Pulmonary atresia

Overview

Pulmonary atresia (uh-TREE-zhuh) with intact ventricular septum (PA/IVS) is a condition in which the pulmonary valve — the valve that allows your blood to flow from the lower right chamber (right ventricle) of your heart to your lungs — hasn't formed properly or is closed (atresia). PA/IVS is a rare type of congenital heart disease that is present from the early stages of a baby's heart development.

In the womb, your baby receives oxygen from the placenta, instead of from the lungs. Blood entering the right side of your unborn baby's heart passes through an opening that allows oxygen-rich (red) blood to flow from the pulmonary artery to the aorta and circulate throughout your baby's body. After birth, the opening is pushed closed as blood begins to flow through your newborn's lungs to provide oxygen.

In children with PA/IVS, the closed pulmonary valve prevents the baby's heart from pumping blood into the lungs to pick up oxygen. Blood must be sent to the lungs by another route to provide oxygen for the newborn.

Read more about congenital heart disease in children, congenital heart disease in adults and pulmonary atresia.

Why choose Mayo Clinic

Mayo Clinic doctors with training in heart disease (cardiologists) treat children who have congenital heart diseases including PA/IVS. For more than 60 years, Mayo Clinic doctors have treated thousands of people who have congenital heart diseases. Cardiologists, surgeons and other specialists dedicated to the needs of people who have congenital heart disease staff the Center for Congenital Heart Disease at Mayo Clinic in Minnesota. The staff works closely to provide efficient, coordinated care.

Mayo Clinic in Rochester, Minn., is ranked among the Best Hospitals for heart and heart surgery by U.S. News & World Report. Mayo Clinic also ranks among the Best Children's Hospitals for heart and heart surgery.

Related book: Mayo Clinic Healthy Heart for Life! book provides an easy-to-follow plan to prevent and conquer heart disease.