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.
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.
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.
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.