Scimitar Syndrome Associated with Agenesis of Right Pulmonary Artery

Scimitar syndrome is a very rare disorder and also known as hypogenetic lung syndrome. The syndrome is characterized with anomalous connection of the pulmonary vein with the vena cava inferior. In this text, scimitar syndrome associated with pulmonary artery hypoplasia is presented. A 21-year-old male patient with complaints of cough, shortness of breath and sputum was admitted. Chest X-ray revealed volume loss of right lung and shift to the right of heart and mediastinum. The middle lobe wasn’t observed in bronchoscopy and thorax CT. Thoracic CT angi-ography images revealed that vein of right lung lower lobe drains right hepatic vein and right pulmonary artery diameter is reduced. Lung perfusion scintigraphy revealed total loss of perfusion of right lung. Scimitar sign on chest X ray is characteristics for scimitar syndrome. However, this sign cannot be seen in some patients. If there is volume loss of right lung and shift to the right of heart and mediastinum, scimitar syndrome should be considered in addition to other diseases.