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CASE LETTER
Year : 2021  |  Volume : 38  |  Issue : 3  |  Page : 288-289  

The sword and the shunt: Scimitar syndrome


Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA

Date of Submission25-Jun-2020
Date of Acceptance12-Jul-2020
Date of Web Publication30-Apr-2021

Correspondence Address:
Arzu Canan
Department of Radiology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/lungindia.lungindia_529_20

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How to cite this article:
Canan A, Batra K. The sword and the shunt: Scimitar syndrome. Lung India 2021;38:288-9

How to cite this URL:
Canan A, Batra K. The sword and the shunt: Scimitar syndrome. Lung India [serial online] 2021 [cited 2021 May 8];38:288-9. Available from: https://www.lungindia.com/text.asp?2021/38/3/288/315297



Sir,

A 50-year-old female was presented to the emergency department with worsening shortness of breath for the last 5 days. Medical history was unremarkable other than hypertension and obstructive sleep apnea. Chest radiography [Figure 1] revealed elevated right hemidiaphragm with a mass-like opacity in the right lower zone obscuring cardiac silhouette. Furthermore, a vertically oriented linear opacity in the right lower zone was noted. Computed tomography angiography (CTA) of the chest demonstrated that right-sided pulmonary veins were draining in a vertical vein which emptying into inferior vena cava (IVC) [Figure 2] and [Figure 3]. Right-sided cardiac chambers and main pulmonary artery were dilated. Hypoplastic right lung and elevated right hemidiaphragm were also present. The constellation of the findings is consistent with Scimitar syndrome. The patient underwent a right heart catheterization that showed a significant left-to-right shunt with a Qp/Qs ratio of 1.8. Nonemergent surgical repair was planned.
Figure 1: Posteroanterior chest radiograph demonstrates elevated right hemidiaphragm, mass-like opacity in the right lower zone obscuring cardiac silhouette. Furthermore, a vertically oriented linear opacity in the right lower zone was noted (arrow)

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Figure 2: Coronal oblique maximum intensity projection computed tomography angiography image demonstrates that all of the right-sided pulmonary veins are draining into the inferior vena cava via a vertical vein (arrow), creating a left-to-right shunt. Note the normal communication between left atrium and left-sided pulmonary veins

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Figure 3: 3D volume-rendered computed tomography angiography image (posterior view) shows the absence of communication between left atrium and right-sided pulmonary veins. The right-sided pulmonary veins are emptying into the inferior vena cava. Also note the hypoplastic right lung. LA: Left atrium, LPA: Left pulmonary artery, RPA: Right pulmonary artery, RPV: Right pulmonary veins

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Scimitar syndrome, also known as hypogenetic lung syndrome, is a congenital partial anomalous pulmonary venous return, which results in left-to-right shunt and eventually pulmonary hypertension and right heart failure. Most commonly, the pulmonary veins of the right lower lobe empty in a vertical vein which drains into the IVC. Although the anomalous vein might be seen on radiography as a tubular opacity parallel to the right heart border creating a shape of Turkish sword (scimitar), CTA provides accurate identification of the pulmonary venous anatomy and draining vein that is crucial for the planning of surgical treatment.[1],[2]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Olson MA, Becker GJ. The Scimitar syndrome: CT findings in partial anomalous pulmonary venous return. Radiology 1986;159:25-6.  Back to cited text no. 1
    
2.
Lyen S, Wijesuriya S, Ngan-Soo E, Mathias H, Yeong M, Hamilton M, et al. Anomalous pulmonary venous drainage: A pictorial essay with a CT focus. J Congenit Cardiol 2017;1:1-3.  Back to cited text no. 2
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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