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Budd-Chiari syndrome: A rare association of Scimitar syndrome

  • Author Footnotes
    1 Xiao Li and Yunfei Ling contributed equally to this work.
    Xiao Li
    Footnotes
    1 Xiao Li and Yunfei Ling contributed equally to this work.
    Affiliations
    Department of Cardiovascular Surgery, Pediatric Heart Center, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041, China
    Search for articles by this author
  • Author Footnotes
    1 Xiao Li and Yunfei Ling contributed equally to this work.
    Yunfei Ling
    Footnotes
    1 Xiao Li and Yunfei Ling contributed equally to this work.
    Affiliations
    Department of Cardiovascular Surgery, Pediatric Heart Center, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041, China
    Search for articles by this author
  • Ke Lin
    Affiliations
    Department of Cardiovascular Surgery, Pediatric Heart Center, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041, China
    Search for articles by this author
  • Shuhua Luo
    Correspondence
    Corresponding author.
    Affiliations
    Department of Cardiovascular Surgery, Pediatric Heart Center, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041, China
    Search for articles by this author
  • Author Footnotes
    1 Xiao Li and Yunfei Ling contributed equally to this work.
Published:October 15, 2021DOI:https://doi.org/10.1016/j.jcct.2021.09.006
      A 3-year-old asymptomatic girl was noted to have a heart murmur. Chest X-ray showed a curvilinear shadow along the right heart border (Fig. 1A) characteristic of the Scimitar sign. Echocardiography revealed a Scimitar vein (SV) draining the entire right lung to the inferior vena cava (IVC) which was stenosed at the junction with SV (Fig. 1B; arrow). CT angiography and hepatic venography revealed a 1.5 cm-long segment of stenosis in the IVC proximal to the insertion of the SV, resulting in outflow obstruction of short hepatic veins (HVs) with resultant intrahepatic veno-venous collaterals which coursed to the IVC above the level of stenosis (Fig. 2). No associated hepatic parenchymal changes were identified, and the liver function was normal. Through cardiac catheterization (supplementary video), she was confirmed to have pulmonary arterial hypertension (32 ​mmHg mean), and SV hypertension (23 ​mmHg mean).
      Fig. 1
      Fig. 1A. Chest X-ray showed “Scimitar sign” along the right heart border (arrowheads). B. Scimitar vein draining to the inferior vena cava, which was stenosed at the junction (arrow).
      Fig. 2
      Fig. 2Enhanced CT of the heart and hepatic veins. A. The entire right lung was drained into the inferior vena cava (IVC) through the Scimitar vein (arrowheads). The IVC was significantly narrowed at the junction (arrow). B to D. Multiple intrahepatic veno-venous collaterals shunted into the right, middle and left hepatic veins connected to the IVC above the level of stenosis (arrow). RA, right atrium; HV, hepatic vein; IVC, inferior vena cava.

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