Scimitar syndrome

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 = D012587

Scimitar 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 a scimitar, 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.

См. также в других словарях:

  • scimitar syndrome — complete or partial venous drainage of the right lung into the inferior vena cava, usually with hypoplasia of the right lung; the anomalous vein has a scimitar shape on a radiograph; see scimitar sign, under sign …   Medical dictionary

  • scimitar sign — on a radiograph of the chest, a scimitar shaped shadow to the right of the lower border of the heart, representing the anomalous vein of the scimitar syndrome …   Medical dictionary

  • Liste der Syndrome — Diese Seite listet in alphabetischer Reihenfolge und ohne Anspruch auf Vollständigkeit Syndrome und Komplexe aus unterschiedlichen medizinischen Fachgebieten auf. Bitte nur Verweise auf den tatsächlichen Titel des Beitrags und keine… …   Deutsch Wikipedia

  • Síndrome de la cimitarra — Clasificación y recursos externos CIE 10 Q26.8 CIE 9 747.49 MeSH …   Wikipedia Español

  • List of diseases (S) — A list of diseases in the English wikipedia.DiseasesTOC NOTOC Sa Sc Se Sh Si Sj Sn So Sp Sq Ss St Su Sw Sy aSaa Sak Sal Sar Sat Say aa Sal* Saal Bulas syndrome * Saal Greenstein syndrome * Sabinas brittle hair syndrome * Saccharopinuria * Sackey… …   Wikipedia

  • Congenital heart defect — Classification and external resources The normal structure of the heart (left) in comparison to two common locations for a ventricular septal defect (right), the most common form of congenital heart defect.[1] …   Wikipedia

  • Coarctation of the aorta — Aortic coarctation Classification and external resources ICD 10 Q25.1 ICD 9 747.10 …   Wikipedia

  • Patent ductus arteriosus — Classification and external resources Heart cross section with PDA ICD 10 Q …   Wikipedia

  • Cerebral arteriovenous malformation — Classification and external resources Large arteriovenous malformation of the parietal lobe ICD 10 Q …   Wikipedia

  • Overriding aorta — Classification and external resources Diagram of a healthy heart and one suffering from overriding aorta ICD 10 Q …   Wikipedia


Поделиться ссылкой на выделенное

Прямая ссылка:
Нажмите правой клавишей мыши и выберите «Копировать ссылку»