Scimitar syndrome (lungs)

Chest X-ray
of a five-year-old girl with wikipedia:Scimitar syndrome
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Scimitar
syndrome chest CT
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Anomalous
pulmonary venous drainage: a pictorial essay with a CT focus. Scimitar Syndrome. a Coronal oblique maximum intensity projection (MIP) CT images. A large right sided pulmonary vein is seen supradiaphragmatic inferior vena cava (IVC) in a patient with Scimitar Syndrome. b Axial CT image of same patient as in (a). The white arrow indicates the anomalous right pulmonary vein. The asymmetry of the pulmonary vasculature provides a clue to identifying the abnormality. The right hemidiaphragm is elevated consistent with hypoplasia of the right lower lobe, a common association with anomalous pulmonary venous drainage. c Chest radiograph of a paediatric patient, demonstrating a tubular opacity in the right paracardiac region consistent with a scimitar vein (arrow). d Coronal MIP CT image showing a large right pulmonary vein draining into the infradiaphragmatic IVC
jcongenitalcardiology.biomedcentral.com • CC-by-4.0
Scimitar
syndrome chest xray
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A case of
scimitar syndrome in a young woman. At the basal pyramid of the lower lobe two abnormal contiguous vessels can be identified, a threadlike vein draining into the inferior caval vein and a small artery originating directly from the abdominal aorta.
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A case of
scimitar syndrome in a young woman. Diffuse opacity of the right lung with dextroposition of the heart.
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Imaging of
congenital lung diseases presenting in the adulthood: a pictorial review. A 25-year-old female with scimitar syndrome. Chest X-ray (a) and coronal CT image (b) demonstrate right lung hypoplasia and curvilinear tubular opacity joining the inferior vena cava above the medial aspect of the right hemidiaphragm (arrows). 3D reconstruction image obtained with the cinematic volume-rendering-technique shows (c) the way of the anomalous pulmonary vein (arrows)
insightsimaging.springeropen.com • CC-by-4.0
Partial
anomalous pulmonary venous return • Scimitar syndrome - Ganzer Fall bei Radiopaedia
Tatco, V. Scimitar syndrome. Case study, Radiopaedia.org. (accessed on 20 Oct 2022) https://doi.org/10.53347/rID-43057 • CC by-nc-sa 3.0 (modified)
Scimitar
syndrome (lungs) • Infracardiac partial anomalous pulmonary venous return (scimitar syndrome) - Ganzer Fall bei Radiopaedia
Ali S, Infracardiac partial anomalous pulmonary venous return (scimitar syndrome). Case study, Radiopaedia.org (Accessed on 01 Dec 2022) https://doi.org/10.53347/rID-52671 • CC by-nc-sa 3.0 (modified)
Pictorial
review of the pulmonary vasculature: from arteries to veins. Scimitar syndrome. a Conceptual illustration of scimitar syndrome. b Coronal contrast-enhanced CT of an adult patient with scimitar syndrome showing the entire right lung pulmonary venous return (white arrows) draining into the inferior vena cava (black arrow)
insightsimaging.springeropen.com • CC-by-4.0
Correction of
the scimitar syndrome, a rare cardiac venous anomaly, leading to Budd–Chiari syndrome: a case report. Scimitar sign. Coronal contrast-enhanced computed tomography shows the anomalous drainage of the right pulmonary vein into the inferior vena cava, representing the scimitar sign on conventional radiography.
jmedicalcasereports.biomedcentral.com • CC-by-4.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Scimitar syndrome, also known as hypogenetic lung syndrome,is characterized by a hypoplastic lung that is drained by an anomalous pulmonary vein into the systemic venous system. It is a type of partial anomalous pulmonary venous return and is one of the several findings in congenital pulmonary venolobar syndrome.

Clinical presentation

Infants present with signs of heart failure, which may be due to associated congenital heart disease . Adults are most frequently asymptomatic but may have recurrent pulmonary infection or dyspnea on exertion.

Pathology

It is essentially a combination of pulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR). It almost exclusively occurs on the right side.

Haemodynamically, there is an acyanotic left to right shunt. The anomalous vein usually drains into:

In some variants, the abnormal lung segment may receive arterial blood supply from the aorta or its branches . Some cases may show the anomalous systemic arteries supplying an area of associated pulmonary sequestration .

Associations

Radiographic features

The diagnosis is made by transthoracic or transesophageal echocardiography, angiography, or by CT or MR angiography.

Plain radiograph

Chest radiographic findings are that of a small, hypoplastic 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 may be blurred.

Treatment and prognosis

Surgical correction should be considered in the presence of significant left to right shunting and pulmonary hypertension. This involves the creation of an interatrial baffle to redirect the pulmonary venous return into the left atrium. Alternatively, the anomalous vein can be re-implanted directly into the left atrium.

Complications

The presence of a left-to-right shunt may lead to the development of pulmonary hypertension and Eisenmenger phenomenon.

History and etymology

The term was first given by C A Neill et al. in 1960 .

Differential diagnosis

General imaging differential considerations include:

See also

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Scimitar-Syndrom:
Lungenvenenfehlmündung
 
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Lungensequester
 
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Lungenhypoplasie
 
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totale
Lungenvenenfehlmündung
pediatricimaging.org • CC-by-nc-sa 4.0
meandernde
Pulmonalvenen
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