Pulmonary Atresia (PA)

The word "Atresia" means "No Opening." The Pulmonary Valve is found between the right ventricle and the pulmonary artery. It has three leaflets that function like a one-way door, allowing blood to flow forward into the pulmonary artery, but not backward into the right ventricle.

In Pulmonary Atresia, the valve that lets blood flow from the lower-right chamber (the right ventricle) to the lungs has not formed or is closed.

Instead of following the normal route through the lungs, oxygen-poor blood travels directly from the upper-right chamber (the right atrium) to the left side of the heart. This oxygen-poor blood is then pumped back into the body. The lack of oxygen-rich blood makes the fingers, toes, and lips appear blue. This condition is called Cyanosis.

Babies with Pulmonary Atresia seem normal before birth. This is because a passageway called the Ductus Arteriosus connects the pulmonary artery to the aorta in the fetal heart. The ductus arteriosus begins to close naturally after birth. In normal babies, a new valve opens that lets blood flow from the right ventricle to the lungs. But in infants with pulmonary atresia, the valve does not open. This means that the only path for blood to reach the lungs will soon begin to close.

SYMPTOMS OF PULMONARY ATRESIA

* Rapid breathing

* Difficulty breathing

* Irritability

* Lethargy

* Pale, cool, or clammy skin

DIAGNOSIS

* CHEST X-RAY – a test that uses radiation to take pictures of structures inside the chest.

* ELECTROCARDIOGRAM(ECG, EKG) – a test that records the heart's activity by measuring electrical currents through the heart muscle; it may show signs of heart strain or enlargement.

* ECHOCARDIOGRAM – a test that uses high-frequency sound waves (ultrasound) to examine the size, shape, and motion of the heart.

* CARDIAC CATHETERIZATION – an x-ray of the heart's circulation that is done after injection of a contrast dye.

TREATMENT FOR PULMONARY ATRESIA

Newborns with pulmonary atresia are usually given a medicine that keeps the ductus arteriosus open after birth.

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 done 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 an operation called the FONTAN PROCEDURE. In this procedure, the right atrium is connected directly to the pulmonary artery.

In some cases, if the right ventricle and pulmonary artery are too small, it may be impossible to repair the defect.