Article Details

Scimitar syndrome is a rare vascular malformation characterized by partial or total abnormal flow of the right pulmonary vein into the inferior vena cava located above or below the diaphragm [1]. All or some of the pulmonary veins from the lower lobe, and sometimes the middle lobe of the right lung, drain anomalously into the IVC, either just above or below the diaphragm. The appearance on chest radiography resembles a curved Turkish sword, a scimitar, with a vertical radiographic shadow along the lower right cardiac border [3]. Scimitar syndrome is also associated with various other congenital anomalies. Other anomalies often found in patients with scimitar syndrome are dextrocardia, hypoplastic right lung and right pulmonary artery, and a systemic arterial connection between the thoracic or abdominal aorta and right lower lobe. Additionally, 70 % of patients with scimitar syndrome have ASD or patent foramen ovale (PFO) [2]. Scimitar syndrome is a rare disease with an incidence of approximately 1-3 per 100,000 live births. They can be divided into infantile and child/adult types. Infantile patients have more deformities and poor prognosis; therefore, there is an urgent need to combine multidisciplinary early precision diagnosis and treatment. Most children with childhood/adult forms are asymptomatic or have mild symptoms, and can live normally without medical intervention [1]. Here, we report a case of a 7-year-old girl who was diagnosed with ASD II by an echo study and confirmed to have Scimitar Syndrome by CT angiography. Multiplanar CT images of malformed drainage veins in the patient’s lungs are presented. We have demonstrated in detail the diagnostic process of this rare disease.

Keywords: Scimitar syndrome; Third world country; Echocardiograph