What Is Pulmonary Atresia

  • Ochuko Atenaga Second Class Upper Division, Psychology, University of Port Harcourt
  • Monica Yi Jin Yang BSc, Biomedical Sciences, GeneralBach, Imperial College London, UK
  • Alaa Soliman Master's in Health Care Administration/Management, Walden University, USA


Pulmonary atresia is a congenital heart disease where the valve that supplies blood to the lungs does not fully develop. This condition occurs when leaflets of blood valves fuse together during formation. As a result, they create a sheet of tissue instead of an opening.

The blood is the major form of transportation within the body. It carries nutrients from organs where they are formed to those that need it, as well as removing body waste from where they are generated to where they are expelled.

With the opening blocked, blood flow to the lungs is restricted. As a result, the lungs will not receive an adequate supply of oxygen. In turn, this could lead to respiratory issues and other complications. Considering that this condition results from a pre-birth defect, it is diagnosed at birth.

This article will enlighten readers on everything they need to know about pulmonary atresia. It will also empower them with ways to identify the disease and measures they can use to manage it.

For a better understanding of how critical this condition is, we need to examine the way the heart works. The heart takes in the air when people breathe in from their noses. From there, the respiratory system processes this air and obtains oxygen through the alveoli.

Veins transport oxygen-rich blood from the lungs to the heart, from where arteries carry them to cells and organs that require oxygen. The same process applies to the removal of waste like carbon dioxide from the body. Veins collect CO2 through the blood to the lungs, where they are exhaled via the node.

Looking at the entire process, it is obvious that veins and arteries have specific purposes. More so, each vein and artery are connected to specific parts of the heart. This arrangement is designed such that the circulatory system works efficiently. Considering the importance of human blood, a default to any artery or vein will be problematic.

This is why pulmonary atresia is a very significant issue. Worse still, it is a birth defect and affects children whose organs are not fully developed.

Types of pulmonary atresia

That said, there are two primary types of pulmonary atresia. Actually, the difference comes down to how much the ventricle is closed. 

Below is a breakdown of the different kinds of this heart disease:

Pulmonary atresia with ventricular septal defect

This is a scenario where the ventricular spectrum allows for blood flow in and out of the heart. The reason is that there is an opening called ductus arteriosus. Although this gap is natural, it often closes after the child is born. However, there are medications that can keep it open.

Besides the defective ventricular opening, the major aortopulmonary collateral artery that connects the heart to the lungs can be very small. Sometimes, this artery may even be missing. As a result, other arteries develop on the main artery to provide the lungs with blood and oxygen.

In this case, the right ventricular will easily maintain its size. This is a life-threatening situation, as children who suffer from this condition will have a lesser volume of oxygen in their blood. In turn, this may lead to respiratory problems or other complications,

Pulmonary atresia with intact ventricular septal

This condition is unlike PA with VSD because the ventricle is completely closed. The passage that connects the heart to the lungs receives very little blood flow. Sometimes, the right ventricle can be so small that it becomes unable to pump blood.

Other times, the absence of a passage may be because there is a thick ventricular wall. Another possible reason is that the child may have abnormal blood vessels other than the right ventricle, supplying blood to the heart.

Causes of pulmonary atresia

Like most birth-related heart defects, there are no certified causes of pulmonary atresia. Most often than not, each child’s case is different from others. As a result, it is very difficult to draw a line.

Besides, studying children before birth is more challenging than other research. This is because the child does not fully utilize his or her organ. The mother’s systems literally provide and support the baby. In this regard, the signs of pulmonary atresia are not very evident.

Signs and symptoms of pulmonary atresia

While the symptoms may not be evident before birth, they often show themselves after birth. Considering the importance of the circulatory system, a vigilant parent should notice the signs. In fact, they are noticeable within the first few hours after birth.

Some of them include the following:

  • Blue colouring of the baby’s skin
  • Abnormal breathing. This may either be difficult breathing or respiration via short gasps

Depending on the situation, some symptoms may not be immediately evident. They begin to manifest later on as the child grows.

They include:

  • Extreme levels of tiredness, even without any rigorous physical activity
  • Excessive sweating or tiredness while nursing from the mother
  • Continuous sleeping

Meanwhile, the doctor can also check the baby for any abnormalities. A good example is checking for a splashing sound within the heart. This sound indicates that there may be an irregular blood flow or a blockage.

However, this brings us back to the point outlined about identifying known causes. The whooshing sound is often absent at birth. Therefore, the simple stethoscope check may not be enough.

Management and treatment for pulmonary atresia

While there are no known causes of pulmonary atresia, there are still some risk factors. As such, scientists believe that avoiding these risk factors is a good way to prevent this heart disease from occurring in children.

Here are some tips to prevent this heart problem:

  • Maintain a healthy weight: One way to reduce the chances of having a child with PA is by properly managing your weight. This is crucial, considering that obesity is a risk factor
  • Regulate the use of routine drugs: Mothers who are on routine drugs should judiciously take them according to the prescription of a doctor. You should also discuss the risks and effects of such medications with a health expert
  • Lead a healthy lifestyle: Avoid harmful health habits like smoking, alcohol intake, etc, during the period of pregnancy. Research has shown that these behaviors can alter the genes and chromosomes of the baby

Sometimes, prevention is not enough. Despite the parent’s best, a child may still be born with the pulmonary atresia birth defect in such cases, the solutions often involve surgery.

In the case of a defect, the doctors may patch up the hole below the right ventricle. This will enable more efficient blood flow from the heart to the lungs. More so, there are certain medications that can help with treatment.

If the case is an intact ventricular septal, the child will require surgery. The surgeon will insert a catheter from the heart into the blood vessel. In addition to that, the doctor can expand the existing valve to improve blood flow.

The expansion of the valve can be done using a balloon. In addition, a small tube can be placed within the valve to keep it open. However, a child with an underdeveloped passage may require multiple operations.

Diagnosis of pulmonary atresia

Besides the signs and symptoms, there are proven methods that doctors can use to check for the presence of this medical condition. The most prominent test is known as echocardiogram. This is a type of ultrasound check that is done on a baby’s heart.

It creates an image depicting the structure of a baby’s heart. From the picture, doctors can easily identify if there are holes between the chambers. It can also help them to ascertain if there are any blockages.

Another method involves inserting a tube into the blood vessel. From there, the surgeon guides the tube to the child’s heart. Through this tube, the doctor can diagnose the blood pressure and oxygen levels. These details can share insights into any abnormality.

The electrocardiogram is also another device for making diagnosis. This method gauges the level of electrical activity within the heart. Doctors can combine it with other tests to give accurate diagnosis.

Risk factors

There are some possible situations that may put a child at risk of suffering from this heart disease. These behaviours listed above are capable of altering the child’s genes and increases the chances of this defect occurring.

Some of them include the following:

  • Either of the parents suffered from a congenital heart defect.
  • Use of certain medications by the mother during pregnancy. One of such drugs include those for high blood pressure
  • The mother did not properly manage her diabetes or suffers from obesity
  • Unhealthy habits from the mother like smoking during pregnancy


The presence of pulmonary atresia can lead to several complications.

These are some of the possible complications that individuals who do not treat pulmonary atresia may have:

  • Death
  • Heart failure
  • Irregular heart palpitations
  • Infection affecting the internal lining of the heart

Considering these complications, individuals with any form of heart problems are advised to visit a doctor. Even after undergoing surgery, those who suffered from this illness will need to check back on a regular basis.


How can I prevent pulmonary atresia?

You can prevent pulmonary atresia by maintaining a healthy lifestyle. Avoid smoking or abusing drugs. Speak with a health expert if you are on routine medication.

How common is pulmonary atresia?

Pulmonary atresia is a very rare congenital heart disease. Only 1 in every 7,000+ babies suffer from this medical condition.

Is pulmonary atresia fatal?

Pulmonary atresia can be fatal if left untreated. In addition, it can lead to complications like heart failure and bacterial infections.

Can pulmonary atresia be cured?

While a baby who suffered from pulmonary atresia can survive, it all depends on the severity of the condition. Even after treatment, the child may require regular visits to the doctor.

When should I see a doctor?

Checkups and diagnosis can be done immediately after birth. Nonetheless, parents should see a doctor if the child is always sleeping, often feeling very tired or has difficulty with eating.


Pulmonary atresia is a very rare heart condition. However, it can be fatal when a child suffers from it. There are no known causes of this disease, as the condition makes research almost impossible.

However, parents who are very diligent can quickly ascertain if their kids suffer from this congenital heart defect. There are several ways to diagnose, manage and treat pulmonary atresia. It all depends on the severity of that particular child.


  1. CDC. Congenital Heart Defects - Facts about Pulmonary Atresia | CDC. Centers for Disease Control and Prevention [Internet]. 2019 [cited 2024 Feb 7]. Available from: https://www.cdc.gov/ncbddd/heartdefects/pulmonaryatresia.html.
  2. Lato K, Gembruch U, Geipel A, Lachmann R, Schneider M, Hraska V, et al. Tricuspid atresia with absent pulmonary valve and intact ventricular septum: intrauterine course and outcome of an unusual congenital heart defect. Ultrasound Obstet Gynecol. 2010; 35(2):243–5.
  3. Babaoğlu K, Doğan Y, Erdem S, Özbarlas N, Başar E, Uzun O. Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome. Anatol J Cardiol. 2022; 26(9):702–9.CloseDeleteEdit
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Ochuko Atenaga

Second Class Upper Division, Psychology, University of Port Harcourt

My name is Ochuko, and I am a seasoned web content writer who simplifies the most complex ideas. For half a decade, I have written content on Blockchain, NFTs, Digital Marketing, eCommerce Development, G Health, Technology, Lifestyle, Tourism, Hospitality, and gambling. During this period, he has worked with top brands like Clario, CryptoGames3D, Amazix, AIKON, Progos Tech, Webzool, Zenith Chain, Jungle NFT Marketplace etc.

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