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A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.
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