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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.
Statistical analysis on pulmonary atresia in United States resulted as in the mean age at last evaluation was 29 years (19-39 years). There were five deaths within the study period (1998-2009). Median age at death was 32 years (30-37 years). Seven patients underwent the Fontan operation, eight patients had a biventricular repair, and five patients remained with palliative shunts. All patients required re-interventions in adulthood.
