Pulmonary atresia is a congenital birth defect of the heart. In this condition the pulmonary valve is absent. Its absence leads to trouble in flow of blood from the right ventricle of heart to the lungs. In a normal person the valve is located in pulmonary artery and it controls blood flow from heart to the lungs for oxygenation of blood.
As the blood cannot flow from right ventricle to pulmonary artery, it takes various other natural routes within the heart for the flow of blood to the lungs. These passages such as a hole called foramen ovale between the right and left upper chamber or passage between the aorta and pulmonary artery normally closes soon after birth.
But in pulmonary atresia it remains open and allows some amount of oxygen poor blood from the right atrium to mix with oxygen rich blood of left atrium. Ultimately the mixed blood is pumped out from the left ventricle to rest of the body through aorta. Since the blood pumped out to the body is not fully oxygenated, the child may have several problems unless he is treated. Pulmonary atresia is considered critical congenital birth defect of the heart. If not treated pulmonary atresia can be fatal.
Causes Of Pulmonary Atresia
The etiology of pulmonary atresia is not known.Often children have other types of heart defects along with pulmonary atresia. Several factors are known to increase the risk of heart defect including pulmonary atresia in the baby. Pulmonary atresia occurs in 1 out of 10000 babies. Both males and females are equally affected. These risk factors are as follows:
- German measles and viral diseases during pregnancy.
- Mother suffering from poorly controlled diabetes.
- One or both parents suffering from congenital heart ailment.
- Drinking alcohol during pregnancy.
- Mother suffering from autoimmune diseases.
- Consumption of certain medicines during pregnancy. Use of medicines for acne and anti epileptics during pregnancy.
- Child suffering from certain congenital diseases such as Down’s syndrome.
Symptoms Of Pulmonary Atresia
The symptoms of most of the heart defect are noticed soon after birth of the child. The symptoms in case of pulmonary atresia develop soon after birth of the child or may develop as the patent ductus arteriosus closes. The sign and symptoms of pulmonary atresia include:
- Bluish discoloration of skin. This condition in medical terminology is called cyanosis. This occurs due to mixing of oxygenated blood with poor oxygenated blood.
- Shortness of breath.
- Rapid pulse and heart rate.
- The infant gets easily tired while breastfeeding.
- The skin is cold and clammy to touch.
Diagnosis And Treatment For Pulmonary Atresia
Pulmonary atresia can be diagnosed during the routine ultrasound examination of the pregnant female. It can also be diagnosed after the baby is born when the doctor examines the infant with his stethoscope for any heart defects.
Following other investigation procedures will help in diagnosing pulmonary atresia:
- 2 D Echocardiogram
- Electrocardiogram (ECG)
- Pulse oximetry
- Chest X-ray
- Cardiac catheterization
A child born with heart defect such as pulmonary atresia needs immediate medical attention since the condition can be fatal if not treated. Treatment may depend on the severity of the symptoms. Medicines and surgery are two modes of treatment.
- Medicines: The child may be given intravenous medicine that helps to prevent closure of patent ductus arteriosus. It is a connection between aorta and pulmonary artery. However, this is not a permanent solution. It only provides some amount of time for the surgeon to determine the type of surgery or procedure that needs to be done.
- Certain procedures through cardiac catheterization are also helpful in case of pulmonary atresia. Procedures such as balloon valvotomy, placement of stent, balloon atrial septostomy are some of the procedures that will help in correcting the defect.
- After a period of time most the child may need surgical repair to increase the blood flow to lungs from pulmonary artery. A series of operation over a period of time may be necessary for rerouting the flow of blood to lungs. Usually the surgical repair is done soon after birth and final procedure is done after one year or two years.
Even after surgery the child may need to take antibiotics whenever necessary as a precautionary measure to prevent endocarditis. The child has to restrict doing exertional physical activities.
Complications And Prognosis Of Pulmonary Atresia
Pulmonary atresia is a fatal condition if not treated. Even after medications and surgery, the child has to be monitored regularly for any changes in his system. There is always risk of infection in the valves if there is any type of structural defect in heart. It also applies to pulmonary atresia. Patient has greater risk of developing endocarditis infection. It is an infection caused by bacteria. The child may also have delay in his development. Often the child needs special care as he grows up.
Prognosis: The prognosis of child with pulmonary atresia is dismal unless he is treated. Even after treatment the child may need extra care as he grows. However, after few heart operations he is able to live but may have to follow regular checkups with his doctor for life time.