Pulmonary Atresia - Treatment

Treatment

At first, the child will need to be admitted to the intensive care unit and possibly placed on oxygen and a ventilator that will help the child breathe. Also, IV medications that assist the heart and lung function may be given. An IV medication called prostaglandin E1 is used for treatment of pulmonary atresia, as it stops the ductus arteriosus from closing, allowing mixing of the pulmonary and systemic circulations. But prostaglandin E1 can be dangerous as it can cause apnea. Another example of preliminary treatment is heart 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 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.

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 three-stage procedure, the right atrium is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, by-passing the heart.