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Pulmonary atresia

Pulmonary atresia (uh-TREE-zhuh) 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 your heart to your lungs, a solid sheet of tissue forms. Blood from the right side of your baby's heart can't go back to the lungs to pick up 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. Depending on your child's condition, more surgeries will often be necessary in childhood. Ongoing medical care through adulthood can improve your child's prognosis.

Symptoms Causes Risk factors Complications Prevention

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. Your baby's signs and symptoms may include:

  • 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

When to see a doctor

Your baby will most likely be diagnosed with pulmonary atresia within the first few hours to the first few days of life. However, if your baby has any symptoms of pulmonary atresia after you've returned home, seek emergency medical attention. Symptoms like cyanosis in babies should be examined by a doctor as soon as possible.

There is no known cause of pulmonary atresia. It's thought that the problems that cause pulmonary atresia begin early in the pregnancy. To understand the problems pulmonary atresia causes, it's helpful to know how the heart works.

How the heart works

The heart is divided into four hollow chambers, two on the right and two on the left. In performing its basic job — pumping blood throughout the body — the heart uses its left and right sides for different tasks. The right side of the heart moves blood to the lungs through vessels called pulmonary arteries. In the lungs, blood picks up oxygen then returns to the heart's left side through the pulmonary veins. The left side of the heart then pumps the blood through the aorta and out to the rest of the body to supply your baby's body with oxygen. Blood moves through your baby's heart in one direction through valves that open and close as the heart beats. The valve that allows blood out of your baby's heart and into the lungs to pick up oxygen is called the pulmonary valve.

In pulmonary atresia, the pulmonary valve doesn't develop properly, preventing it from opening. Blood can't flow from the right ventricle to the lungs. Before birth, the improperly formed valve isn't life-threatening, because the placenta provides oxygen for your baby instead of the lungs. Blood entering the right side of your baby's heart passes through a foramen ovale — a hole between the top chambers of your baby's heart (atria) that lets oxygen-rich blood move to the left side of the heart and be pumped on to the rest of your baby's body.

After birth, your baby's lungs must provide oxygen for his or her body. In pulmonary atresia, without a working pulmonary valve, blood must find another route to reach your baby's lungs.

The foramen ovale often shuts soon after birth, but may stay open in pulmonary atresia, allowing oxygen-poor blood to pass through the upper chambers of the heart. From there, it goes to the left ventricle, out the aorta (the body's main artery), on to the rest of the body. However, this blood flow can't adequately supply your baby's body with oxygen.

Newborn babies also have a temporary connection between the body's aorta and the pulmonary artery, called the ductus arteriosus. This allows some of the oxygen-poor blood to pass into the lungs where it can pick up oxygen to supply your baby's body. The ductus arteriosus normally closes within a few hours or days after birth, but can be kept open with medications.

In some cases, there may be a second hole in the tissue that separates the main pumping chambers of your baby's heart, called a ventricular septal defect (VSD). The VSD allows a pathway out for blood to enter the left ventricle. If there's no VSD, the right ventricle receives little blood flow before birth and often doesn't develop fully. This is a condition called pulmonary atresia with intact ventricular septum (PA/IVS).

Doctors aren't sure why some babies develop pulmonary atresia. However, certain environmental and genetic risk factors may play a role. They include:

  • Rubella (German measles). Having rubella during pregnancy can cause problems in your baby's heart development. Your doctor can test you for immunity to this viral disease before pregnancy and vaccinate you against it if you aren't immune.
  • Diabetes. Having this chronic condition may interfere with the development of your baby's heart. You can reduce the risk by carefully controlling your diabetes before attempting to conceive and during pregnancy. Gestational diabetes generally doesn't increase your baby's risk of developing pulmonary atresia or other heart defects.
  • Medications. Taking certain medications while pregnant is known to cause birth defects. Give your doctor a complete list of the medications you take before attempting to become pregnant. Medications that increase risk include thalidomide (Thalomid) and some anti-seizure medications.
  • Drinking alcohol during pregnancy. Avoid alcohol during pregnancy because babies with fetal alcohol syndrome may also develop congenital heart defects.
  • Heredity. Congenital heart defects appear to run in families and are associated with many genetic syndromes. If you already have a child with a congenital heart defect, a genetic counselor can predict the approximate odds that your next child will have one.

Even with treatment, you'll need to carefully monitor your child's health for any changes that could signal a problem. As your child grows, his or her heart changes size and shape, which can make more treatments necessary.

Complications of pulmonary atresia can include:

  • Developmental delays. Children with congenital heart defects often develop and grow more slowly than do children who don't have heart defects. Your child may be smaller than other children of the same age and, if the nervous system has been affected, may learn to walk and talk later than other children.
  • Heart infections (endocarditis). People with structural heart problems, such as pulmonary atresia, are at a higher risk of infectious endocarditis than is the general population. Infectious endocarditis is an inflammation of the inner lining of the heart caused by a bacterial infection.
  • Stroke. Although uncommon, children with pulmonary atresia and a ventricular septal defect are at increased risk of stroke due the possibility of a blood clot traveling through the heart to the brain.
  • Heart failure. This serious complication, which makes it difficult for the heart to pump blood to the body, usually develops in the first six months after birth in babies who have a significant heart defect. Signs of congestive heart failure include rapid breathing, often with gasping breaths, and poor weight gain.

Because the exact cause of pulmonary atresia is unknown, it may not be possible to prevent it. However, there are some things you can do that might reduce your child's overall risk of birth defects and pulmonary atresia, such as:

  • Get a German measles (Rubella) vaccine. If you develop German measles during pregnancy, it may affect your baby's heart development. Being vaccinated before you try to conceive likely eliminates this risk.
  • Control chronic medical conditions. If you have diabetes, keeping your blood sugar in check can reduce the risk of heart defects. If you have other chronic conditions, such as epilepsy, that require the use of medications, discuss the risks and benefits of these drugs with your doctor.
  • Avoid harmful substances. During pregnancy, leave painting and cleaning with strong-smelling products to someone else. Also, don't take any herbs, dietary supplements or drugs without consulting your doctor first.
  • Take a multivitamin with folic acid. Daily consumption of 800 micrograms of folic acid has been shown to reduce birth defects in the brain and spinal cord, and may help reduce the risk of heart defects as well.
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