Pulmonary Vein Stenosis

Pulmonary vein stenosis is a congenital heart defect in which there is a narrowing or blockage in the veins that bring oxygenated blood from the lungs to the heart.

About Pulmonary Vein Stenosis

The right side of the heart pumps blood to the lungs, where the blood becomes oxygenated before traveling back to the heart through the pulmonary veins and being pumped out to the rest of the body. When pulmonary vein stenosis occurs, oxygenated blood leaving the lungs cannot easily return to the left side of the heart, causing a backup of blood in the lungs.

The exact cause of pulmonary vein stenosis is not known. While pulmonary vein stenosis can be present at birth, it can also occur as a complication associated with another heart or lung problem. Pulmonary vein stenosis is typically progressive (tends to get worse with time) and can be a very difficult problem to manage.

Symptoms of Pulmonary Vein Stenosis

As the pulmonary veins become narrower, the blood has difficulty flowing from the lungs, which causes pulmonary congestion and can lead to congestive heart failure.

Symptoms of pulmonary vein stenosis may include:

• Fast, shallow breathing
• Fatigue
• Difficulty feeding
• Poor weight gain
• Rapid heart rate
• Shortness of breath
• Skin, lips, or nail beds that appear blue in color (cyanosis)
• Skin that is pale

Diagnosing Pulmonary Vein 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. When a child has pulmonary vein stenosis, these abnormal heart and lung sounds are caused by the backing up of the blood in the lungs. If your pediatrician suspects increased blood flow, a recommendation to see a pediatric cardiologist may be made.

Tests performed when diagnosing pulmonary vein 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.

Treating Pulmonary Vein Stenosis

Treatment options for children with pulmonary vein stenosis are depending upon how severe the narrowing is as well as the number of veins affected.

Treatment options for pulmonary vein stenosis may include:

• Medications: Medications may be prescribed to help relieve the congestion in the lungs, to lower the elevated pressure in the lungs, or to attempt to slow the thickening of the walls of the pulmonary veins that is causing the narrowing.
• Balloon Angioplasty: Using the same method as cardiac catheterization, 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 to stretch the vein open and improve the narrowing. A stent may be added after the balloon is removed to ensure the area remains open.
• Surgery: If surgery is required, an individualized treatment plan will be created for your child and the type of surgery will be dependent upon the details specific to their condition.

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