Pulmonary Atresia (PA)

What is pulmonary atresia?

Pulmonary AtresiaPulmonary 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.

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 for normal growth and function.

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.

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.

Although each child may experience symptoms differently, the following are the most common symptoms of pulmonary atresia:

  • Rapid breathing
  • Difficulty breathing
  • Poor feeding
  • Exhaustion
  • 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, so it is very important to contact the child’s physician if you suspect the child may be experiencing any of these symptoms.

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 a newborn. The 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 and condition. Some tests that may be recommended include the following:

  • Chest X-ray. A diagnostic test that uses X-ray beams to produce images of internal tissues, bones, and organs onto film. Learn more about chest X-rays.
  • 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. Learn more about electrocardiogram.
  • 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. Learn more about echocardiogram.
  • 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. Learn more about cardiac catheterization.

How is pulmonary atresia treated?

Specific treatment for pulmonary atresia will be determined by your child’s physician. Children with pulmonary atresia will most likely be admitted to the cardiovascular intensive care unit (CVICU) or neonatal intensive care unit (NICU) once symptoms are noted. Initially, the child may be placed on oxygen, and possibly even on a ventilator, to assist his or her breathing. IV medications may be given to help the heart and lungs function more efficiently.

Other important aspects of initial treatment include the following:

  • A cardiac catheterization procedure can be used as a diagnostic procedure, as well as an initial treatment procedure for some heart defects. A cardiac catheterization procedure may be performed to evaluate the defect(s), and the amount of blood that is able to get to the lungs to become oxygenated. Learn more about cardiac catheterization.
  • As part of the cardiac catheterization, a procedure called balloon atrial septostomy may be performed to improve mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood between the right and left atria. A special catheter with a balloon in the tip is used to create an opening in the atrial septum (wall between the left and right atria). The catheter is guided through the foramen ovale (a small opening present in the atrial septum that closes shortly after birth) and into the left atrium. The balloon is then inflated, and the catheter is quickly pulled back through the hole, into the right atrium, and a hole is created, allowing blood to mix between the atria. This procedure may also be done in the CVICU.
  • An IV medication called prostaglandin E1 may also be given to keep the ductus arteriosus from closing.

Learn more about heart surgery.

These interventions will allow time for your baby to stabilize his or her blood while more definite repair is planned.

Ultimately, surgery is necessary to improve blood flow to the lungs on a permanent basis. The exact type of surgery needed depends on a number of factors, including the size of the right ventricle and whether there is a communication between the right and left ventricles (a ventricular septal defect or VSD). Your child’s cardiologist, heart surgeon and intensive care doctor will carefully review your child’s unique situation and explain their recommendations for treatment.

What is the long-term outlook after pulmonary atresia surgical repair?

The long-term outlook after pulmonary atresia repair varies from child to child. Children should receive lifelong follow-up care at a center offering pediatric (and ultimately adult) congenital cardiac care. It is not unexpected for further surgery to be performed to replace conduits or revise a palliation.

Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individual’s lifespan.

Consult your child’s physician regarding the specific outlook for your child.

Postoperative Care for Your Child

After surgery, infants will return to the CVICU to be closely monitored during recovery.

While your child is in the CVICU, special equipment will be used to help him or her recover, and may include the following:

  • Ventilator. A machine that helps your child breathe while he or she is under anesthesia during the operation. A small, plastic tube is guided into the windpipe and attached to the ventilator, which breathes for your child while he or she is too sleepy to breathe effectively on his or her own. After a pulmonary atresia, children will benefit from remaining on the ventilator overnight or even longer so they can rest.
  • Intravenous (IV) catheters. Small, plastic tubes inserted through the skin into blood vessels to provide IV fluids and important medicines that help your child recover from the operation.
  • Arterial line. A specialized IV placed in the wrist or other area of the body where a pulse can be felt, that measures blood pressure continuously during surgery and while your child is in the CVICU.
  • Nasogastric (NG) tube. A small, flexible tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
  • Urinary catheter. A small, flexible tube that allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. After surgery, the heart will be a little weaker than it was before, and, therefore, the body may start to hold onto fluid, causing swelling and puffiness. Diuretics may be given to help the kidneys remove excess fluid from the body.
  • Chest tube. A drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.
  • Heart monitor. A machine that constantly displays a picture of your child’s heart rhythm, and monitors heart rate, arterial blood pressure, and other values.

The child may need other equipment not mentioned here to provide support while in the CVICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.

After catheterization or surgical procedure, patients are kept as comfortable as possible with several different medications; some relieve pain, and some relieve anxiety. The staff will also ask for your input on how best to soothe and comfort your child.

As your child recovers, you will be provided with instruction on how to care for the recovering child at home. Caregivers are provided written instructions regarding medications, activity limitations and follow-up appointments before the child is discharged.

Infants who have spent a lot of time on a ventilator, or who were fairly ill while in the CVICU, may have trouble feeding initially. These babies may have an oral aversion; they might equate something placed in the mouth, such as a pacifier or bottle, with a less pleasant sensation, such as being on the ventilator. Some infants are just tired, and need to build their strength up before they will be able to learn to bottle-feed. Strategies used to help infants with nutrition include the following:

  • High-calorie breast milk or formula. Special nutritional supplements may be added to pumped breast milk or formula that increase the number of calories in each ounce, thereby allowing your baby to drink less and still take in enough calories to grow properly.
  • Supplemental tube feedings. Feedings given through a small, flexible tube that passes through the nose, down the esophagus, and into the stomach, that can either supplement or take the place of bottle feedings. Infants who can drink part of their bottle, but not all, may be fed the remainder through the feeding tube. Infants who are too tired to bottle feed at all may receive their formula or breast milk through the feeding tube alone.
  • Feeding therapy. Feeding therapists often work with babies who have heart surgery to help them learn how to eat. CHOC has developmental specialists who specialize in working with infants and children who have difficulty with eating. If your infant or child is not taking in enough calories by mouth for healing and growth, your physician may order an evaluation and treatment with one of our feeding team. Learn more about feeding and swallowing therapy.

Caring for Your Child at Home Following Pulmonary Atresia Surgical Repair

Pain medications, such as acetaminophen, may be recommended to keep the child comfortable. Your child’s physician will discuss pain control before the child is discharged from the hospital.

If any special treatments are to be given at home, the nursing staff will ensure that you are able to provide them, or a home health agency may assist you.

You may receive additional instructions from your child’s physicians and the hospital staff.

Call us for a consultation

Our pediatric cardiologists are available to provide consultations and second opinions to families and referring physicians.

To schedule a consultation,
Call 714-509-3939

If you are pregnant and would like a prenatal consultation, our team of fetal cardiologists are available for families and referring physicians to provide immediate consultations, second opinions and fetal echocardiograms for pregnant women. Our office is open 8 a.m. – 5 p.m. We schedule appointments within 48 hours, and offer same day appointments for urgent cases. Get more information about the CHOC fetal cardiology program.

To schedule a prenatal consultation,
Call 714-509-3960