Chest
Volume 142, Issue 4, October 2012, Pages 1039-1041
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Selected Reports
A Case of Variant Scimitar Syndrome

https://doi.org/10.1378/chest.11-2732Get rights and content

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.

Section snippets

Case Report

In February 2007, a 25-year-old man complained of dyspnea classified as New York Heart Association II. He was a current smoker (10 pack-years), reporting one episode of hemoptysis after physical effort and pneumonia 5 years ago. Standard radiography showed right hilar anomalies. Spirometry showed that FEV1 was 2.6 L (56% predicted) and FVC was 3.56 L (64% predicted). FEV1/FVC, forced expiratory flow at 75%, forced expiratory flow at 25% to 75%, and residual volume were 80%, 45%, 39%, and 124%

Discussion

The patient presented dyspnea related to restrictive syndrome, tobacco intake, and lack of physical activity. Hemoptysis was probably related to systemic vascularization, although in this case, systemic arterial supply was not nearby the bronchi.

Description and classification of these anatomic anomalies is difficult. This is a case of scimitar syndrome, as defined by hypogenetic lung with PAPVR. However, anomalous venous drainage is not into the IVC, as usually described in scimitar syndrome,

Acknowledgments

Financial/nonfinancial disclosures: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Other contributions: We thank Sylvain Ordureau, Useful Progress, and Paris-Descartes University for allowing us to use the Volviz software for three-dimensional reconstruction. We also thank Sophie Thébaud for correcting the English.

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