Pulmonary Atresia

Pulmonary atresia is a congenital heart malformation in which the valve from the right side of the heart that normally allows the movement of blood from the heart to the lungs does not form during pregnancy. One of every 10,000 babies is born with pulmonary atresia.

During pregnancy, a natural opening (the “foramen ovale”) exists between the two upper chambers of the heart allowing blood to flow between them. Normally, this opening closes at birth. But in cases of pulmonary atresia, sometimes the foramen ovale remains open after birth. Likewise, a blood vessel, the “patent ductus arteriosis,” allows blood to the lungs before birth, but closes at birth. Medication can be given to keep this vessel open.



Typical Heart - Cross Section




Heart with Pulmonary Atresia with Intact Ventricular Septum (VS)

 

Pulmonary atresia might be discovered during a prenatal ultrasound. A fetal echocardiogram can be done to help confirm the diagnosis.

After birth, the signs of the condition include fast breathing or shortness of breath, and ashen or bluish color to the skin, poor feeding, and extreme sleepiness. A cardiac catheterization can be done to finalize the diagnosis.

Treatment will most likely include surgery, and several types are available depending on the severity of the malformation. In some cases, a catheter can be inserted and a stent placed to keep open the ductus arteriosis. Then, open heart surgery will be necessary to repair or replace the pulmonary valve or to place a tube between the right ventricle and the arteries to the lungs. Finally, the heart must be rebuilt as a single ventricle, creating one pumping chamber instead of the usual two. If all else fails, a heart transplant may be needed.

Pulmonary Atresia