Scimitar syndrome
| Scimitar syndrome | |
|---|---|
| Specialty | Medical genetics  |
Scimitar syndrome, or pulmonary 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 Scimitar syndrome after 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]
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
- ^ Sehgal, Arvind; Loughran-Fowlds, Allison, Clinical Brief: Scimitar Syndrome (PDF), medIND — Biomedical journals from India, retrieved 2008-02-28
- ^ a b Scimitar Syndrome, Children's Hospital Boston, retrieved 2008-02-28
External links
- Holt, PD; Berdon, WE; Marans, Z; Griffiths, S; Hsu, D (February 11, 2004), "Scimitar vein draining to the left atrium and a historical review of the scimitar syndrome", Pediatric radiology, 34 (5): 409–13, doi:10.1007/s00247-004-1149-0, ISSN 0301-0449, PMID 14872299
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Further reading
- A case of Scimitar Syndrome
- 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.
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