Pulmonary Atresia -Procedures, Side effects, Recovery time, Costs and alternatives

It is a heart disease in which the pulmonary valve fails to develop properly. This is a congenital disorder, which means it is present from birth and can be diagnosed before birth, or within a few hours after the child is born. Pulmonary valves are responsible for transporting blood from heart to the pulmonary artery which then goes into the lungs. Then the blood gets oxygenated and transported back to the heart to be pumped to the rest of the body. But in the case of infants suffering from this condition, the pulmonary valve is usually blocked so the heart cannot send blood to the lungs. As a result, the infant’s body will lack proper oxygenated blood which can be life-threatening.

There are no definite causes for this disorder, and any new born baby can develop this defect. Genetics and heredity may sometimes lead to this condition, but it cannot be considered as a major cause. Another risk factor is if the expecting mother gets involved in habits like smoking and drinking alcohol before or during pregnancy. Sometimes, certain medications taken during pregnancy may also contribute to the child developing this condition.

The most prominent symptom of this disease is cyanosis, i.e., bluish colour of the skin. This is due to the lack of oxygen in the blood, as deoxygenated blood is blue in colour. Other signs and symptoms include fatigue, shortness of breath, and getting tired quickly while feeding. Symptoms may show up after a few hours of birth or it may also take a few days to become noticeable.

Treatment for this disorder focuses mainly on providing an alternate passage for the blood to reach the lungs from the heart, since the pulmonary valves are blocked or restricted.

Diagnosis will be done by the doctor upon observing the symptoms. It’s important to know that the exact methods of diagnosis and treatment may differ as per the affected child’s condition. But the following tests are usually conducted during diagnosis:

Sometimes, the child may be diagnosed before birth. If so, then doctors prepare accordingly to make arrangements for the child to be delivered safely. Most times, the condition is detected within a few hours of the birth. If a child is confirmed to have pulmonary atresia, then the doctor will decide what is the best course of treatment. Some methods like medications or catheterisation can help provide short-term relief, but surgery will be required to fix the problem for long-term. These are the major surgical procedures:

• Blalock-Taussig shunt: This is an operation where a shunt (artificial passage) is created between the aorta and the pulmonary artery. So even if the pulmonary valve is blocked, some blood from aorta can flow to the pulmonary artery through the shunt and finally reach the lungs. An important point to consider is that the child will outgrow the shunt after some months, so it usually does not provide a permanent solution. This operation is usually performed within a short time span after birth..
• Glenn Procedure: In this procedure, a large vein that brings deoxygenated blood back to the heart is connected surgically to the pulmonary artery instead. Thus, it becomes possible for the deoxygenated blood to reach the lungs. It also helps the right ventricle to grow in size. Glenn procedure is generally performed a few months after the birth..
• Fontan Procedure: This operation is normally conducted when the child is of 2 years or above, though there are exceptions. It is required if the right ventricle is small and underdeveloped. In this procedure, most (or all) blood coming to the heart from other regions of the body is directed straight into the pulmonary artery, thus completely bypassing the heart..
• Heart transplant: In some cases, if the heart is not repairable then a heart transplant may be suggested. But the chances of requiring a heart transplant is very rare. Pulmonary atresia and its development and aftermath are influenced by many factors, so either one or more of the above mentioned surgical procedures may be required for permanent treatment.

Any new born infant diagnosed with this condition is eligible for the treatment. However, there are multiple methods to provide treatment, and the doctor/cardiologist will accordingly decide the appropriate procedure. Since this condition is diagnosed early after birth, the treatment will be initiated immediately.

If some other medical conditions are also present in a child having pulmonary atresia, then immediate surgical treatment may be temporarily avoided. The surgery may not be possible if the child is not very healthy physically. But there are additional methods and stop gap measures to handle such cases, until it becomes possible to go for surgery.

Pulmonary atresia and its treatment have very few side effects, which are listed below:

• Anaesthetic side effects: It is common after most surgeries that make use of anaesthesia.
• Delay in growth and development: Children with pulmonary atresia tend to grow slower than healthy children.
• Risk of blood clotting and heart stroke
• Irregular heartbeat
• Chances of infection such as endocarditis

There are two stages of post-treatment guidelines for children who undergo surgery for pulmonary atresia. The first stage is immediate post-operative care, usually provided at the hospital until the child is in normal condition. The second stage is the long term care that parents must provide to ensure the child has a healthy growth and can live life free from complications. Both stages are discussed below.

1. Post-operative care The child will be kept in the ICU after surgery for observation. Here, the medical professionals will handle the situation, and some equipments will be used such as a ventilator to assist with breathing, or a heart monitor to observe the vitals of the heart. The stitches will be removed within a week or two at most.
2. Long-term care After the child is discharged and free to go home, a lot of care must be given to maintain the proper growth of the child. The first step is to maintain a regular follow up with the doctor to monitor the child’s condition. Medications and diet will also be advised by the doctor and should be followed strictly. The child can be involved in normal activities as they grow, but timely check-ups must be done to ensure all is well. Other than that, parents need to make sure that the child gets good nutrition, and has a healthy and active lifestyle.

Since this disease affects children, the process of recovery after surgery will vary greatly. Also, in most cases, the process of surgery itself can take a few years because many children need to undergo two or three surgeries over time. If multiple surgeries are to be performed, the child may have to stay in the ICU for a few weeks after each surgery. Complete recovery after each surgery may also take months.

The overall treatment procedure for pulmonary atresia varies among every individual case, and so does the cost. Whether the child requires single or multiple surgeries, the location of the hospital where surgery is to be performed, extra services and tests that need to be performed, and other similar factors will affect the cost of treatment. As such, it is not possible to estimate even an average price range for the treatment. Even if treatment requires just a single surgery, the minimum expense will go above the range of 1 lakh.

More than 90% of children who successfully complete the surgical treatment for pulmonary atresia go on to live normal and healthy lives. The condition in itself is life-threatening, but the treatment is certainly effective and the results are permanent in majority of cases.

Surgery is the only possible long-term treatment for pulmonary atresia. But there are some alternate methods which may be required before surgery is possible, which are as listed below.

• In the foetal stage, a developing baby has a blood vessel called ductus arteriosus that serves as a connection between the aorta and pulmonary artery. This duct closes after birth, but an IV medication called prostaglandin E1 can keep the ductus arteriosus open after birth, which allows blood to reach the lungs for some time period after birth and diagnosis.
• A type of catheterization process called balloon atrial septostomy is also used sometimes. In this process, a balloon is introduced to the heart with the help of a catheter. This balloon enlarges a hole called foramen ovale which lies in between the left and right atrium. Foramen ovale also provides a similar function as the ductus arteriosus, and closes after birth. Keeping it open after birth helps blood to be oxygenated for a period of time. It is important to know that these methods function as temporary procedure to buy more time until a permanent surgical treatment is decided upon.