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Pulmonary Atresia

With this condition, the pulmonary valve, between the right ventricle and the pulmonary artery, is not formed completely. “Atresia” refers to a missing heart structure. The valve cannot open properly, which means the blood cannot move from the right ventricle to the lungs. If the constriction occurs right at the valve, it is called pulmonary valvular atresia.

Often this defect occurs with other heart defects, including ventricular septal defect.

Pulmonary atresia occurs in about one baby per 14,000 births, and is the tenth most common type of heart disease in newborns.

Pulmonary Atresia
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With this condition, the pulmonary valve, between the right ventricle and pulmonary artery, along with the main pulmonary artery, either doesn't exist or is only partially formed. The result is that blood can't flow as usual from the right ventricle to the lungs. Instead, blood reaches the lungs by way of a patent ductus arteriosus.
What are the symptoms of pulmonary atresia?

The main symptoms are a murmur, cyanosis, rapid breathing or trouble breathing, irritability, low energy, and clammy skin. The degree of symptoms depends on the severity of the defect and the presence of other defects.

The doctor may order any of the following tests: chest X-ray, electrocardiogram, echocardiogram, cardiac catheterization, and cardiac magnetic resonance imaging.

How is pulmonary atresia treated?

Treatment might involve giving your child oxygen in the intensive care unit to help them breathe, and possibly drugs to help ease the heart’s work. Another drug, called prostaglandin, which is given by IV, can help keep the ductus arteriosus open. This opening allows blood to flow to the lungs.

Cardiac catheterization can sometimes be done to help correct a problem. The procedure may involve a balloon atrial septostomy to improve the mix of oxygen-rich blood and oxygen-poor blood between the atria.

Surgery is another option. This is typically done in stages, starting with the insertion of a BT shunt, followed by the bi-directional Glenn procedure and removal of the BT shunt, and then, when the child is a bit older, the Fontan procedure.

What is the long-term outlook for children with pulmonary atresia?

Surgical repair of this condition has an excellent outcome, though this depends on whether the surgery was corrective or palliative. Full repair enables most children to lead full, normal lives.

Fraser Golding, MD, FRCPC