Pulmonary atresia surgery


 * Associate Editor(s)-In-Chief: Priyamvada Singh, M.B.B.S. [mailto:psingh@perfuse.org], ; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Overview
A patient with pulmonary atresia will require immediate intervention. Surgical intervention is necessary to improve permanently improve blood flow.

Surgical Therapy
Preliminary treatment is cardiac catheterization to evaluate the defect or defects of the heart; this procedure is much more invasive. Ultimately, however, the patient will need to have a series of surgeries to improve the blood flow permanently.

The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, open heart surgery can be performed to make blood flow through the heart in a normal pattern. If the right ventricle is small and unable to act as a pump, doctors may perform another type of operation called the Fontan procedure. In this two-stage procedure, the right atrium is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, by-passing the heart.

The first surgery will likely be performed shortly after birth. A shunt can be created between the aorta and the pulmonary artery to help increase blood flow to the lungs. As the child grows, so does the heart and the shunt may need revised in order to meet the body's requirements.

Cardiac catheterization
A cardiac catheterization procedure can be used as a diagnostic procedure, as well as initial treatment procedure as balloon atrial septostomy to improve mixing oxygenated blood and unoxygenated blood between the right and left atria.

Atrial Septostomy: a special catheter with a balloon in the tip is used to create an opening in the atrial septum. The catheter is guided through the foramen ovale to the left atrium (LA). Once the ballon is in the LA is inflated and then pulled back opening a bigger hole between the right atrium and the LA to mix blood.

If the hospital does not have a catheterization lab with skill physician to perform the ballon atrial septostomy, an intravenous medication called prostaglandin is administered to keep the ductus arteriousus from closing.