Specialties & Conditions Pulmonary Vein Stenosis
Pulmonary Vein Stenosis
What Is Pulmonary Vein Stenosis?
Pulmonary vein stenosis is a rare but serious problem in which the veins that connect the lungs to the heart become narrowed.
In the normal heart, blood with less oxygen or “blue blood” returns from the body to the right heart (an atrium and then a ventricle) and is then pumped by the right ventricle through vessels known as pulmonary arteries. The pulmonary arteries carry blue blood to the lungs where it picks up oxygen and becomes “oxygenated” or “red blood”. This blood then returns from the lungs to the heart through four or more veins known as “pulmonary veins”. The blood is then pumped to all parts of the body to deliver the oxygen that is needed for the body to function well.
When there is a blockage in the pulmonary veins, known as “pulmonary vein stenosis” it is more difficult for blood to flow back to the heart from the lungs.
What Are the Symptoms of Pulmonary Vein Stenosis?
The following symptoms can occur in patients with pulmonary vein stenosis:
- Difficulty breathing
- Poor growth
- High blood pressure in the lungs (pulmonary hypertension)
In severe and untreated cases, life-threatening heart failure can occur.
Sometimes, just one of these veins is narrowed and there are not many symptoms. Unfortunately, pulmonary vein stenosis is often a progressive disease that can get worse over time in any affected vein and can also spread to other veins in a short period of time.
Who Is at Risk for Pulmonary Vein Stenosis?
Pulmonary vein stenosis rarely occurs in children without any other health problems. It is more common in premature babies, especially in those with chronic lung disease. When pulmonary vein stenosis occurs without a previous injury or procedure on the pulmonary veins, it is called primary pulmonary vein stenosis.
Pulmonary vein stenosis can occur in children who are born with congenital heart defects that affect the connections of the pulmonary veins to the heart including total and partial anomalous pulmonary venous return (TAPVR or PAPVR).
Pulmonary vein stenosis can also occur after surgery to repair the abnormal drainage of pulmonary veins to the heart. When pulmonary vein stenosis occurs after surgery on the pulmonary veins it is called secondary pulmonary vein stenosis.
How Is Pulmonary Vein Stenosis Diagnosed?
Pulmonary vein stenosis is often diagnosed when an echocardiogram (ultrasound of the heart) is performed to find a cause for symptoms, such as rapid breathing or poor weight gain. Premature infants who require breathing support are screened with echocardiograms to look for pulmonary hypertension and pulmonary vein stenosis may be found in these infants before they have obvious symptoms.
Once pulmonary vein stenosis is suspected, additional tests are often performed and may include:
- Additional echocardiograms
- Computed tomography (CT) scan
- Cardiac magnetic resonance imaging (MRI)
- Lung perfusion scan
- Cardiac catheterization
How Is Pulmonary Vein Stenosis Treated?
Children with pulmonary vein stenosis are often treated with a combination of medications to improve their symptoms and with procedures to improve the narrowing of the pulmonary veins. The best type of procedure for each child depends on the location and extent of the pulmonary vein narrowing and other health characteristics of each patient. Regardless of the type of treatment chosen, children with pulmonary vein stenosis require close monitoring for worsening of the pulmonary vein stenosis over time.
Cardiac Catheterization
During a cardiac catheterization “cath” procedure, the interventional cardiologist will insert a long catheter in your child’s leg vein that reaches up to the heart and can be used to examine and take pictures of each of the pulmonary veins. Special catheters with balloons are then used to push open any narrowed pulmonary vein. A very thin, mesh tube (stent) can be placed inside the narrowed area of the vein to keep the pulmonary veins expanded.
This procedure can improve the narrowing of the pulmonary veins in the short-term, but the narrowing can recur. Cardiac catheterizations are often repeated to treat new or recurrent narrowing in the veins or to increase the size of the pulmonary veins as children grow.
Cardiac Surgery
Some cases of pulmonary vein narrowing are best treated with an open-heart surgery to open the area of narrowing in the veins and allow a normal, healthy part of the vein to drain back to the heart. Depending on the location of the stenosis within the vein, the surgeon will select the most appropriate approach to repair the stenosis. In some cases, pulmonary vein stenosis is treated and does not recur after a single operation.
Many children will require a combination of surgery and catheter procedures.
Medications
Most children with pulmonary vein stenosis are also treated with medications. These may include diuretics and medications to specifically treat pulmonary hypertension. Diuretics help to clear extra fluid from the lungs.
Specific pulmonary hypertension medications can be helpful in some children with pulmonary vein stenosis who have higher blood pressure in their lungs than can be explained just by the pulmonary vein stenosis. There is ongoing research to find other medications to help slow or prevent pulmonary vein stenosis. An immune suppressing medicine called “sirolimus” has shown promise in slowing down the worsening of pulmonary vein stenosis.
For more information about treatment offered at Lurie Children's, visit our Pulmonary Vein Stenosis Program page.
