Pulmonary atresia with intact ventricular septum

Symptoms of pulmonary atresia with intact ventricular septum, also called PA/IVS, may appear soon after birth. They can include:

• Blue or gray skin. Changes may be harder or easier to see depending on the baby’s skin color.
• Fast breathing or shortness of breath.
• Tiredness.
• Poor feeding.

Pulmonary atresia with intact ventricular septum, also called PA/IVS, is often found during pregnancy or soon after birth. If your baby has symptoms of PA/IVS after you’ve left the hospital, call a healthcare professional right away.

Babies with pulmonary atresia need treatment right away. It’s best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart conditions present at birth.

Treatment for pulmonary atresia with intact ventricular septum, also called PA/IVS, may include medicines and one or more procedures or surgeries.

Medications

Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it keeps the baby stable until a surgery or procedure can be done to permanently fix blood flow.

Surgeries or other procedures

A baby with pulmonary atresia with intact ventricular septum needs one or more surgeries or procedures to improve blood flow and fix the heart.

Some of these treatments are done in the first days to weeks of a baby’s life. Others are done later. The type of surgery or procedure depends on many things. These include the size of the right lower heart chamber and heart valves and whether the baby has other heart conditions.

Treatments in the first days to weeks of life

• Balloon valvotomy. This treatment helps open the pulmonary valve. The doctor inserts a long, thin tube called a catheter into a blood vessel in the baby’s groin and guides it to the heart. A balloon on the tip of the catheter is inflated to widen the valve opening. The balloon is deflated, and the catheter and balloon are removed.
• Stenting. The doctor places a rigid tube called a stent in the natural opening between the aorta and pulmonary artery. This opening is called the ductus arteriosus. It usually closes soon after birth. Keeping it open lets blood go to the lungs.
• Shunting. This surgery uses a tube to create a new path for blood to flow to the pulmonary arteries. The tube is called a shunt. An example of this treatment is the Blalock-Taussig shunt procedure or RV-PA shunt. Once this is done, medicine is not needed to keep the ductus arteriosus open. Babies eventually outgrow this shunt.

Surgeries done later

• Glenn operation. This surgery most often is done between 4 and 6 months of age. The surgeon connects a large vein, called the superior vena cava, to the pulmonary artery. Another large vein keeps blood flowing to the right side of the heart. If this surgery is done without using a heart-lung machine, it’s called an off-pump Glenn procedure.
• 1.5 ventricle repair. This surgery may be done in babies with PA/IVS who have a relatively well-formed lower right heart chamber. A surgeon creates a pathway from the lower right heart chamber to the pulmonary artery. The Glenn procedure also is done. The term “1.5 ventricle” means that the lower right heart chamber is doing some but not all of the heart’s work.
• Fontan procedure. If the lower right lower heart chamber stays too small to do its work, surgeons may make a new path for blood to flow. The pathway lets most, if not all, of the blood coming to the heart to go into the pulmonary artery. The Fontan procedure is most often done when a child is 3 or 4 years old.
• Hybrid procedures. These are surgical and catheter treatments that are done at the same time. They’re sometimes done without a heart-lung machine.

After treatment, babies with pulmonary atresia with intact ventricular septum, also called PA/IVS, should have regular health checkups with a heart doctor trained to treat children. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with PA/IVS live into adulthood. Adults with the condition should be followed by a doctor with training in adult congenital heart diseases.

Here are some tips for caring for someone with pulmonary atresia after coming home from the hospital:

• Go to scheduled health checkups. A person born with pulmonary atresia needs regular checkups, even as an adult. A doctor trained in congenital heart diseases, called a congenital cardiologist, often provides care. Get recommended vaccines, including yearly flu vaccines.
• Ask about exercise and activity. Some children with a congenital heart defect may need to limit exercise or sports activities. However, many others with a congenital heart defect can participate in such activities. Your child’s care team can tell you which sports and types of exercise are safe for your child.
• Practice good oral hygiene. Brushing and flossing teeth and getting regular dental checkups can help prevent infection.
• Ask about preventive antibiotics. Sometimes, a congenital heart defect can increase the risk of infection in the lining of the heart or heart valves. This infection is called infective endocarditis. Antibiotics may be recommended before dental procedures to prevent infection, especially for people who have a mechanical heart valve.

Your baby is likely to be diagnosed with pulmonary atresia soon after birth while still in the hospital. Then you’ll be referred to a doctor trained in heart diseases, called a cardiologist, for ongoing care.

Here’s some information to help you get ready for your appointment.

What you can do

When you make the appointment, ask if there’s anything you need to do before you go. For instance, you might need to fill out forms or restrict your child’s diet. For some imaging tests, your child may need to not eat or drink for a time before the tests.

Take a family member or friend to the appointment, if possible. This person can help you remember the details you’re given.

Make a list of:

• Your child’s symptoms, including ones that don’t seem linked to pulmonary atresia. Try to recall when you noticed them.
• Key personal facts, including family history of congenital heart defects, pulmonary hypertension, or other heart or lung diseases.
• All medicines, vitamins, or supplements that your child takes and the doses. Also list medicines you took while pregnant.
• Questions to ask your child’s healthcare professional.

For pulmonary atresia, some questions to ask include:

• What are other possible causes for my child’s symptoms or condition?
• What tests will my child need?
• What’s the best treatment?
• What other treatments are there?
• Are there activities my child should not do?
• How often should my child be screened for changes?
• Can you suggest a specialist who treats congenital heart defects?
• Are there brochures or other printed material that I can have? What websites do you suggest?

Be sure to ask all the questions you have about your child’s condition.

What to expect from your child’s doctor

Be ready to answer questions, such as:

• Has anyone else in your family been diagnosed with pulmonary atresia or another congenital heart defect?
• Does your child always have symptoms or do the symptoms come and go?
• How bad are the symptoms?
• What, if anything, seems to make the symptoms better?
• What, if anything, seems to make the symptoms worse?