Pulmonary Atresia With Intact Ventricular Septum

Pulmonary Atresia with Intact Ventricular Septum (PA/IVS)

Pulmonary atresia is a rare birth defect where the valve between the heart and lungs (the pulmonary valve) doesn't develop properly. This means blood can't flow from the right lower heart chamber (the right ventricle) to the lungs. Babies with this condition don't get enough oxygen in their blood.

In some cases, there's a natural connection (ductus arteriosus) between the main artery carrying oxygenated blood out of the heart (aorta) and the pulmonary artery. This connection usually closes after birth, but sometimes it needs to be kept open with medicine to help the baby get more oxygen.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a type where there's no hole between the two main pumping chambers of the heart. If there is a hole, it's called pulmonary atresia with ventricular septal defect (VSD).

Because of the lack of oxygen, immediate treatment is crucial. Treatment usually involves a combination of medications, procedures, and surgeries to improve blood flow and repair the heart.

Diagnosing PA/IVS:

Doctors use several tests to diagnose PA/IVS:

• Pulse oximetry: A painless sensor measures the oxygen level in the blood.
• Electrocardiogram (ECG/EKG): This measures the heart's electrical activity, showing how the heart beats. Sticky sensors (electrodes) are placed on the chest, arms, and legs.
• Chest X-ray: This shows the shape and size of the heart and lungs.
• Echocardiogram: Sound waves create moving pictures of the heart, showing how blood flows through the heart and valves, including the pulmonary valve. A fetal echocardiogram can detect PA/IVS during pregnancy.
• Cardiac catheterization: A thin tube (catheter) is inserted into a blood vessel (usually in the groin), guided to the heart. Dye is used to make the heart's arteries show up better on X-rays.

Treatment for PA/IVS:

Treatment for PA/IVS typically involves medications, procedures, or surgeries. The goal is to improve blood flow to the lungs and repair the heart. Treatment often starts soon after birth. The specific approach depends on factors like the size of the heart chambers and valves, and whether the baby has other heart problems.

• Medications: Medications might be given through an IV to keep the ductus arteriosus open. This is temporary, allowing time for more permanent solutions.
• Procedures:
  • Balloon valvotomy: A balloon is used to widen a narrowed pulmonary valve opening.
  • Balloon atrial septostomy: A balloon widens a small hole (foramen ovale) in the wall between the heart's upper chambers to improve blood flow to the lungs.
  • Stenting: A small, rigid tube (stent) is placed in the ductus arteriosus to keep it open.
  • Shunting: A new path (bypass/shunt) is created to direct blood to the lungs. Examples include the Blalock-Taussig shunt.
  • Glenn procedure: A large vein is connected to the pulmonary artery to help the right ventricle grow. This is often done when the baby is 4-6 months old.
  • Fontan procedure: A new path is created to direct most or all of the blood flowing to the heart into the pulmonary artery. This procedure is usually performed when the child is 2-3 years old.
  • Hybrid procedures: Combining surgical and catheter treatments, sometimes performed without a heart-lung machine.

Long-Term Care:

After treatment, regular checkups with a pediatric cardiologist are essential. Many people with PA/IVS live long and healthy lives into adulthood. Adults with PA/IVS should be under the care of a doctor specializing in adult congenital heart disease.

Pulmonary atresia is typically discovered shortly after a baby is born. Doctors use various tests to assess the baby's heart health.

Several tests can help diagnose pulmonary atresia:

• Pulse oximetry: This test measures the amount of oxygen in the baby's blood. A small sensor, like a sticker, is placed on a fingertip. Low oxygen levels can signal a problem with the heart or lungs.

• Chest X-ray: An X-ray of the chest shows the size and shape of the heart and lungs. It can help doctors see if there are any unusual features.

• Electrocardiogram (ECG or EKG): This painless test records the heart's electrical activity. Sticky electrodes (small pads) are placed on the chest, and sometimes the arms and legs. These electrodes are connected to a machine that records the heart's rhythm and patterns. The results show how well the heart is beating.

• Echocardiogram: This is usually the most important test for diagnosing pulmonary atresia. Sound waves are used to create a picture of the heart's structure and how the heart chambers and valves work. This allows doctors to see how blood flows through the heart. If this test is done before the baby is born, it's called a fetal echocardiogram.

• Cardiac catheterization: In this test, a thin, flexible tube (catheter) is inserted into a blood vessel in the arm or groin and guided to an artery in the heart. Special dye is injected through the catheter. The dye makes the heart's arteries show up more clearly on X-rays. This gives detailed information about blood flow and how the heart functions. Doctors can sometimes perform specific heart treatments during a cardiac catheterization procedure.

Babies born with pulmonary atresia, a condition where the pulmonary artery is too narrow or closed, need immediate medical attention. The best course of action depends on how severe the problem is.

Sometimes, doctors give medicine through an IV to keep a blood vessel (ductus arteriosus) open. This helps buy time for figuring out the best surgery or procedure. It's not a long-term fix, but it's crucial for giving the medical team more time to decide on the right approach.

One way to treat pulmonary atresia is with a thin, flexible tube called a catheter. Doctors insert this tube into a large blood vessel in the baby's groin and guide it to the heart. These catheter procedures can include:

• Balloon atrial septostomy: A balloon is used to widen a small opening (foramen ovale) between the heart's upper chambers. This opening usually closes after birth, but widening it allows blood to flow more easily from the right to the left side of the heart.
• Stent placement: A small, rigid tube (stent) is placed in the ductus arteriosus to keep it open and ensure blood reaches the lungs.

Babies with pulmonary atresia often need multiple heart surgeries over time. The specific type of surgery depends on the size of the baby's heart chambers and pulmonary arteries.

Different surgical options for pulmonary atresia include:

• Shunting: A new pathway (bypass shunt) is created to direct blood from the main artery (aorta) to the pulmonary arteries. This helps get blood to the lungs, but most babies eventually outgrow this shunt.
• Glenn procedure: A large vein that carries blood back to the heart is connected to the pulmonary artery. Another vein continues to bring blood to the right side of the heart, which then pumps it through the repaired pulmonary valve. This helps the right side of the heart grow stronger.
• Fontan procedure: If the right lower heart chamber is too small, this procedure creates a new path for most of the blood returning to the heart to flow directly into the pulmonary artery.
• Heart transplant: In some severe cases, the heart is too damaged to be repaired, and a heart transplant may be necessary.

If a baby with pulmonary atresia also has a hole in the wall between the heart's lower chambers (ventricular septal defect or VSD), surgeons typically repair the hole and create a connection between the right pumping chamber and the pulmonary artery. Sometimes, an artificial valve is used in this repair.

Taking care of a child with pulmonary atresia after leaving the hospital involves several key steps.

Regular Checkups are Crucial: Your child needs ongoing medical checkups, even as they grow into adulthood. A doctor specializing in birth defects of the heart (a congenital cardiologist) is often the best person to manage their care. Make sure they get all recommended vaccines, including the yearly flu shot.

Managing Exercise and Activity: Some children with congenital heart conditions might need to limit sports or strenuous exercise. Others can participate in normal activities. Talk to your child's healthcare team to discuss what's safe and appropriate for their specific condition. They'll be able to give you personalized advice.

Good Oral Hygiene is Important: Keeping teeth clean, brushing and flossing regularly, and attending regular dental checkups is vital to prevent infections. This is true for everyone, but especially important for children with heart conditions.

Preventive Antibiotics Might Be Needed: A congenital heart defect can sometimes increase the risk of a serious infection in the heart's lining or valves, called infective endocarditis. In some cases, your child's doctor may recommend taking antibiotics before dental procedures to prevent this infection. This is particularly important if your child has an artificial heart valve.

Finding Support is Helpful: Connecting with other parents whose children have similar heart conditions can offer valuable comfort and support. Ask your child's healthcare team about local support groups or online communities. Knowing you're not alone can make a big difference.