Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia is a congenital (present at birth) heart defect in which your pulmonary valve doesn’t develop normally or stays blocked after birth. Without a working pulmonary valve, blood can’t flow through your pulmonary artery to reach your lungs, where it gets oxygen. Instead, oxygen-poor blood goes throughout your body.

The pulmonary valve, one of four valves in your heart, islocated on the right side of your heart. It’s between your right ventricle and your pulmonary artery (the blood vessel that carries blood to your lungs).The pulmonary valve usually opens to let blood flow from your right ventricle into your pulmonary artery.

How does pulmonary atresia affect my baby?

In addition to not having a normal pulmonary valve, a baby with pulmonary atresia may have:

• Lips, fingers, and toes that look blue because of a lack of oxygen in their blood.
• An underdeveloped right ventricle and tricuspid valve (the valve connecting the right atrium and right ventricle).
• An opening (the foramen ovale) between their heart’s right atrium and left atrium that may stay open instead of closing like it should after birth. This allows oxygen-poor blood to pass from the right atrium to the left atrium, where it flows through the left ventricle to the aorta.

In babies with pulmonary valve atresia, there also may be a problem with a dividing wall in their heart. This wall normally helps blood go where it’s supposed to go.

Your heart has four chambers, with a solid wall called the septum between your heart’s two sides. The right side sends blood to your lungs to get oxygen. The left side of your heart moves oxygen-rich blood to the rest of your body through your aorta (the main artery in your heart).

Pulmonary atresia types

• Pulmonary atresia with a ventricular septal defect. This opening in the wall (septum) between the right and left ventricles allows oxygen-rich blood to mix with oxygen-poor blood.
• Pulmonary atresia with an intact ventricular septum. The wall between the left and right sides of your heart is whole (intact).

Who does pulmonary atresia affect?

Genetic factors, such as an abnormal gene or chromosomal defect, may increase the chances of heart defects in certain families. Some children with genetic disorders like DiGeorge’s syndrome or velocardiofacial syndrome may be at greater risk for pulmonary atresia.

Is pulmonary atresia rare?

Yes. Pulmonary atresia occurs in about one out of 10,000 live births.