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Article Abstract

Objectives: Anomalous origin of one pulmonary artery branch from the aorta in the presence of separate aortic and pulmonary valves is a rare but important entity necessitating early diagnosis and surgery to prevent irreversible vascular pulmonary disease. We evaluated our experience with seven infants having this anomaly.

Study Design: Between December 2003 and 2009, a total of seven infants (2 girls, 5 boys, age range 4 days to 84 days) were diagnosed as having anomalous origin of one pulmonary artery branch from the aorta. Clinical records were reviewed for clinical features, operative procedures, and postoperative follow-up.

Results: Common symptoms were dyspnea, tachypnea, and poor feeding. All the cases were diagnosed by echocardiography. The right pulmonary artery in six cases and the left pulmonary artery in one case originated from the ascending aorta. In addition, three patients had patent ductus arteriosus (PDA), five patients had patent foramen ovale, and one patient had interruption of the aortic arch and aortopulmonary window. All patients underwent surgical re-implantation of the anomalous pulmonary artery branch to the pulmonary trunk. Associated surgical procedures included PDA ligation in three patients, and total repair of interrupted aortic arch and aortopulmonary window in one patient. There were no in-hospital deaths. Two patients had prolonged intubation (10 and 16 days). All patients were discharged in good clinical condition. During a follow-up period ranging from two months to six years, only one patient developed stenosis at the site of anastomosis.

Conclusion: Prompt diagnosis at infancy, improved surgical technique, and good patient care decrease mortality and morbidity associated with anomalous origin of the pulmonary artery from the aorta.

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