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Pulmonary Valve Stenosis

Pulmonary stenosis is a rare heart defect that results in a narrowing of the pulmonary valve, the one-way opening that lets blood flow from the heart to the lungs. The areas just before or after the pulmonary valve might also be affected. 

The stenosis (narrowing) may be mild, moderate or severe. Mild cases rarely get worse, while moderate to severe pulmonary stenosis usually gets worse over time.

When the pulmonary valve becomes too narrow to let blood flow easily, the blood backs up, resulting in serious issues, including arrhythmias (irregular heartbeats), infections and heart failure.

Causes of pulmonary stenosis

  • Pulmonary stenosis is primarily congenital (present at birth) and is usually discovered at birth when the doctor hears a murmur in the baby’s heart. The cause is unknown, though genetics may play a part. Many times, other congenital heart defects are found in addition to pulmonary stenosis, which can affect the course of treatment.
  • Pulmonic stenosis can also result many years after surgery for another congenital heart defect, tetralogy of Fallot.
  • Pulmonary stenosis may also be a complication of other health conditions later in life, such as rheumatic fever, Noonan's syndrome or carcinoid syndrome.

Pulmonary stenosis symptoms

Though some people with pulmonary stenosis have no symptoms at all, others have one or more of the following:

  • Heart murmur (heard through a stethoscope, it is a blowing, whooshing or grating sound that the blood makes as it moves through the heart)
  • Chest pain
  • Heart palpitations (irregular, pounding or skipped heartbeats)
  • Fainting
  • Swelling in the abdomen, feet or face
  • Shortness of breath or rapid breathing
  • Fatigue (especially after exercising)

How can I get help for pulmonary stenosis?

Call your primary care doctor if you are experiencing any of the symptoms of pulmonary stenosis. After examining you, your primary care doctor may send you to a cardiologist for further evaluation and treatment.

If you are experiencing severe shortness of breath or chest pain, seek immediate medical care by calling 911 or going to an emergency room for evaluation.

Care for pulmonary stenosis at Rush

At the Rush Center for Adult Structural Heart Disease, you will be examined, undergo diagnostic tests and meet with a team of heart specialists, including an interventional cardiologist and a cardiac surgeon, to learn more about your condition and your treatment options. 

Diagnosis

To get a close look at the structure of your heart and how it is functioning, you may need one or more of the following diagnostic tests:

  • Cardiac catheterization: Your doctor may order cardiac catheterization, a minimally invasive procedure using a catheter (a thin, flexible tube) that is guided to the heart. A dye is injected through the catheter that enhances X-ray images of the arteries in your heart. The test shows how well your heart is functioning as well as the pressure inside the chambers of your heart.
  • Echocardiogram: Using sound waves, this test shows the structure of your heart and how it is functioning.
  • Electrocardiogram (ECG): This test measures the electrical activity of your heart, including arrhythmias (irregular heartbeats).
  • Imaging: You may have a chest X-ray, MRI and/or CT scan to get a more precise view of your heart and lungs.

Treatment

Treatment depends on how severe your stenosis is, your overall condition and whether there are other heart problems present. Your treatment team may recommend one or more of the following:

  • Watchful waiting: If you have mild to moderate pulmonary stenosis, you may not need treatment at this time and your doctor will continue to monitor your condition.
  • Medication: If you have other heart defects in addition to pulmonary stenosis, your doctor may prescribe medication to help your heart function better.
  • Valvuloplasty: If you don't have any other heart deformities, your cardiologist may perform valvuloplasty (balloon dilation) to stretch the narrow opening and allow more blood to flow through. Your cardiologist guides a catheter (a thin, flexible tube) to your pulmonary valve, then inflates a balloon at the tip of the catheter. Balloon valvuloplasty can be quite effective in relieving the obstruction.
  • Surgery: If there are other heart deformities present, or valvuloplasty is not possible or has not been effective, your heart surgeon may perform open heart surgery to correct your stenosis or replace the pulmonary valve with a biological or synthetic valve.

Why choose Rush for care of pulmonary stenosis

  • Team-based approach: At the Rush Center for Adult Structural Heart Disease, you meet with a team of specialists — including an interventional cardiologist and a cardiac surgeon — at the same time to ensure coordinated, comprehensive care.
  • Expert care: Rush's heart program is consistently ranked among the best in the country by U.S.News & World Report.
  • Collaboration and convenience: Some of the procedures used to treat pulmonary stenosis take place in Rush's interventional platform, where procedures are centralized to allow specialists to collaborate more easily and ensure convenience for patients and families.

Departments and programs that treat this condition