Pulmonary Atresia

Pulmonary atresia is a heart defect present at birth (congenital) that's normally diagnosed within the first few hours or days of life. In pulmonary atresia, the valve that lets blood out of the heart to go to your baby's lungs doesn't form correctly. Instead of opening and closing to allow blood to travel from your heart to your lungs, a solid sheet of tissue forms. Blood from the right side of your baby's heart can't go back to the lungs to pick up oxygen.Pulmonary atresia may occur with or without a ventricular septal defect (VSD).

Bluish colored skin (cyanosis), Fast breathing, Fatigue, Poor eating habits (babies may get tired while nursing or sweat during feedings), Shortness of breath.The temporary measure, which has to be taken in new-borns with pulmonary atresia, is usually given an intravenous drug (injected into a vein) called prostaglandin E1 to prevent the ductus arteriosus from closing. Blood can flow from the right side of the heart to the left side and pass through the left ventricle to the lungs to pick up oxygen by keeping the ductus arteriosus open.

In Norway the statistical analysis of pulmonary atresia is resulted as sixty-four fetuses met inclusion criteria (median gestational age 22 weeks) with 47 live births.   One patient required a transannular patch repair after the initial valve-sparing repair. Twelve of 29 (41%) patients received prostaglandins at birth. Type of surgical repair, use of prostaglandins and postnatal death were among the outcomes investigated. A higher PV/AoV ratio was associated with valve-sparing repair (P=0.04). Fetal z-scores of the PV, AoV and right pulmonary artery at 29 to 32 weeks gestational age correlated with respective postnatal z-scores (P=0.01).