Scimitar syndrome

Jump to: navigation, search
Scimitar syndrome
Scimitar-syndrome.jpg
ICD-10 Q26.8
ICD-9 747.49

WikiDoc Resources for Scimitar syndrome

Articles

Most recent articles on Scimitar syndrome

Most cited articles on Scimitar syndrome

Review articles on Scimitar syndrome

Articles on Scimitar syndrome in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Scimitar syndrome

Images of Scimitar syndrome

Photos of Scimitar syndrome

Podcasts & MP3s on Scimitar syndrome

Videos on Scimitar syndrome

Evidence Based Medicine

Cochrane Collaboration on Scimitar syndrome

Bandolier on Scimitar syndrome

TRIP on Scimitar syndrome

Clinical Trials

Ongoing Trials on Scimitar syndrome at Clinical Trials.gov

Trial results on Scimitar syndrome

Clinical Trials on Scimitar syndrome at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Scimitar syndrome

NICE Guidance on Scimitar syndrome

NHS PRODIGY Guidance

FDA on Scimitar syndrome

CDC on Scimitar syndrome

Books

Books on Scimitar syndrome

News

Scimitar syndrome in the news

Be alerted to news on Scimitar syndrome

News trends on Scimitar syndrome

Commentary

Blogs on Scimitar syndrome

Definitions

Definitions of Scimitar syndrome

Patient Resources / Community

Patient resources on Scimitar syndrome

Discussion groups on Scimitar syndrome

Patient Handouts on Scimitar syndrome

Directions to Hospitals Treating Scimitar syndrome

Risk calculators and risk factors for Scimitar syndrome

Healthcare Provider Resources

Symptoms of Scimitar syndrome

Causes & Risk Factors for Scimitar syndrome

Diagnostic studies for Scimitar syndrome

Treatment of Scimitar syndrome

Continuing Medical Education (CME)

CME Programs on Scimitar syndrome

International

Scimitar syndrome en Espanol

Scimitar syndrome en Francais

Business

Scimitar syndrome in the Marketplace

Patents on Scimitar syndrome

Experimental / Informatics

List of terms related to Scimitar syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-in-Chief: Keri Shafer, M.D. [2]


Synonyms and related keywords: Venolobar syndrome, hypogenetic lung syndrome.

Overview

Scimitar syndrome (a variant of Partially Anomalous Pulmonary Venous Return or PAPVR) is a rare congenital heart defect is characterized by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system. It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return. It almost exclusively occurs on the right side. The hemodynamics are that of an acyanotic left to right shunt. The anomalous vein most commonly drains into the IVC, right atrium or portal vein. The lung is frequently perfused by the aorta, but the bronchial tree is still connected and thus the lung is not sequestered. CXR findings are that of a small lung with ipsilateral mediastinal shift, and in one third of cases the anomalous draining vein may be seen as a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). Scimitar syndrome is associated with congenital heart disease, ipsilateral diaphragmatic anomalies and vertebral anomalies.

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 dextrocardia, pulmonary artery hypoplasia and pulmonary arterial 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.

External links

  • [3]
  • 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.



Linked-in.jpg