Pulmonary Atresia

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Pulmonary atresia — which means "no opening" — with intact ventricular septum (PA/IVS) is a rare congenital heart disease. Pulmonary atresia is when the valve that lets blood flow from the lower-right chamber (right ventricle) of the heart to the lungs has not formed properly or is closed. This abnormality is present from the very early stages of heart development (around the first eight weeks of pregnancy), resulting in an underdeveloped right ventricle, which is the pumping chamber of the heart.

Before birth, while the baby is developing, this condition does not threaten life because the placenta provides oxygen for the baby and the lungs are not yet functioning. Blood entering the right side of the fetal heart passes through an opening called the foramen ovale that allows oxygen-rich (red) blood to pass through to the left side of the heart and circulate throughout the body. After birth, the placenta no longer provides oxygen for the newborn — the lungs must provide it.

Two connections within a newborn's heart can provide temporary help in getting blood flow to the lungs — the foramen ovale and the patent ductus arteriosus (PDA). As these connections spontaneously begin to close after birth, the baby becomes critically ill due to an inadequate blood flow to the lungs where the blood picks up oxygen. This situation cannot support life, since oxygen-poor (blue) blood cannot meet the body's demands. The baby becomes very blue (cyanotic) and sick.

Receiving a diagnosis of PA/IVS can be frightening and overwhelming; however, there are surgical options for treating infants with this condition.