CTICasesScimitar Syndrome Variant (Right Upper Lobe PAPVR)
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Imaging findings
Echocardiography initially suggested dextrocardia (actually dextroversion). The patient (a young girl) has no right upper lobe, and the right superior pulmonary vein drains into the superior vena cava (SVC), representing a right upper partial anomalous pulmonary venous return (PAPVR). This is a variant of Scimitar syndrome, which classically involves hypoplastic right lung and anomalous drainage of the right inferior pulmonary vein. The patient also has a left SVC. Airway anatomy is difficult to assess fully but suggests no right upper lobe bronchus.
Key takeaways
This case illustrates a rare variant of partial anomalous pulmonary venous return (PAPVR) that falls within the spectrum of Scimitar syndrome. While classical Scimitar involves hypoplastic right lung and anomalous inferior pulmonary vein drainage, this patient exhibited no right upper lobe with anomalous right superior pulmonary vein drainage into the SVC, alongside other congenital anomalies like left SVC and dextroversion. Comprehensive imaging is needed to characterize such complex congenital anomalies.
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