| 词条 | Scimitar syndrome |
| 释义 |
| name = Scimitar syndrome | synonyms = | image = Scimitar syndrome chest CT.jpg | caption = Scimitar syndrome chest CT | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Scimitar syndrome, or congenital pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium).[1] This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as Scimitar syndrome after the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava.[2] This radiographic density often has the shape of a scimitar, a type of curved sword.[2] The syndrome was first described by Catherine Neill in 1960.[3] PresentationThe anomalous venous return forms a curved shadow on chest x-ray such that it resembles a scimitar. This is called the Scimitar Sign. Associated abnormalities include right lung hypoplasia with associated dextroposition of the heart, pulmonary artery hypoplasia and pulmonary sequestration.Incidence is around 1 per 100,000 births.[4] DiagnosisThe diagnosis is made by transthoracic or transesophageal echocardiography, angiography, and more recently by CT angiography or MR Angiography. TreatmentSurgical correction should be considered in the presence of significant left to right shunting (Qp:Qs ≥ 2:1) and pulmonary hypertension. This involves creation of an inter-atrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium. References1. ^{{Citation|url=http://medind.nic.in/icb/t05/i3/icbt05i3p249.pdf|title=Clinical Brief: Scimitar Syndrome|last=Sehgal|first=Arvind|last2=Loughran-Fowlds|first2=Allison|publisher=medIND — Biomedical journals from India|accessdate=2008-02-28 }} 2. ^1 {{Citation|url=http://www.childrenshospital.org/az/Site2165/mainpageS2165P0.html|title=Scimitar Syndrome|publisher=Children's Hospital Boston|accessdate=2008-02-28}} 3. ^{{cite journal|url=http://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(06)68565-6.pdf|title=Catherine Neill|first=Ivan|last=Oransky|journal=The Lancet|volume=367|issue=9519|page=1312|year=2006|doi=10.1016/s0140-6736(06)68565-6}} 4. ^M.Hasan, A.Varshney. A case of scimitar syndrome: anesthetic considerations regarding non cardiac surgery. Pediatric Anesthesia and Critical Care Journal 2016;4(2):89-90 doi:10.14587/paccj.2016.18 . Further reading{{refbegin}}
External links{{Medical resources| DiseasesDB = | ICD10 = {{ICD10|Q|26|8|q|20}} | ICD9 = {{ICD9|747.49}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = D012587 }}{{Congenital vascular defects}}Lungenvenenfehlmündung#Das Scimitar-Syndrom 3 : Congenital heart defects|Syndromes affecting the heart|Syndromes affecting the lung |
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