Scimitar syndrome

From Wikipedia, the free encyclopedia

Scimitar syndrome Classification and external resources
ICD-10	Q26.8
ICD-9	747.49
MeSH	D012587

Scimitar syndrome, or pulmanory venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung.^[1] 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.^[2] This radiographic density often has the shape of a scimitar, a type of curved sword.^[2]

Contents 1 Presentation 2 Diagnosis 3 Treatment 4 References 5 External links 6 Further reading

[edit] 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.

[edit] Diagnosis

The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, and more recently by CT angiography or MR Angiography.

[edit] 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.

[edit] References

1. ^ Sehgal, Arvind & Loughran-Fowlds, Allison, Clinical Brief: Scimitar Syndrome, medIND — Biomedical journals from India, <http://medind.nic.in/icb/t05/i3/icbt05i3p249.pdf>. Retrieved on 28 February 2008 
2. ^ ^{a b} Scimitar Syndrome, Children's Hospital Boston, <http://www.childrenshospital.org/az/Site2165/mainpageS2165P0.html>. Retrieved on 28 February 2008 

[edit] External links

• Holt, S & Berdon, WE (February 11, 2004), “Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome”, Pediatric radiology: 409-13, ISSN 0301-0449, <http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=14872299&query_hl=2&itool=pubmed_DocSum> 

[edit] 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.

v • d • e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747) Cardiac chambers and connections Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo) Cardiac septa Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome Valvular heart disease/ heart chambers Right: pulmonary and tricuspid valves pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly Left: aortic and mitral valves aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome Other congenital malformations of heart Dextrocardia - Levocardia - Cor triatriatum Vascular malformation Great arteries aorta: Patent ductus arteriosus - Aortic coarctation - Interrupted aortic arch - Overriding aorta - Aneurysm of sinus of Valsalva - Vascular ring pulmonary artery: Pulmonary atresia Great veins superior vena cava: Persistent left superior vena cava pulmonary vein: Total anomalous pulmonary venous connection - Scimitar syndrome Other Arteriovenous malformation (Cerebral arteriovenous malformation) See also non-congenital conditions (I, 390-459)