What is pulmonary atresia?
Pulmonary atresia is a heart defect present at birth (congenital) that’s normally diagnosed within the first few hours or days of life. In pulmonary atresia, the valve that lets blood out of the heart to go to your baby’s lungs (pulmonary valve) doesn’t form correctly.
Instead of opening and closing to allow blood to travel from the heart to the lungs, a solid sheet of tissue forms. So blood can’t travel by its normal route to pick up oxygen from the lungs. Instead, some blood travels to the lungs through other natural passages within the heart and its arteries.
These passages are necessary when your baby is developing in the womb and they normally close soon after birth. Babies with pulmonary atresia typically have a bluish cast to their skin because they aren’t getting enough oxygen.
Pulmonary atresia is a life-threatening situation. Procedures to correct your baby’s heart condition and medications to help your baby’s heart work more effectively are the first steps to treat pulmonary atresia.
How common is pulmonary atresia?
Pulmonary Atresia is a rare defect, occurring with equal frequency in boys and girls, and is related to the congenital heart defect known as Tetralogy of Fallot. Please discuss with your doctor for further information.
What are the symptoms of pulmonary atresia?
The common symptoms of pulmonary atresia are:
- Blue- or gray-toned skin (cyanosis)
- Fast breathing or shortness of breath
- Easily tiring or being fatigued
- Feeding problems, such as tiring or sweating while eating
- Pale, clammy skin that may feel cool to the touch
If your baby is born with pulmonary atresia, symptoms will be noticeable soon after birth. Pulmonary atresia symptoms may show up within hours to several days.
There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.
When should I see my doctor?
If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.
What causes pulmonary atresia?
As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is linked with another type of congenital heart defect called a patent ductus arteriosus (PDA).
Pulmonary atresia may occur with or without a ventricular septal defect (VSD).
If the person does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS).
If the person has both problems, the condition is called pulmonary atresia with VSD. This is an extreme form of tetralogy of Fallot.
Although both conditions are called pulmonary atresia, they are actually different defects. This article discusses pulmonary atresia without a VSD.
Persons with pulmonary atresia with intact ventricular septum may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart.
What increases my risk for pulmonary atresia?
There are many risk factors for pulmonary atresia, such as:
- A mother who had German measles (rubella) or another viral illness during early pregnancy
- A parent who has a congenital heart defect
- Drinking alcohol during pregnancy
- Smoking before or during pregnancy
- A mother who has poorly controlled diabetes
- A mother who has lupus, an autoimmune disorder
- Use of some types of medications during pregnancy, such as the acne drug isotretinoin (Claravis, Amnesteem, others), some anti-seizure medications and some bipolar disorder medications
- The presence of Down syndrome, a genetic condition that results from an extra 21st chromosome
Diagnosis & treatment
The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.
How is pulmonary atresia diagnosed?
Tests to diagnose pulmonary atresia may include:
- X-ray. An X-ray shows the size and shape of your child’s internal tissues, bones and organs. This can help your baby’s doctor see the extent of your baby’s pulmonary atresia.
- Electrocardiogram (ECG). In this test, sensor patches with wires attached (electrodes) measure the electrical impulses given off by your child’s heart. This test detects any abnormal heart rhythms (arrhythmias or dysrhythmias) and may show heart muscle stress.
- In an echocardiogram, sound waves create detailed images of your child’s heart. Your child’s doctor usually uses an echocardiogram to diagnose pulmonary atresia. Your doctor may diagnose your baby’s pulmonary atresia through an echocardiogram of your abdomen before you deliver your baby (fetal echocardiogram).
- Cardiac catheterization. In this test, your child’s doctor inserts a thin, flexible tube (catheter) into a blood vessel in your child’s groin and guides it to your child’s heart using X-ray imaging. This test provides detailed information about your child’s heart structure and the blood pressure and oxygen levels in your child’s heart, pulmonary artery and aorta. Your child’s doctor may inject a special dye into the catheter to make the arteries visible under X-ray.
How is pulmonary atresia treated?
Most babies with pulmonary atresia will need medication to keep the ductus arteriosus open after birth. Keeping this blood vessel open will help with blood flow to the lungs until the pulmonary valve can be repaired.
Treatment for pulmonary atresia depends on its severity.
In some cases, blood flow can be improved by using cardiac catheterization (inserting a thin tube into a blood vessel and guiding it to the heart). During this procedure, doctors can expand the valve using a balloon or they may need to place a stent (a small tube) to keep the ductus arteriosus open.
In most cases of pulmonary atresia, a baby may need surgery soon after birth. During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery. If a baby has a ventricular septal defect, the doctor also will place a patch over the ventricular septal defect to close the hole between the two lower chambers of the heart. These actions will improve blood flow to the lungs and the rest of the body. If a baby with pulmonary atresia has an underdeveloped right ventricle, he or she might need staged surgical procedures, similar to surgical repairs for hypoplastic left heart syndrome.
Most babies with pulmonary atresia will need regular follow-up visits with a cardiologist (a heart doctor) to monitor their progress and check for other health conditions that might develop as they get older. As adults, they may need more surgery or medical care for other possible problems.
Lifestyle changes & home remedies
What are some lifestyle changes or home remedies that can help me manage pulmonary atresia?
The following lifestyles and home remedies might help you cope with pulmonary atresia:
- Strive for good nutrition. Your baby may have a difficult time taking in enough calories, both because he or she tires more easily during feeding and because of an increased demand for calories. It’s often helpful to give your baby frequent, small feedings.
- Preventive antibiotics. Your child’s cardiologist will likely recommend that your child take preventive antibiotics before certain dental and other procedures to prevent bacteria from entering the bloodstream and infecting the inner lining of the heart (infective endocarditis). Practicing good oral hygiene — brushing and flossing teeth, getting regular dental checkups — is another good way of preventing infection.
- Help your child stay active. Encourage as much normal play and activity as your child is able to tolerate, with ample opportunity for rest and nap time. Staying active helps your child’s heart stay fit. As your child grows, talk with the cardiologist about which activities are best for your child. If some are off-limits, such as competitive sports, encourage your child in other pursuits rather than focusing on what he or she can’t do.
- Keep up with routine well-child care. Standard immunizations are encouraged for children with congenital heart defects, as well as vaccines against the flu, pneumonia and respiratory syncytial virus infections.
- Keep regular follow-up appointments with your child’s doctor. Your child may need regular annual appointments with his or her doctor trained in congenital heart conditions, even throughout his or her adult life.
If you have any questions, please consult with your doctor to better understand the best solution for you.
Hello Health Group does not provide medical advice, diagnosis or treatment.
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Facts about Pulmonary Atresia. https://www.cdc.gov/ncbddd/heartdefects/pulmonaryatresia.html. Accessed December 6, 2017.
Pulmonary atresia. https://medlineplus.gov/ency/article/001091.htm. Accessed December 6, 2017.
Pulmonary Atresia. http://www.pted.org/?id=pulmonaryatresia1. Accessed December 6, 2017.
Review Date: December 4, 2017 | Last Modified: December 6, 2017