Pulmonary Artery Stenosis

Pulmonary artery stenosis (narrowing) affects the artery that carries blood to your child’s lungs to pick up oxygen. To make up for this limited blood flow, right ventricle pressure can go high enough to injure heart muscle.

Causes #

Tetralogy of Fallot #

A condition characterized by four problems in your child’s heart, impairing normal blood flow.

Pulmonary atresia #

A defect in which the pulmonary valve, which connects the right ventricle to the pulmonary artery, doesn’t form at all. This keeps blood from flowing to your child’s lungs.

Truncus arteriosus #

One combined heart artery instead of the normal two lets oxygen-rich blood blend with oxygen-poor blood.

Aortic valve stenosis #

A problem with this valve reduces how much blood goes from your child’s heart to their body.

Atrial septal defect #

A hole in the wall between your child’s two upper heart chambers (atria) lets blood with and without oxygen mix.

Ventricular septal defect #

A hole in the wall that separates your child’s two lower chambers (ventricles) can make too much blood go to their lungs.

Transposition of the great vessels #

The two major arteries that carry blood away from your child’s heart are in each other’s places. This impairs normal blood flow and limits how much oxygen can get to your child’s cells.

Patent ductus arteriosus #

This is a connection between your child’s pulmonary artery and aorta. If it doesn’t close after birth as it should, too much blood goes to the lungs.

Symptoms #

• Shortness of breath.

• Fatigue.

• Heavy or rapid breathing.

• Rapid heart rate.

• Swelling in the feet, ankles, face, eyelids and/or abdomen.

• Dizziness or passing out.

• Cyanosis (blue discoloration) of the lips, fingers, and toes.

• Reduced exercise tolerance (not able to keep up with other kids or play as much as usual).

Diagnosis #

Electrocardiogram (ECG or EKG) #

A test that records the electrical changes that occur during a heartbeat, reveals abnormal heart rhythms (arrhythmias) and detects heart muscle stress.

Chest X-ray #

A test to show the size and shape of the heart, lungs and pulmonary arteries.

Echocardiogram #

A test that uses sound waves to make a moving picture of the heart muscle and valves. Cardiac magnetic resonance imaging (MRI): A test that uses three-dimensional imaging to show how blood flows through your child’s heart and vessels.

CT scan #

An X-ray procedure that uses a computer to combine many X-ray images of your child’s heart into cross-sectional views. IV contrast (dye) lets your child’s provider see their heart’s anatomy and blood circulation.

Cardiac catheterization #

A procedure that involves inserting a thin tube (catheter) into a vein or artery and passing it into the heart. A provider can check the level of oxygen, measure pressure changes and make X-ray movies of the heart.

Pulmonary angiography #

A dye-enhanced X-ray of your heart’s pulmonary arteries and veins.

Perfusion scan #

A test in which you get an injection of a small amount of a radioactive material. A special machine shows how well blood is flowing through each lung.

Treatment #

Pulmonary artery stenosis treatments include:

Balloon dilation (angioplasty) #

Your child’s provider will:

• Move a balloon dilation catheter into the narrowed area of the artery.

• Carefully inflate the balloon, going from low pressure to higher pressure.

• Widen the narrowed artery.

• Deflate and remove the balloon.

Balloon dilation and stent placement (preferred method) #

Your child’s provider will:

• Position a balloon-expandable stent across the artery’s narrow part.

• Mount the stent on a balloon angioplasty catheter and cover it with a sheath.

• Move the stent into position.

• Remove the sheath from the stent-balloon angioplasty assembly.

• Inflate the balloon to the right pressure, expand the stent and anchor it in place.

The Cutting Balloon #

• This balloon is similar to a standard one. However, the balloon has small blades running up and down its length.

• When your child’s surgeon inflates the balloon, they activate the blades, which cut through the narrowed area.

• This makes the vessel easier to dilate and results in a larger opening.

• This option gives good results for many people who don’t have an associated congenital heart problem. However, the artery can become narrow again in as many as 21% of people over several months.

Surgery #

Surgeons use various methods to repair pulmonary artery stenosis. The choice depends on what the stenosis is like. They also look at the surrounding vessels and other structures.