Pulmonary Atresia

Overview

What is pulmonary atresia?

Pulmonary atresia is a congenital (present at birth) heart defect in which your pulmonary valve doesn’t develop normally or stays blocked after birth. Without a working pulmonary valve, blood can’t flow through your pulmonary artery to reach your lungs, where it gets oxygen. Instead, oxygen-poor blood goes throughout your body.

The pulmonary valve, one of four valves in your heart, is located on the right side of your heart. It’s between your right ventricle and your pulmonary artery (the blood vessel that carries blood to your lungs).The pulmonary valve usually opens to let blood flow from your right ventricle into your pulmonary artery.

How does pulmonary atresia affect my baby?

In addition to not having a normal pulmonary valve, a baby with pulmonary atresia may have:

  • Lips, fingers, and toes that look blue because of a lack of oxygen in their blood.
  • An underdeveloped right ventricle and tricuspid valve (the valve connecting the right atrium and right ventricle).
  • An opening (the foramen ovale) between their heart’s right atrium and left atrium that may stay open instead of closing like it should after birth. This allows oxygen-poor blood to pass from the right atrium to the left atrium, where it flows through the left ventricle to the aorta.

In babies with pulmonary valve atresia, there also may be a problem with a dividing wall in their heart. This wall normally helps blood go where it’s supposed to go.

Your heart has four chambers, with a solid wall called the septum between your heart’s two sides. The right side sends blood to your lungs to get oxygen. The left side of your heart moves oxygen-rich blood to the rest of your body through your aorta (the main artery in your heart).

Pulmonary atresia types

  • Pulmonary atresia with a ventricular septal defect. This opening in the wall (septum) between the right and left ventricles allows oxygen-rich blood to mix with oxygen-poor blood.
  • Pulmonary atresia with an intact ventricular septum. The wall between the left and right sides of your heart is whole (intact).

Who does pulmonary atresia affect?

Genetic factors, such as an abnormal gene or chromosomal defect, may increase the chances of heart defects in certain families. Some children with genetic disorders like DiGeorge’s syndrome or velocardiofacial syndrome may be at greater risk for pulmonary atresia.

Is pulmonary atresia rare?

Yes. Pulmonary atresia occurs in about one out of 10,000 live births.

Symptoms and Causes

What are the symptoms of pulmonary atresia?

Symptoms of pulmonary atresia often appear within the first few hours or days of a baby’s life and may include:

  • Rapid breathing.
  • A bluish tinge to the skin, especially the lips, fingers and toes.
  • Cool, pale or clammy skin.
  • Breathing problems.
  • Fatigue or tiredness.
  • Irritability.
  • Poor feeding.

What causes pulmonary atresia?

The exact cause of pulmonary atresia is unknown. Pulmonary valve atresia occurs during the first eight weeks that a fetus is developing in the uterus.

Diagnosis and Tests

How is pulmonary atresia diagnosed?

While you’re pregnant, your healthcare provider will do standard screenings to check on the health of the fetus. If they see something of concern on an ultrasound (using harmless sound waves), they can do a fetal echocardiogram. This also uses sound waves to get a closer look at the fetal heart.

After you have your baby, their healthcare provider will check their heart and lungs to find out if there are any problems. If they hear a heart murmur through a stethoscope, they’ll order tests that may include:

  • Pulse oximetry: This noninvasive test tells how much oxygen is in your baby’s blood.
  • Chest X-ray: An imaging method that uses painless X-rays to get a picture of the inside of your baby’s chest from the outside.
  • Electrocardiogram (EKG or ECG): A painless and noninvasive way to measure the electrical activity of your baby’s heart to find out if there’s any stress on their heart muscle.
  • Echocardiogram: A painless, noninvasive imaging method. It uses harmless sound waves to produce a moving picture of your baby’s heart and heart valves.
  • Cardiac catheterization: An invasive procedure that lets a provider view your baby’s heart. They insert a small, flexible tube into a blood vessel in your baby’s groin. They move it through to the inside of their heart. They may use contrast dye to make a stronger image of your child’s heart and its valves.

Management and Treatment

How is pulmonary atresia treated?

Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs.

Pulmonary atresia treatment depends on how the condition affects your child and on their:

  • General health.
  • Age.
  • Medical history.

Short-term treatments

Temporary treatments include medication and balloon atrial septostomy. Most children will probably need more surgery to improve blood flow to their lungs.

  • Medication. Newborns with pulmonary atresia usually receive an IV drug (injected into a vein) called alprostadil. This drug prevents the ductus arteriosus (which connects the aorta to the pulmonary artery) from starting to close as it normally would within a few hours to days after birth. By keeping this open, oxygen-poor blood can flow from your baby’s aorta to their pulmonary arteries. Then it goes to their lungs to pick up oxygen.
  • Balloon atrial septostomy. Your child’s healthcare provider can use cardiac catheterization to enlarge the opening in their septum (the wall between the right atrium and left atrium). This improves blood flow to your baby’s lungs. They insert a catheter into your child’s heart through a blood vessel and guide it through the foramen ovale. They inflate the balloon at the tip of the catheter. They quickly remove the catheter to make an opening in the atrial septum. Oxygen-poor blood can then flow from the right to the left atrium and into the left ventricle and aorta.

What surgeries are used?

The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including:

  • The size of their right ventricle and pulmonary artery.
  • The ability of their right ventricle to pump blood.

Surgery for pulmonary atresia with a ventricular septal defect

Usually, the right ventricle is well developed and can pump blood to your baby’s lungs. Surgery involves:

  • Closing your baby’s ventricular septal defect.
  • Placing a donated artery and valve between your child’s right ventricle and pulmonary artery. This lets blood flow through their right ventricle into their pulmonary artery and to their lungs.

Surgery for pulmonary atresia without a ventricular septal defect

In this case, your baby’s right ventricle is usually poorly developed. They need a series of three operations during the first few years of their lives. These open heart surgeries redirect the flow of oxygen-poor blood directly to their pulmonary artery and lungs:

  1. The first operation involves placing a shunt or tube directly from your baby’s aorta or one of its branches to their pulmonary artery. This ensures that enough blood is flowing to their lungs during the first few months of life.
  2. Between 4 and 8 months of age, your child has a second operation, called the bi-directional Glenn or hemi-Fontan procedure. A healthcare provider removes the shunt that was put in during the first operation. Then they connect the superior vena cava (a major vein that carries deoxygenated blood from the upper body to the heart) and the right pulmonary artery. This allows blood to flow from your child’s superior vena cava directly to their pulmonary artery and lungs.
  3. Between ages 3 and 6 years old, your child receives their third and final operation, known as the Fontan procedure. During this surgery, a provider connects the inferior vena cava (the vein that carries deoxygenated blood from the lower body to the heart) to the pulmonary artery.

Complications of the treatment

After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. They’ll need a ventilator (breathing machine) and heart monitor. In addition, they’ll get medications through an IV catheter to provide pain relief and lessen the work their heart has to do.

Some infants may have feeding problems or might be too weak to eat after surgery. They may receive nourishment through a nasogastric tube or high-calorie formula instead of, or along with, formula or a parent’s milk.

Outlook / Prognosis

What can I expect if my child has pulmonary atresia?

Complications of pulmonary atresia may include:

It is also very important to monitor for complications after surgical repair. Fontan circulation is not normal, so your child will need close lifelong follow up. Over time, people with Fontan circulation may develop signs of heart failure (difficulty breathing, fatigue, swelling in the belly and legs, poor exercise tolerance), liver dysfunction, and abnormal heart rhythms. Some people need a heart transplant.

Is pulmonary atresia fatal?

Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation.

Outlook for pulmonary atresia

Pulmonary atresia life expectancy varies depending on how severe your child’s condition is and other individual factors. Survival rates are better today than they were in previous decades.

About 60% to 85% of people who have a Fontan procedure (the last in the surgical series) are alive 20 years later. However, many have long-term complications. Some say a person who had a Fontan procedure and is now 35 years old has as many years of life left as someone who is already 72.

What is the survival rate of pulmonary atresia?

Without having surgery to fix pulmonary atresia with a ventricular septal defect, the survival rate is 50% at age 1 and 8% at 10 years of age. Most people don’t live into their 30s without surgery.

Can pulmonary atresia be cured?

No. Medical procedures and surgeries can improve your child’s condition, but they aren’t cures.

Living With

When should my child see their healthcare provider?

Your child will have regular follow-up appointments with their pediatric cardiologist. This starts two to four weeks after leaving the hospital. Some children may need more heart catheterization or surgeries as they grow older, or will need special medications.

People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. They may have appointments at least every six months.

Long-term complications may include:

Regular follow-up care can reduce or prevent these complications.

What questions should I ask my doctor?

  • What activities and sports will my child be able to do?
  • What’s the outlook for my child’s individual situation?
  • What’s the next step in my child’s treatment?

A note from Cleveland Clinic

Pulmonary atresia is a complex heart condition that can look different from person to person. Don’t be afraid to ask your child’s healthcare provider questions about your child’s specific situation. Once you bring your baby home from the hospital, be sure to keep taking them to their follow-up appointments with their cardiologist. These regular visits help your child’s provider catch any complications that may be developing.

Last reviewed by a Cleveland Clinic medical professional on 06/06/2022.

References

  • American Heart Association. Single Ventricle Defects. (https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/single-ventricle-defects) Accessed 6/6/2022.
  • Centers for Disease Control and Prevention. Facts about Pulmonary Atresia. (https://www.cdc.gov/ncbddd/heartdefects/pulmonaryatresia.html) Accessed 6/6/2022.
  • Chaix M, Khairy P. Single Ventricle Post Fontan Palliation: Tricuspid Atresia, Pulmonary Atresia, and Hypoplastic Left Heart Syndrome. In: Fuster V, Narula J, Vaishnava P, Leon MB, Callans DJ, Rumsfeld J, Poppas A. eds. Fuster and Hurst's The Heart, 15e. McGraw Hill; 2022. Accessed 6/6/2022.
  • Fukui D, Kai H, Takeuchi T, Gondo T, Oba T, Mawatari K, Tonai T, Matsuo Y, Ueda S, Niiyama H, Ueno T, Imaizumi T. Longest survivor of pulmonary atresia with ventricular septal defect: well-developed major aortopulmonary collateral arteries demonstrated by multidetector computed tomography. (https://pubmed.ncbi.nlm.nih.gov/22064959/) Circulation. 2011 Nov 8;124(19):2155-7. Accessed 6/6/2022.
  • National Heart, Lung, and Blood Institute. Congenital Heart Defects. (https://www.nhlbi.nih.gov/health-topics/congenital-heart-defects) Accessed 6/6/2022.
  • Pulmonary Atresia With Ventricular Septal Defect. In: Crawford MH, Aras M, Sanchez JM. eds. Quick Dx & Rx: Cardiology. McGraw Hill; 2018. Accessed 6/6/2022.

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