Annals of Pediatric Cardiology
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Anomalous aortic origin of the pulmonary arteries: Case series and literature review

1 Mediterranean Congenital Heart Center – Bambino Gesù – San Vincenzo Hospital, Taormina, Italy
2 Department of General Surgery, Hospital Santo Tomas, Panamá
3 Department of Clinical Directors, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
4 Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
5 Medical Director, Bambino Gesù Children's Hospital IRCCS, Rome, Italy
6 Emeritus Professor, Royal College of London, London, UK
7 Department of Imaging, Bambino Gesù Children's Hospital IRCCS, Rome, Italy

Correspondence Address:
Salvatore Agati,
Director Congenital Heart Surgery, Mediterranean Congenital Heart Center, Bambino Gesù, Taormina
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/apc.APC_89_18

Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature.

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