of something you have read on the site. If you think you may have a medical emergency, immediately call your doctor or dial 911.
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