- Scimitar syndrome
Infobox_Disease
Name = PAGENAME
Caption =
DiseasesDB =
ICD10 = ICD10|Q|26|8|q|20
ICD9 = ICD9|747.49
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D012587Scimitar syndrome, or pulmanory venolobar syndrome, is a rare
congenital heart defect characterized by anomalous venous return from the right lung. [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 |format=PDF] This anomalous pulmonary venous return can be either partial (PAPVR) or total (TAPVR). The syndrome associated with PAPVR is more commonly known as The term "Scimitar syndrome" arises from the curvilinear pattern created on a chest radiograph by the pulmonary veins that drain to the inferior vena cava.Citation
url=http://www.childrenshospital.org/az/Site2165/mainpageS2165P0.html
title=Scimitar Syndrome
publisher=Children's Hospital Boston
accessdate=2008-02-28] This radiographic density often has the shape of ascimitar , a type of curved sword.Presentation
The 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.Diagnosis
The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, and more recently by CT angiography or MR Angiography.
Treatment
Surgical 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.
References
External links
*Citation
url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=14872299&query_hl=2&itool=pubmed_DocSum
title=Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome
journal=Pediatric radiology
id= ISSN 0301-0449
last=Holt
first=PD
last2=Berdon
first2=WE
last3Marans
first3=Z
last4=Griffiths
first4=S
last5=Hsu
first5=D
volume34
issue5
pages=409-13
date=February 11 ,2004 Further reading
*Halasz NA, Halloran KH, Liebow AA. Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava. Circulation 1956;14(5):826-46.
*Kramer U, Dornberger V, Fenchel M, Stauder N, Claussen CD, Miller S. Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging. Eur Radiol 2003;13 Suppl 4:L147-50.
*Brown JW, Ruzmetov M, Minnich DJ, et al. Surgical management of scimitar syndrome: an alternative approach. J Thorac Cardiovasc Surg 2003;125(2):238-45.
Wikimedia Foundation. 2010.
