Scimitar syndrome
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| Scimitar syndrome Classification and external resources | |
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| ICD-10 | Q26.8 |
| ICD-9 | 747.49 |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-525-6884
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Synonyms and related keywords: Venolobar syndrome, hypogenetic lung syndrome.
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.
Chest x-ray: Scimitar Syndrome (Image courtesy of Dr Donna D'Souza) |
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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.
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
