Scimitar Syndrome: Pathology, Clinical Presentation, Radiographic Features, and Treatment

Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia. We searched the mail databases such as Medline (via PubMed), Scopus and EMBASE and Google Scholar. Diagnosing infantile scimitar syndrome requires meticulous attention and high suspicion of the early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization. Scimitar syndrome can be initially suspected from a chest X-ray, but it is typically confirmed via Computed Tomography (CT) angiography. The clinical spectrum of Scimitar syndrome ranges from severely ill infants to asymptomatic adults, which may present respiratory or cardiac failure, hemoptysis and pulmonary hypertension, tachypnea, chest infection, and failure to thrive. The diagnosis can be made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR angiography. Considering the wide clinical spectrum of scimitar syndrome, the medical intervention depends on the severity of presentation and the amount of blood flowing to the Inferior Vena Cava (IVC) from completely or partially anomalous pulmonary veins. In the presence of significant left to right shunting and pulmonary hypertension, surgical intervention should be considered.