Presentation
Fever, dyspnea and cough for 10 days.
Patient Data
cardiac dextroposition
curvilinear opacity in the right lung extending from the mid zone towards the medial border of the right hemidiaphragm (confirmed to be the scimitar vein draining into the IVC on CT)
ovoid structure on the medial side of the right middle lung (confirmed on CT to be the main pulmonary trunk)
right lung smaller than the left, as evidenced by right hemidiaphragm elevation compared to the left and rightward displacement of the mediastinum.
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two anomalous pulmonary veins located in the right lung that both drain into the IVC
main scimitar vein is located anteriorly and superiorly relative to the second anomalous vein
the smaller vein is partly calcified
cardiac dextroposition and right lung hypoplasia
hyparterial right main bronchus with abnormal branching pattern
bronchial wall thickening and mild bronchiectasis in the right lung
left pulmonary artery asymmetrically larger than the right (best seen in the coronal plane)
focal consolidation in the left upper lobe, consistent with pneumonia
Case Discussion
Scimitar syndrome most commonly presents with one anomalous pulmonary vein draining into the IVC. This case is an unusual variant in which the patient has two anomalous veins that drain into the IVC: one classic scimitar vein and another smaller vessel. Heart size was normal and there was no evidence of chamber dilatation.
Despite having an extra vessel, the patient was asymptomatic and denied having any history of chronic shortness of breath or fatigue. This is likely because the volume of blood being drained from the two vessels is still not significant enough to cause a severe left-to-right shunt. Her presentation to the ED was due to pneumonia, and her diagnosis of scimitar syndrome was incidental. Her condition improved with antibiotics, and she was discharged the next day.
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