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Scimitar syndrome with vein stenosis in an infant
  1. Fany Corvol1,
  2. Sébastien Hascoet2,
  3. Emmanuel Le Bret3 and
  4. Nadia Nathan1,4
  1. 1 Pediatric Pulmunology and Reference Center for Rare Lung Diseases (RespiRare), Sorbonne University, Paris, France
  2. 2 Congenital Heart Diseases Department, INSERM UMR-S 999, Université Paris-Saclay, Marie-Lannelongue Surgical Centre, Le Plessis-Robinson, France
  3. 3 Ramsay Santé, Clinique du Sport, Paris, France
  4. 4 Laboratory of childhood genetic diseases, UMR S-933, INSERM, Paris, France
  1. Correspondence to Professor Nadia Nathan; nadia.nathan{at}


Scimitar syndrome is characterised by right lung hypoplasia and abnormal pulmonary venous return, known as the ‘scimitar vein’. We report the case of an infant girl with scimitar syndrome who developed a severe respiratory distress mimicking asthma. Pulmonary hypertension (PH) was diagnosed, attributed to scimitar vein stenosis and a left-to-right shunt. Scimitar vein stenosis, a rare complication of scimitar syndrome, can lead to severe PH, highlighting the importance of prompt management in specialised care centres.

  • Anatomic Variation
  • Cardiovascular medicine
  • Respiratory system
  • Paediatrics
  • Pulmonary hypertension

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  • Contributors The following authors were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content—FC, SH, ELB and NN. The following authors gave final approval of the manuscript—FC, SH, ELB and NN.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.