Pulmonary Atresia

What is pulmonary atresia?

Anatomy of the heart, normal

Pulmonary atresia (PA) is a heart defect that occurs due to abnormal development of the fetal heart during the first eight weeks of pregnancy.

Pulmonary atresia means that there is an abnormal development of the pulmonary valve. The pulmonary valve is found between the right ventricle and the pulmonary artery, which is the large artery that goes to the lungs. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery and to the lungs, but not backward into the right ventricle.

With pulmonary atresia, problems with the valve prevent the leaflets from opening; therefore, blood cannot flow forward from the right ventricle to the lungs.

Anatomy of a heart with pulmonary atresia with VSD

Often, if blood is blocked from exiting the pulmonary valve, there is a second opening in the ventricular wall. The ventricular wall is the wall of heart muscle that separates the left ventricle from the right ventricle of the heart. This opening is called a ventricular septal defect (VSD).

There may also be a second opening, or "hole" between the two upper chambers of the heart,the left and right atria. This defect is called an atrial septal defect (ASD). These holes allow blood in the obstructed right ventricle a way out of the heart. The blood then ultimately crosses to the left side of the heart and is pumped out to the body.

This situation cannot support life, because the blood never makes it to the lungs to become oxygenated, and oxygen-poor (blue) blood cannot meet the body's demands.

Sometimes, newborns will rely on a connection between the aorta and the pulmonary artery, called the ductus arteriosus.That allows some of the oxygen-poor (blue) blood to bypass the blocked right ventricle and get to the lungs. This ductus arteriosus is persistent from normal fetal circulation, and unfortunately, this ductus arteriosus normally closes within a few hours or days after birth.

Because of the low amount of oxygen provided to the body, pulmonary atresia is a heart problem that can ultimately result in cyanosis, or a blue color to the skin from lack of oxygen.

Prevention & Risk Treatment

Prevention & Risk Treatment

What causes pulmonary atresia?

The problem occurs as the heart is forming during the first eight weeks of fetal development.

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most of the time, this heart defect occurs sporadically (by chance), with no clear reason for its development.

What are the symptoms of pulmonary atresia?

Symptoms can be noted shortly after birth or several weeks later as the ductus arteriosus closes. The most obvious symptom is blue, or cyanotic, skin in a newborn.

The following are the most common symptoms of pulmonary atresia. However, each child may experience symptoms differently. Symptoms include:

  • Rapid breathing
  • Difficulty breathing
  • Poor feeding
  • Lethargy
  • Pale, cool, or clammy skin
  • Blue color of the lips or skin

The symptoms of pulmonary atresia may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

Diagnosis

Diagnosis

How is pulmonary atresia diagnosed?

A pediatric cardiologist and/or a neonatologist may be involved in your child's care. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A neonatologist specializes in illnesses affecting newborns, both premature and full-term.

Cyanosis is a major indication that there is a problem with your newborn. Your child's physician may have also heard a heart murmur during a physical examination.

Diagnostic testing for congenital heart disease varies by the child's age, clinical condition, and institutional preferences. Some tests that may be recommended include the following:

  • Chest X-ray. A diagnostic test which uses X-ray beams to produce images of internal tissues, bones, and organs onto film.
  • Electrocardiogram (ECG or EKG). A test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle stress.
  • Echocardiogram (echo). A procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
  • Cardiac catheterization. A cardiac catheterization is an invasive procedure that gives very detailed information about the structures inside the heart. Under sedation, a small, thin, flexible tube (catheter) is inserted into a blood vessel in the groin, and guided to the inside of the heart. Blood pressure and oxygen measurements are taken in the four chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye is also injected to more clearly visualize the structures inside the heart.
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Brayden's Story

"The world of congenital heart defects is scary, but to know that there are people like those at Children's National to help guide you along the way is very comforting."

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Riley's Story

"Richard Jonas, MD, and his fellows came in and explained it all to me the best they could. One even drew me a picture. I was dealing with an issue that I really had no idea was such a big issue."

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