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University Hospitals of Cleveland Case Western Reserve University School of Medicine Cleveland, OH 44106
Pulmonary vein anomalies are unusual but well-represented entities in the imaging literature. Partial anomalous pulmonary venous return, best characterized by the scimitar syndrome, is a well-recognized cause of a left-to-right shunt that may require surgical intervention. Less well described are pulmonary vein anomalies with normal venous drainage to the left atrium. We present the imaging findings of an anomalous unilateral single pulmonary vein mimicking a solitary pulmonary nodule. Multidetector CT was instrumental in depicting this unusual anatomic variant.
A 75-year-old man with a history of rheumatoid arthritis and coronary artery disease underwent preoperative chest radiography in preparation for total shoulder replacement. A 1-cm pulmonary nodule was noted in the lingular segment, which in retrospect had been present on a chest radiograph obtained in 1994 (Fig. 3A). Chest CT was performed for further evaluation. Although the CT study suggested an arteriovenous malformation because of a suboptimal contrast bolus and a 10-mm slice thickness, further characterization was not possible. The patient returned to our department for complete evaluation with multidetector CT. After a timing bolus was administered to optimize contrast enhancement of the lingular abnormality, the hilar region was dynamically imaged during a contrast injection at 4 mL/sec using a 2.5-mm slice thickness and a 1-mm reconstruction interval. Evaluation of the vascular abnormality showed it to be a left inferior pulmonary vein that failed to enter the left atrium in the expected location (Fig. 3B). Instead, the vein traveled anteriorly and superiorly to enter the superior pulmonary vein. Selective three-dimensional reconstruction of the pulmonary venous system optimally depicted this unusual abnormality (Figs. 3C).
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Congenital anomalies of the pulmonary veins have been classified by Remy-Jardin and Remy [1] as abnormal pulmonary venous drainage without abnormal course, abnormal venous return with abnormal course (scimitar syndrome), and abnormal course with normal connection. Our case illustrates this third type, a rare entity with, to our knowledge, fewer than 20 cases reported in the world literature. Goodman et al. [2] have described a meandering right pulmonary vein with normal venous drainage into the left atrium. Another group of researchers have also described this entity as mimicking the scimitar vein except for its normal venous connection to the left atrium [3]. Interestingly, these patients also had hypoplasia of the ipsilateral lung and pulmonary artery. Another isolated case report described the abnormal course of normally draining pulmonary veins associated with cor triatriatum of the left atrium [4]. The isolated nature of the pulmonary vein anomaly in our patient most closely resembles the description by Remy-Jardin and Remy of the rare juxtahilar type of unilateral single pulmonary vein. Recognition of the anatomy of this unusual anomaly is important: unlike pulmonary arteriovenous malformation or anomalous pulmonary venous return, the anomalous single pulmonary vein drains appropriately into the left atrium. Although extremely rare, this venous anomaly is important to recognize because it requires no further diagnostic or therapeutic intervention.
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  P. P. Agarwal, J. M. Seely, and F. R. Matzinger MDCT of Anomalous Unilateral Single Pulmonary Vein Am. J. Roentgenol., November 1, 2004; 183(5): 1241 - 1243. [Full Text] [PDF]
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