文章基本信息

标题：Scimitar Syndrome: Pathology, Clinical Presentation, Radiographic Features, and Treatment
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作者：Yazdan Ghandi ; Alireza Farsi
期刊名称：International Journal of Pediatrics
印刷版ISSN：2345-5047
电子版ISSN：2345-5055
出版年度：2019
卷号：7
期号：12
页码：10611-10620
DOI：10.22038/ijp.2019.43898.3648
出版社：Mashhad University of Medical Sciences
摘要：Scimitar syndrome is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia.Wesearched 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.
关键词：Congenital anomaly; Pulmonary hypertension; Scimitar Syndrome