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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.
Results from Cox proportional hazards model analysis showed that low birth weight (P = .024), unipartite right ventricular morphology (P = .001), and the presence of a dilated right ventricle (P < .001) were independent risk factors for death. The presence of coronary artery fistulae, right ventricular dependence, or the tricuspid valvar z score did not prove to be risk factors for death. After up to 9 years of follow-up, 29% have achieved a biventricular repair, 3% a so-called one-and-a-half ventricular repair, and 10.5% a univentricular repair, with 16.5% still having a mixed circulation (41% died).
