Fany Corvol, Sébastien Hascoet, Emmanuel Le Bret, Nadia Nathan
Sorbonne University. Université Paris-Saclay, Marie-Lannelongue Surgical Centre. Ramsay Santé, Clinique du Sport. Sorbonne University.
France
British Medical Journal Case Reports
BMJ Case Rep 2024; 17:
DOI: 10.1136/bcr-2024-259955
Abstract
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.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
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