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A Case of Variant Scimitar SyndromeA Meandering Pulmonary Vein in Scimitar Syndrome

Antoine Legras, MD; Claude Guinet, MD; Marco Alifano, MD, PhD, FCCP; Alain Lepilliez, MD; Jean-François Régnard, MD
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From the Service de Chirurgie Thoracique (Drs Legras, Alifano, Lepilliez, and Régnard), and the Service de Radiologie (Dr Guinet), Hôpital Hôtel-Dieu, Paris, France.

Correspondence to: Antoine Legras, MD, Service de Chirurgie Thoracique, Hôtel-Dieu, 1, Place du Parvis Notre-Dame, 75181 Paris, France; e-mail: le_ant@hotmail.fr


Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2012;142(4):1039-1041. doi:10.1378/chest.11-2732
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Scimitar syndrome is one of the large congenital pulmonary venolobar syndromes and is defined as hypogenetic lung associated with partial anomalous pulmonary venous return. We report the case of a 25-year-old man with complex and exceptional variant scimitar syndrome. A chest CT scan with three-dimensional (3-D) reconstruction led us to identify hypoplastic right lung with homolateral hemidiaphragm agenesis and hypogenetic right pulmonary artery. There was a large and sinuous systemic arterial supply and anomalous venous return directed into the left atrium (venous return being usually directed into the right atrium or inferior vena cava in scimitar syndrome). Hyperoxia test showed no shunt. This variant scimitar syndrome has been previously reported and anomalous venous return called “meandering” pulmonary vein. Diagnosis and management of these patients with complex congenital anomalies are difficult because of their exceptional condition. Chest CT scan with 3-D reconstruction offers an accurate noninvasive diagnosis.

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