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Pulmonary Atresia

Also known as: PA.

What is ​Pulmonary Atresia (PA)?

Pulmonary atresia is a birth defect of the heart where the valve that controls the flow of blood from the right side of the heart to the lungs does not form, preventing blood picking up oxygen.

What causes pulmonary atresia?

Pulmonary atresia is a congenital heart defect, which means that babies are born with it. Researchers aren't sure of the exact cause, but it often occurs along with other heart problems such as patent ductus arteriosus or ventricular septal defect.

What are the symptoms of pulmonary atresia?

Babies born with PA usually have symptoms at birth or soon afterwards. The lack of oxygen caused by pulmonary atresia leads to problems breathing and a blue tint to the skin. Sleepiness, fatigue & poor feeding also occur.

What are pulmonary atresia care options? 

Pulmonary atresia is a “critical congenital heart defect”. Most babies will need medications to keep the ductus arteriosus open after birth to improve blood flow to the lungs. Surgical procedures will be needed to correct the defect.

Reviewed by: Jack Wolfsdorf, MD, FAAP

This page was last updated on: 5/22/2026 8:00:11 PM

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Cyanotic Heart Disease Cyanotic heart disease refers to a group of congenital heart defects in babies that present with a characteristic blue color of the skin
Congenital Heart Defects/Disease Any unusual physical feature or health problem that is present at the birth of a baby is known as a birth defect or a congenital anomaly.
Ventricular Septal Defect Ventricular septal defect is a defect that babies are born with. It is often present with other heart problems.
Patent Ductus Arteriosus (PDA) Patent ductus arteriosus is a heart condition that affects some babies soon after birth.
Cyanosis A bluish tinge of the lips, tongue, nail beds or skin is called cyanosis. This occurs because of a lack of oxygen in the red cells of blood and is never normal.
Right Ventricular Outflow Tract Reconstruction RVOT reconstruction is a surgical repair of the RVOT. Frequently, a pulmonary valve will also need to be replaced as part of the reconstruction of the heart.