Pulmonary artery stenosis is a narrowing (stenosis) that occurs in the pulmonary artery, a large artery that sends oxygen-poor blood into the lungs to be enriched with oxygen. The narrowing may occur in the main pulmonary artery and/or in the left or right pulmonary artery branches. This narrowing makes it difficult for blood to reach the lungs to pick up oxygen. Without enough oxygen, the heart and body cannot function as they should. In an effort to overcome the narrowing, the pressure in the right ventricle (the chamber that pumps blood into the pulmonary arteries) rises to levels that can be damaging to the heart muscle.
If the narrowing in the artery is less than 50 percent, your child may not experience any symptoms. However, if the narrowing of the artery is more than 50 percent, your child may experience any of the following symptoms:
Pulmonary artery stenosis is a congenital heart defect, meaning it is a defect that is inborn or exists at birth. Stated another way, the defect is an abnormality, not a disease. Pulmonary artery stenosis is often present in combination with other congenital heart defects, such as:
Other causes of pulmonary artery stenosis can include: other syndromes that affect the heart (such as rubella syndrome [a group of heart and other health problems in an infant caused by rubella infection in the mother during pregnancy] and Williams syndrome [a group of abnormalities affecting the heart and other organs]) and surgical procedures used to correct other heart defects (for example, pulmonary artery banding - a purposeful narrowing of the artery to reduce blood flow to the lungs).
During a routine examination, your child’s doctor may hear abnormal heart sounds (a murmur) when listening to the heart. If abnormal sounds are identified, your doctor will order other tests that can include:
Additional tests may be ordered as necessary.
If your child is diagnosed with pulmonary artery stenosis, your pediatrician or primary care doctor will recommend that you meet with a congenital heart specialist (a doctor who has the training and equipment to determine your child’s heart problem and order the necessary special tests, medical care, heart surgery, and follow-up checkups). The best treatment approach will depend on your child’s symptoms as well as other characteristics of the stenosis. Mild to moderate narrowing in one or more pulmonary artery branches usually does not require treatment, but severe cases do require some form of therapy. Available treatments include:
This treatment method consists of moving a balloon dilation catheter into the narrowed area of the artery. The balloon is carefully inflated – first under low pressure and then under higher pressure – until the narrowed area is widened. The balloon is then deflated and removed. Although the narrowing is improved in a majority of patients following balloon dilation, overtime the artery can again become narrow in as many as 15% to 20% of cases, requiring further ballooning. Different types of balloons are currently being developed that will likely lead to better and longer-lasting results.
Balloon dilation and stent placement
In an effort to improve on the results of balloon dilation, a search for a more effective treatment was begun and led to the development of the stainless steel balloon-expandable stent. Stent placement is accomplished by positioning the stent across the narrowed segment of the artery. The stent is mounted on a balloon angioplasty catheter and covered with a sheath as it is moved into position. The sheath then is withdrawn off the stent-balloon angioplasty assembly and the balloon is inflated to its recommended pressure, expanding the stent and anchoring it in place.
The Cutting Balloon Image with permission, from Boston Scientific Corporation
The Cutting Balloon™
This procedure is similar to standard balloon dilation but the balloon has been specially designed with small blades running up and down its length. When the balloon is inflated, the blades are activated and they cut through the narrowed area, making the vessel easier to dilate and resulting in a larger opening. Cutting balloons are available in different sizes.
Various methods of surgical repair of pulmonary artery stenosis are used, the choice of which depends on the characteristics of the stenosis and the surrounding vessels and other structures.
Doctors vary in quality due to differences in training and experience; hospitals differ in the number of services available. The more complex your medical problem, the greater these differences in quality become and the more they matter.
Clearly, the doctor and hospital that you choose for complex, specialized medical care will have a direct impact on how well you do. To help you make this choice, read more about our Sydell and Arnold Miller Family Heart & Vascular Institute outcomes.
The Center for Adult Congenital Heart Disease in the Sydell and Arnold Miller Family Heart & Vascular Institute is a specialized center involving a multi-disciplinary group of specialists, including cardiologists, cardiac surgeons and nurses from Cardiovascular Medicine, Pediatric Cardiology, Pediatric and Congenital Heart Surgery, Cardiothoracic Surgery, Diagnostic Radiology, Pulmonary, Allergy and Critical Care Medicine, and Transplantation Center, who provide a comprehensive approach to diagnosing and treating adult congenital heart disease.
Learn more about specialized teams of doctors who treat congenital heart disease.
You may also use our MyConsult second opinion consultation using the Internet.
For younger patients with congenital heart disease:
If you need more information, click here to contact us, chat online with a nurse or call the Miller Family Heart and Vascular Institute Resource & Information Nurse at 216.445.9288 or toll-free at 866.289.6911. We would be happy to help you.
Congenital heart disease is diagnosed by a murmur on a physical exam and several diagnostic tests:
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Our outcomes speak for themselves. Please review our facts and figures and if you have any questions don’t hesitate to ask.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition. This document was last reviewed on: 02/21/2019