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Pulmonary atresia
Pulmonary atresia with intact ventricular septum (wall between the lower heart chambers) (PA/IVS) is a condition in which the valve that allows your blood to flow from the lower-right chamber (right ventricle) of your heart to your lungs (pulmonary valve) hasn't formed properly or is closed (atresia). PA/IVS is a rare congenital heart disease present from the early stages of a baby's heart development.
In the womb, your baby receives oxygen from the placenta, and your baby's lungs aren't functioning yet. Blood entering the right side of your baby's heart passes through an opening that allows oxygen-rich (red) blood to flow to the left side of the heart 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.
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.
Read more about congenital heart disease in children and congenital heart disease in adults at www.MayoClinic.com.
Related book: Mayo Clinic Healthy Heart for Life! book provides an easy-to-follow plan to prevent and conquer heart disease.
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