Also called PA/IVS, this rare congenital heart defect is diagnosed at or shortly after birth. Learn the symptoms and how it's treated.
Pulmonary atresia (uh-TREE-zhuh) is a rare heart problem present at birth, also called a congenital heart defect.
In pulmonary atresia, the valve between the heart and lungs isn't fully formed. This valve is called the pulmonary valve. Blood can't flow from the right lower heart chamber, called the right ventricle, to the lungs.
Some blood may move through a natural opening between the aorta, the artery carrying oxygen-rich blood out of the heart, and the pulmonary artery. This opening, called the ductus arteriosus, most often closes soon after birth. But it can be kept open with medicines.
In pulmonary atresia with intact ventricular septum (PA/IVS), there isn't a hole between the two pumping chambers of the heart. If there is a hole, the condition is called pulmonary atresia with ventricular septal defect (VSD).
A baby with pulmonary atresia doesn't get enough oxygen in the blood. Urgent treatment is needed. Treatment for pulmonary atresia with intact ventricular septum may include a mix of medicines, procedures or surgery to fix the heart.
Tests used to diagnose pulmonary atresia with intact ventricular septum (PA/IVS) include:
Babies with pulmonary atresia need treatment right away. It's best to get treatment at a medical center with surgeons and other healthcare professionals who have experience with complex heart problems present at birth.
Treatment for pulmonary atresia with intact ventricular septum (PA/IVS) may include medicines and one or more procedures or surgeries.
Medicine may be given through an IV to keep the ductus arteriosus open. This is not a permanent treatment for pulmonary atresia. But it gives the care team more time to determine the best type of surgery or procedure for the baby.
A baby with pulmonary atresia with intact ventricular septum (PA/IVS) most often needs one or more surgeries or procedures to improve blood flow and fix the heart.
Some of these treatments are done in the first days to weeks of a baby's life. Others are done later. The type of surgery or procedure depends on many things. These include the size of the right lower heart chamber and heart valves, and whether the baby has other heart problems.
After treatment, babies with pulmonary atresia should have regular health checkups, ideally with a pediatric heart doctor. This type of healthcare professional is called a pediatric cardiologist. Due to advances in treatment and technology, many people with pulmonary atresia with intact ventricular septum (PA/IVS) live into adulthood. Adults with PA/IVS should be followed by a doctor with specialized training in adult congenital heart disease.