Pulmonary Stenosis

Pulmonary stenosis, also known as pulmonic stenosis, is a congenital heart defect that affects the pulmonary valve between the right ventricle of the heart and the pulmonary artery. It occurs when the valve is too small, too narrow, or stiff.

About Pulmonary Stenosis

Pulmonary stenosis occurs in the first eight weeks of fetal development as the heart’s four chambers develop from a hollow tube. The pulmonary valve has three leaflets that let blood flow into the pulmonary artery and prevent blood from flowing backward into the right ventricle. In the case of pulmonary stenosis, the valve may not open fully because the leaflets are too thick or partially fused together. Pulmonary stenosis can also occur if the heart muscle under the valve is too thick, constricting the flow of blood through the valve, or the pulmonary artery is too narrow.

A defective valve or slim passageway forces the right ventricle to work harder to push blood through the smaller opening. With time, the right ventricle can tire and stop pumping efficiently, causing higher blood pressure in the right atrium (upper chamber) and veins returning blood to the heart. As a result, your child may suffer congestive heart failure. With pulmonary stenosis, the right ventricle can thicken from being overworked, increasing the risk of cardiac arrhythmia. Children with defective heart valves are also at a higher risk for developing infective endocarditis, an infection of the heart’s inner lining or a heart valve.

Moderate to severe pulmonary stenosis can worsen over time but treatments can be very effective, and your child should be able to have a healthy, active life.

Symptoms of Pulmonary Stenosis

The severity of pulmonary stenosis varies. Children with mild pulmonary stenosis may not have any symptoms. Children who have severe pulmonary stenosis may experience significant symptoms and can feel very sick.

Symptoms of pulmonary stenosis may include:

• A blue color in the skin, lips, and nails
• Fatigue
• Heavy or rapid breathing
• Heart murmur
• Poor weight gain
• Rapid heart rate
• Shortness of breath
• Swelling of the feet, ankles, face, eyelids, or abdomen
• Trouble feeding

Diagnosing Pulmonary Stenosis

During physical examinations, your child’s doctor listens to your child’s heart and lungs and may detect a heart murmur, which are extra sounds heard throughout the cardiac cycle due to increased blood flow. If your pediatrician suspects increased blood flow, a recommendation to see a pediatric cardiologist may be made.

Tests performed when diagnosing pulmonary stenosis may include:

• Cardiac Catheterization: During cardiac catheterization, a small catheter (thin tube) is inserted into a larger blood vessel, typically in the groin, and guided to the heart where blood pressure and oxygen measurements can be taken in the aorta and pulmonary artery as well as the four chambers of the heart. A dye can also be injected through the tube to make the heart’s structure more visible on an X-ray.
• Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies.
• Chest X-Ray: A chest x-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs.
• Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood.
• Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles.
• Pulse Oximetry: A pulse oximeter, or oxygen saturation test, is an electronic tool used to measure oxygen in the bloodstream.

Treating Pulmonary Stenosis

If your child has mild to moderate pulmonary stenosis, treatment may not be necessary. Your child's pediatric cardiologist will likely recommend regular appointments to monitor your child’s heart function to ensure pulmonary stenosis doesn't worsen. Children with severe to critical pulmonary stenosis may require treatment to open up the valve.

Treatment options for pulmonary stenosis may include:

• Balloon Valvuloplasty: Using the cardiac catheterization method, a small tube can be inserted through a blood vessel in the groin and guided to the heart. A balloon can be pushed through the tube and inflated in the aorta to stretch the narrow portion.
• Surgery: A pediatric heart surgeon may perform a surgical repair utilizing different methods. The valve may be incised or scar tissue released from the leaflets, the valve may be removed and replaced with an outflow patch to enlarge the narrowed area, or the valve may be replaced entirely.

After recuperating from treatment, most children born with pulmonary stenosis can go on to lead active, unrestricted lives. Your pediatric cardiologist may recommend ongoing care for the rest of your child’s life to monitor the heart and the pulmonary valve. Another balloon valvuloplasty may be needed to stretch the valve again or the valve may need to be replaced as your child grows.

Care Team Approach

The Texas Center for Pediatric and Congenital Heart Disease, a clinical partnership between Dell Children’s Medical Center and UT Health Austin, takes a multidisciplinary approach to your child’s care. This means your child and your family will benefit from the expertise of multiple specialists across a variety of disciplines. Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way. We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your child’s care with referring physicians and other partners in the community to ensure that we are providing comprehensive, whole-person care.

Learn More About Your Care Team