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Chirurgia 2011 June;24(3):177-80

language: English

Horseshoe lung-Scimitar syndrome complex associated with severe pulmonary hypertension in a newborn: case report

Olukman Ö. 1, Çalkavur S. 1, Öner T. 2, Yilmaz N. 1, Can D. 3, Tavli V. 2, Hazan E. 4, Atlihan F. 1

1 Department of Neonatology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
2 Department of Pediatric Cardiology, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
3 Department of Pediatric Respiratory Diseases, Dr. Behçet Uz Children’s Hospital, İzmir, Turkey
4 Department of Cardiovascular Surgery, Faculty of Medicine, Dokuz Eylül University, İzmir, Turkey


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Horseshoe lung is a rare congenital anomaly in which the posterobasal segments of the right and left lungs are fused by an isthmus of pulmonary parenchyma. Although it may appear as an isolated problem, it is often associated with Scimitar syndrome. Scimitar syndrome is a rare congenital cardiopulmonary malformation which is characterized by partial or total anomalous pulmonary venous drainage of the right or left lung into the inferior vena cava. While some cases stay asymptomatic until adult age, others may present in early infancy or childhood. Here we report a 14-day-old newborn who was admitted to neonatal intensive care unit with severe respiratory and circulatory failure and diagnosed as horseshoe lung-Scimitar syndrome complex on computed tomography and conventional angiography after developing severe pulmonary hypertension resistant to medical treatment. Partial anomalous pulmonary venous return and a large, artery originating from the descending aorta supplying the right lower lung was confirmed in cardiac catheterization. Coil embolization of the feeding artery to the lower right lung was successfully performed and pulmonary hypertension was relieved dramatically soon after the procedure. The case was found worth presenting in order to remind clinicians of Scimitar syndrome in the differential diagnosis of severe pulmonary hypertension during neonatal period and emphasize the importance of coil embolization into abnormal feeding arteries as an alternative treatment choice which can be performed easily with low interventional risks and high success.

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