Scimitar Syndrome Research Paper

Scimitar syndrome, first described by Chassinat in 1836, is a variant of partial anomalous pulmonary venous drainage (PAPVD). It is a rare complex congenital anomaly characterized by abnormal pulmonary venous return of the right lung into the IVC or right atrium. It is usually diagnosed in childhood but it may be delayed until later in life when it is discovered incidentally (1). It is often associated with not only other cardiac defects including atrial septal defect,patent ductus arteriosus (2), hypoplasia of the left heart and aorta (3) but also lung anomalies like congenital right pulmonary hypoplasia and anomalous blood supply of the right pulmonary lower lobe from the infra diaphragmatic segment of the aorta ( 4) . It is a very rare …show more content…
Clinical features vary and depend upon the age of presentation and underlying complications of heart failure, pulmonary hypertension or repeated pulmonary infections due to these complications. Infants usually present with more severe disease and have poorer prognosis as compared to children and adults with this condition (5, 6). Infants with this condition present with tachycardia, failure to thrive, poor feeding, lethargy and cyanosis. Adult patients present with fatigue, exertional dyspnea and recurrent lower respiratory tract infections (5), although children with this condition have higher incidence of respiratory complications (7). Diagnosis requires careful history, physical examination and appropriate imaging studies. Physical examination may demonstrate a prominent precordial bulge (in patients with large left to right shunt), a systolic murmur or diastolic rumble. Aortic and pulmonary components of S2 are usually fixed split. Chest x-ray usually demonstrates scimitar-shaped “Turkish sword sign” (8) or “Scimitar Sign” (9). “Scimitar Sign” (fig. 1) is also present in Anomalous unilateral single pulmonary vein (AUSPV) which needs to be differentiated from scimitar syndrome due to different