Scimitar syndrome

Scimitar syndrome
Chest MR angiography in a patient with scimitar syndrome
Classification and external resources
Specialty	medical genetics
ICD-10	Q26.8
ICD-9-CM	747.49
MeSH	D012587

Scimitar syndrome, or pulmonary venolobar syndrome, is a rare congenital heart defect characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium).^[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

Chest x-ray of a five-year-old girl with Scimitar syndrome. The heart (blue outline) is shifted into the right half of the chest, and the anomalous pulmonary venous return (red) has a shape reminiscent of a Scimitar.

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

1. Sehgal, Arvind; Loughran-Fowlds, Allison, Clinical Brief: Scimitar Syndrome (PDF), medIND — Biomedical journals from India, retrieved 2008-02-28
2. Scimitar Syndrome, Children's Hospital Boston, retrieved 2008-02-28

External links

• Holt, PD; Berdon, WE; Marans, Z; Griffiths, S; Hsu, D (February 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. PMID 14872299.

Further reading

• A case of Scimitar Syndrome

• Halasz NA, Halloran KH, Liebow AA (November 1956). "Bronchial and arterial anomalies with drainage of the right lung into the inferior vena cava". Circulation 14 (5): 826–46. doi:10.1161/01.cir.14.5.826. PMID 13374858.
• Kramer U, Dörnberger V, Fenchel M, Stauder N, Claussen CD, Miller S (December 2003). "Scimitar syndrome: morphological diagnosis and assessment of hemodynamic significance by magnetic resonance imaging". Eur Radiol 13 (Suppl 4): L147–50. doi:10.1007/s00330-003-2061-y. PMID 15018181.
• Brown JW, Ruzmetov M, Minnich DJ, et al. (February 2003). "Surgical management of scimitar syndrome: an alternative approach". J. Thorac. Cardiovasc. Surg. 125 (2): 238–45. doi:10.1067/mtc.2003.113. PMID 12579091.