Pulmonary Atresia with Intact Ventricular Septum (PA/IVS) is a rare congenital heart defect, meaning it is present at birth. PA/IVS can often be diagnosed during pregnancy with a fetal echocardiogram, a specialized ultrasound that looks closely at the baby's heart.
After birth, Babies with PA/IVS usually show signs such as:
- Bluish skin or lips (called cyanosis)
- Fast or difficult breathing
- Trouble feeding or appearing very tired
Because the pulmonary valve is blocked, the baby depends on an extra blood vessel call the ductus arteriosus to get the blood to the lungs. This vessel is part of the normal fetal hearts, but typically closes soon after birth, so doctors give medicine (prostaglandin) to keep it open to get blood flow to the lungs until intervention can be done.
In PA/IVS:
- The pulmonary valve, which normally allows blood to flow from the right side of the heart to the lungs, does not form properly.
- Because the valve is blocked, blood cannot travel from the right ventricle to the lungs to pick up oxygen.
- The wall between the two lower chambers of the heart (the ventricles) is intact, so there is no extra opening to get blood flow to the lungs.
As a result, the right ventricle often remains very small and underdeveloped. Babies with PA/IVS cannot get enough oxygen without special care.
