Scimitar syndrome (also known as pulmonary venolobar syndrome or hypogenetic lung syndrome) 4 is characterised by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system.
Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into
- congenital heart disease (e.g. ASD, VSD, tetralogy of Fallot, PDA)
- ipsilateral diaphragmatic anomalies (e.g. accessory diaphragm, diaphragmatic hernia
- localised bronchiectasis
- horseshoe lung
- vertebral anomalies (e.g. hemivertebrae 4)
- genitourinary tract abnormalities 4
The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR angiography.
Chest radiographic 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”). The right heart border maybe blurred.
A potential complication that could develop is development or pulmonary hypertension an Eisenmenger physiology.
Surgical correction should be considered in the presence of significant left to right shunting 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.
The term was first given by C A Neill et.al in 1960 5.
General imaging differential considerations include
- 1. Dahnert WF. Radiology Review Manual. (2011) ISBN:1609139437. Read it at Google Books - Find it at Amazon
- 2. Melduni RM, Mookadam F, Mulligan ME. Scimitar syndrome: another one for the radiologists. Radiology. 2006;241 (2): 629. doi:10.1148/radiol.2412051952 - Pubmed citation
- 2. Olson MA, Becker GJ. The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return. Radiology. 1986;159 (1): 25-6. Radiology (citation) - Pubmed citation
- 4. Berrocal T, Madrid C, Novo S et-al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24 (1): e17. doi:10.1148/rg.e17 - Pubmed citation
- 5. Schramel FM, Westermann CJ, Knaepen PJ et-al. The scimitar syndrome: clinical spectrum and surgical treatment. Eur. Respir. J. 1995;8 (2): 196-201. Eur. Respir. J. (link) - Pubmed citation
- 6. Baskar karthekeyan R, Saldanha R, Sahadevan MR et-al. Scimitar syndrome: experience with 6 patients. Asian Cardiovasc Thorac Ann. 2009;17 (3): 266-71. doi:10.1177/0218492309104750 - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Pulmonary venolobar syndrome||✓|
|Congenital pulmonary venolobar syndrome (CVLS)||✗|
|Hypogenetic lung syndrome||✓|