A health condition in which the arteries that carry blood to the child’s lungs to get fresh oxygen is called pulmonary artery stenosis (narrowing). It also puts extra strain on the heart muscle. Without treatment, this condition can lead to heart failure and other issues throughout the body. However, this condition can be treated with medicines and some procedures. Furthermore, narrowing may occur in the primary pulmonary artery or in its left or right branches.
Symptoms
The symptoms are often different among people with pulmonary artery stenosis. While mild forms of this condition may not cause any symptoms, in more severe cases, you may experience the following symptoms. Check below some examples:
- Dyspnea (shortness of breath)
- Heavy or fast breathing
- Fast heart rate
- Extreme tiredness (fatigue)
- Swelling (edema) in the ankles, face, feet, and eyelids, or abdomen (belly)
- Dizziness
- Passing out
- Cyanosis (blue discoloration) of the fingers, toes, and lips
- Reduced exercise tolerance – In such cases, the child is not able to keep up with other kids as much as usual
Do not hesitate to visit a healthcare professional if any of the previous symptoms occur.
Causes
While some children are born with pulmonary artery stenosis and have problems with the heart valves or other parts of the heart, some of them do not have heart problems. In some cases, people may develop pulmonary artery stenosis due to certain rare conditions. Check below some of them:
Congenital Causes of Pulmonary Artery Stenosis
Approximately 40% of children who are born with pulmonary artery stenosis do not have other heart problems. Only 2% – 3% of people with this condition have congenital (present at birth) heart defects. Check below some of them:
- Tetralogy of Fallot – This disorder is characterized by 4 problems in the child’s heart, impairing normal blood flow.
- Pulmonary atresia – This is a condition in which the pulmonary valve (that connects the right ventricle to the pulmonary artery) does not develop properly. That’s why the blood cannot flow properly to the lungs.
- Truncus arteriosus – In such cases, people are born with one combined heart artery instead of two. As a result, the oxygen-rich blood blends with oxygen-poor blood.
- Aortic valve stenosis – This is a heart problem in which the valve reduces the amount of blood that goes through the child’s body.
- Atrial septal defect – This congenital heart defect causes a hole in the wall between the child’s two upper heart chambers (atria). Hence, it lets the blood with and without oxygen mix.
- Ventricular septal defect – In such cases, the hole occurs between the lower chambers of the heart (ventricles), and causes an increased flow of blood to the lungs.
- Transposition of the great vessels – This condition causes the blood flow away from the child’s heart through two major arteries. As a result, it impairs normal blood flow and reduces the amount of oxygen the cells get throughout the body.
- Patent ductus arteriosus – Normally, there is an opening between the pulmonary artery and the aorta during pregnancy in the fetus. This opening often closes shortly after birth. In people with patent ductus arteriosus, this opening does not close, which often leads to increased blood flow to the lungs.
Surgical Causes of Pulmonary Artery Stenosis
In some cases, people may develop this condition after surgery. For example:
- Lung transplant
- Surgical procedures to fix the child’s congenital heart defects or improve blood flow
- Pulmonary artery banding – This procedure is used to narrow the child’s artery to reduce excessive blood flow to the lungs.
Other Causes of Pulmonary Artery Stenosis
There are some infections and genetic conditions that may also contribute to pulmonary artery stenosis. Examples include:
- Rubella syndrome – This is a group of heart and other diseases that children often get during pregnancy.
- Williams syndrome – This syndrome causes a group of abnormalities that negatively affect the heart and other organs.
- Alagille syndrome – In such cases, children often have problems with the heart and liver.
- Takayasu’s arteritis – This is a type of inflammation that can cause serious damage to the large blood vessels in the child’s body.
Diagnosis
Mostly, the diagnosis of this condition begins with a physical examination in which doctors will use a stethoscope to listen to your heart for abnormalities (such as a murmur). However, to confirm or rule out this condition, doctors often perform the following tests and procedures. Examples include:
- Electrocardiogram (ECG or EKG) – This is a quick and painless test used to measure the electrical activity of the heart. It usually helps identify irregular heart rhythms (arrhythmias) and heart muscle stress.
- Chest X-ray – This is an imaging test used to examine the shape and size of the heart, lungs, and pulmonary arteries.
- Echocardiogram – This test uses sound waves to produce a moving image of the heart muscle and its valves.
- Cardiac MRI (magnetic resonance imaging) – This is another imaging test used to get more detailed images of the heart, lungs, and pulmonary arteries.
- CT (computed tomography) scans – This test is used to get cross-sectional pictures of the heart, lungs, and other organs in the body. It helps doctors see your child’s heart anatomy and blood circulation.
- Cardiac catheterization – During this procedure, doctors often use a small and flexible tube (catheter) that is inserted into a vein or artery and guided to the heart muscles. This test can check the level of oxygen in the blood.
- Pulmonary angiography – This is a dye-enhanced X-ray of the heart’s pulmonary arteries and veins.
- Perfusion scan – During this test, doctors will inject a small amount of radioactive material and then use a special machine to see how the blood is flowing through each lung.
Once your child is diagnosed with pulmonary artery stenosis, doctors may recommend seeing a congenital heart specialist. They may also categorize your condition in terms of type I, II, III, or IV, which means how many parts of the artery are narrowed.
Treatment
Doctors usually prescribe different treatments for children with pulmonary artery stenosis because it depends on several factors. These include the cause and severity of the condition, overall health, the child’s response to treatment, and others. Generally, doctors often prescribe the following treatments. For example, balloon dilation (angioplasty), balloon dilation and stent placement, the cutting balloon, or surgery. In addition, the previous treatment options may cause some complications. For instance, about 15% to 20% of people get the same artery narrowed again over time, despite successful treatment. Check some treatment complications below:
- Pulmonary artery rupture, dissection, or aneurysm
- Pulmonary edema (swelling)
- Ventricular arrhythmias
- Blood clots
This article does not contain all possible treatment complications. However, talk with your doctor about ways to reduce the risk of experiencing them.
Frequently Asked Questions
Who does pulmonary artery stenosis affect?
In general, this condition occurs quite rarely and usually affects children with congenital heart defects, or it may happen in isolation (the child does not have other heart problems). Sometimes, it may appear after some heart surgeries.
How soon after treatment will my child feel better?
Commonly, the child will begin to feel better right away after procedures to expand the pulmonary artery.
What happens after surgery?
Usually, after surgery, doctors will recommend antibiotics and blood thinners (also known as anticoagulants) for a short period. These medicines are used to prevent infections and blood clots. If you have additional questions, ask your healthcare provider.
