Description

Pulmonary Atresia (uh-TREE-zhuh) is a problem of the heart present at birth. That means that it is a congenital defect of the heart. In this condition, the valve that helps to move the blood from the heart to the lungs doesn't form properly. The valve is called the pulmonic valve.

In place of a valve that opens and closes, a solid sheet of tissue. As well, blood can't travel to their usual route to get the oxygen from the lungs. Instead, the part of blood that travels to the lungs through other passages within the heart and its arteries.

A baby in the womb of the needs of these other passages. But usually closes shortly after birth.

Pulmonary atresia is a life-threatening condition that needs emergency treatment. The treatment includes surgery to repair the heart and medicines to help the heart work better.

Symptoms

The symptoms of pulmonary atresia can be seen shortly after birth. These may include:

  • The blue or the gray skin, the lips or fingernails due to low levels of oxygen. Depending on the color of the skin, these changes may be more difficult or more easy to see.
  • Rapid breathing or difficulty breathing.
  • Tire easily.
  • Not feeding well.

When to see a doctor

Pulmonary atresia is most often found shortly after birth. If your baby has symptoms of pulmonary atresia, after having left the hospital, get medical help right away.

Causes

The cause of the pulmonary atresia is not clear. During the first six weeks of pregnancy, the baby's heart begins to form and starts to beat. The major blood vessels that run to and from the heart also begins to develop during this critical time. It is at this point in the development of the baby to a congenital defect of the heart, such as pulmonary atresia may begin to develop.

To understand how the pulmonary atresia occurs, it can be useful to know how the heart works.

How the heart works

The typical heart consists of four chambers. There are two upper chambers, called atria, and two lower chambers, called ventricles.

The right side of the heart moves the blood to the lungs. In the lungs, the blood picks up oxygen and then returns to the heart from the left side. The left side of the heart pumps blood through the body's main artery, called the aorta. The blood goes to the rest of the body.

In pulmonary atresia, the pulmonary valve does not form as usual, and therefore cannot be opened. The blood can't flow from the right ventricle to the lungs.

Before the birth, not to have an opening of the pulmonary valve does not affect the oxygenation of the baby. That is because the baby gets oxygen from the tissue that connects the baby to the breast, called the placenta. The oxygen-rich blood from the placenta goes to the baby's right upper chamber of the heart.

The blood goes to the right side of the baby's heart, then passes through a hole between the upper chambers of the baby's heart. The hole is called the foramen ovale. This allows oxygen-rich blood to move the rest of the baby's body through the aorta.

After birth, the lungs are necessary for oxygen. In pulmonary atresia, without a job of the pulmonary valve, the blood must find another way of getting to the lungs of the baby.

The blood from the right side of the heart can cross the foramen ovale to the left side of the heart. From there it can be pumped to the aorta. Newborns have a temporary opening, called the ductus arteriosus, between the aorta and the pulmonary artery. This opening allows part of the blood that travel to the lungs. There, the blood picks up oxygen to send to the rest of the baby's body.

The ductus arteriosus is most often closes shortly after birth. But medicines can keep it open.

Sometimes there is a second hole in the tissue between the main pumping chambers of the baby's heart. This hole is a ventricular septal defect (VSD).

The VSD allows the blood to flow from the right lower heart chamber to the lower left chamber of the heart. People with pulmonary atresia and ventricular septal defect often have other changes with the lungs and the arteries that carry blood to the lungs.

If there is a ventricular septal defect , the right lower chamber of the heart is little blood flow before birth. The camera often does not completely form. This is a condition called pulmonary atresia with intact ventricular septum (PA/IVS).

Risk factors

Pulmonary atresia occurs because the baby's heart is forming during pregnancy. Certain health conditions or the use of illicit drugs during pregnancy may increase the baby's risk of pulmonary atresia or other congenital heart defects. The risk factors include:

  • Obesity.
  • Alcohol, or tobacco.
  • Diabetes.
  • The use of some types of medications during pregnancy, such as certain acne and high blood pressure medicines.

Some types of congenital heart defects occur in families. This means that they are inherited. If you or someone in your family was born with a heart problem, including pulmonary atresia, ask your care team is the genetic testing is right for you. Screening can help to show the risk of certain birth defects of the heart in the future of children.

Complications

Without treatment, pulmonary atresia with most often leads to death. After the surgery, pulmonary atresia, babies need regular check-ups throughout their lives to see for complications.

Complications of pulmonary atresia may include:

  • Bacterial infection of the inner wall of the heart and its valves, called infective endocarditis.
  • Irregular heartbeats, called arrhythmias.
  • The weakening of the function of the heart.

Prevention

It might not be possible to avoid pulmonary atresia. But to get good prenatal care is important. Some things you can do before or during pregnancy may help lower your baby's risk of birth defects of the heart. They include:

  • Control of other health conditions. If you have diabetes, keep your blood sugar under control. For other conditions that require medication, talk with your health care professional about taking these medications during pregnancy.
  • I do not smoke and not be around people who smoke. If you smoke, stop smoking. Smoking during pregnancy increases the risk of congenital heart defects in the baby.
  • Aim for a healthy weight. Obesity increases the risk of having a baby with a congenital heart defect.
  • Vaccination is recommended. Have rubella, also called German measles during pregnancy can cause problems with a baby's heart development. A blood test before pregnancy can determine if you are immune to rubella. A vaccine is available for those who are not immune.

Diagnosis

Pulmonary atresia is usually diagnosed shortly after birth. Tests are done to check the baby's heart health.

Tests

Tests to diagnose pulmonary atresia may include:

  • Pulse oximetry. A sensor that is placed at the tip of the fingers of the records of the amount of oxygen in the blood. Very little oxygen can be a sign of a heart or lung problem.
  • X-ray of the chest. A chest x-ray shows the size and shape of the heart and lungs.
  • Electrocardiogram (ECG or EKG). This quick and painless test records the electrical activity of the heart. It is shown how the heart is beating. Sticky patches called electrodes are put on the chest and sometimes the arms and legs. The cables connect the patches to a computer, that prints or displays the results.
  • The echocardiogram. This test uses sound waves to create images of the beating heart. An echocardiogram is usually the main test to diagnose pulmonary atresia. It is shown how the blood moves through the heart and the heart valves. If an echocardiogram is done in a baby before birth, it is called a fetal echocardiogram.
  • The cardiac catheterization. A doctor threads a thin tube, called a catheter through a blood vessel in the arm or in the groin to an artery in the heart. The coloring is sent through the catheter. This makes the arteries of the heart are shown more clearly on x-rays. The test can provide detailed information on the flow of the blood and how the heart works. Certain heart treatments can be done during cardiac catheterization.

Treatment

Babies need emergency medical care for pulmonary atresia symptoms. The choice of surgery or procedures depends on the severity of the condition.

Drugs

Drugs can be administered through an IV to keep the ductus arteriosus open. This is not a long-term treatment of pulmonary atresia. But it gives health care professionals more time to decide what type of surgery or procedure can be better.

Surgery or other procedures

Sometimes, pulmonary atresia, the treatment can be performed by using a long, thin tube called a catheter. A doctor inserts the tube into a large blood vessel at a baby of the groin and the guide for the heart. Catheter-based procedures for pulmonary atresia include:

  • Balloon atrial septostomy. A balloon is used to widen the natural hole in the wall between the upper chambers of the heart. This hole, the foramen ovale, most often closes shortly after birth. Doing the larger hole allows blood to move easily from the right side of the heart to the left side.
  • The placement of a Stent. A doctor may put in a rigid tube called a stent in the ductus arteriosus to prevent it from closing. This keeps the blood flow to the lungs.

Infants with pulmonary atresia often need many surgeries of the heart over time. The type of heart surgery depends on the size of the child's lower right chamber of the heart and the pulmonary artery.

Types of surgery for pulmonary atresia include:

  • The derivation. This implies the realization of a new path for the flow of blood, called a "bypass" of the derivation. The derivation is from the main blood vessel leaving the heart, called the aorta, the pulmonary arteries. This allows sufficient flow of blood to the lungs. But most babies outgrow this derivation within a couple of months.
  • Glenn procedure. In this surgery, one of the large veins that returns blood to the heart binds to the pulmonary artery. Another of the great vein maintains the flow of blood into the right side of the heart. The heart then pumps the blood through the pulmonary valve has been repaired. This can help to the right ventricle to grow.
  • The Fontan procedure. If the right lower chamber of the heart remains too small to do its job, the surgeons can use this procedure to make a path. The pathway that allows the majority, if not all, of the blood that leaves the heart to flow into the pulmonary artery.
  • Heart transplant. In some cases, the heart is too damaged to fix. After a heart transplant may be necessary.

If the baby also has a ventricular septal defect (VSD), a surgery is performed to plug the hole. The surgeon then makes a connection from the right side of the pumping chamber to the pulmonary artery. This repair can be to use an artificial valve.

Lifestyle and home remedies

Here are some tips to take care of a person with pulmonary atresia after you get home from the hospital:

  • Come to health screenings. A person who is born with pulmonary atresia needs regular check-ups, even as an adult. A medical doctor trained in congenital heart disease, called congenital cardiologist, often providing the care. To obtain the recommended vaccinations, including annual influenza vaccinations.
  • Ask about the exercise and the activity. Some children with a congenital heart defect may need to limit your exercise or sports activities. However, many others with a congenital heart defect may participate in such activities. Your child's care team can say that the sports and types of exercise are safe for your child.
  • Practice good oral hygiene. Brushing and flossing and getting regular dental checkups can help prevent the infection.
  • Ask about the preventive antibiotics. Sometimes, a congenital defect of the heart can increase the risk of infection in the lining of the heart or of the 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.

Coping and support

Talk with other parents who have a child with a congenital heart defect that could give comfort and support. Ask a member of your child's care team about local support groups.

Preparing for your appointment

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

Here's some information to help you prepare for your appointment.

What you can do

When you make the appointment, ask if there is something that you need to do before you go. For example, 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 testing.

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

Make a list of:

  • Your child's symptoms, including those that do not appear to be linked to the pulmonary atresia. Try to remember when you noticed his presence.
  • Key staff of the facts, including the family history of congenital heart defects, pulmonary hypertension or other heart or lung diseases.
  • All medications, vitamins or supplements that your child takes and the dose. Also the list of medicines taken during pregnancy.
  • Questions to ask your child's healthcare provider.

Pulmonary atresia, some questions are:

  • What are other possible causes of my child's symptoms or condition?
  • What tests will my child need?
  • What is the best treatment?
  • What other treatments are there?
  • There are 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 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 prepared to answer questions, such as:

  • Has someone in your family has been diagnosed with pulmonary atresia or other congenital heart defect?
  • Does your child always have symptoms or the symptoms come and go?
  • How bad are the symptoms?
  • What, in any case, it seems that the symptoms better?
  • What, if anything, appears to worsen your symptoms?
Symptoms and treatment of Pulmonary atresia