A rare cause of recurrent wheezing and respiratory distress: Scimitar syndrome

• Published in: Türk Kardiyoloji Derneği arşivi Vol. 39; no. 7; pp. 595 - 598
• Main Author: Ergul, Yakup
• Format: Journal Article
• Language: Turkish; English
• Published: Turkey 01.10.2011
• Subjects: Cardiac Catheterization; Diagnosis, Differential; Echocardiography; Humans; Infant; Male; Pulmonary Veins - abnormalities; Radiography; Scimitar Syndrome - diagnosis; Scimitar Syndrome - diagnostic imaging; Scimitar Syndrome - surgery; Vena Cava, Inferior - abnormalities
• ISSN: 1016-5169; 1308-4488
• DOI: 10.5543/tkda.2011.01581

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung. We present a 15-month-old boy who was diagnosed with Scimitar syndrome after examinations for recurrent wheezing and respiratory distress. The chest radiograph showed a scimitar sign in the right hemithorax, obscuring the contours of the right atrium. Echocardiography showed dilatation of the right heart cavities and increased flow in the inferior vena cava, without a cardiac abnormality. The patient underwent cardiac catheterization for radiographic and hemodynamic evaluations, during which a scimitar vein was detected, draining the right pulmonary veins to the inferior vena cava. Coil occlusion was performed on the abnormal artery arising from the infradiaphragmatic aorta. The patient was referred to surgery for repair of the anomalous pulmonary venous return and resection of the sequestered pulmonary segment.