Anomalous unilateral single pulmonary vein: Two cases mimicking arteriovenous malformations and a review of the literature

Abstract

Total anomalous pulmonary venous drainage is a rare congenital anomaly. It usually involves a pulmonary to systemic venous shunt and most cases have a septal defect in order to survive. Anomalous pulmonary venous drainage with pulmonary venous shunting is an extremely rare and entirely benign entity. We present two such cases, in which there was atresia of the left superior pulmonary vein and drainage via a tortuous collateral vein to the left inferior pulmonary vein. This collateral was mistaken on plain film and CT for a pulmonary arteriovenous malformation. Awareness of this anomalous unilateral single pulmonary vein and its radiological appearances may help in avoiding unnecessary pulmonary angiography.

Anomalous Unilateral Single Pulmonary Vein Mimicking Pulmonary Nodules on HRCT of the Lungs and Scimitar Syndrome on Chest Radiograph: Multidetector CT Findings

“…6 In the first report of the anomaly in two children presenting with recurrent pulmonary infections, symptoms were attributed to pressure effect of the abnormal vein on the middle lobe bronchus. 7 The presence of asthma in our case may be an incidental coincidence of the two abnormalities and to our knowledge have not been reported earlier.…”

“…As few as 20 cases have been reported in the literature, most of them initially misinterpreted as other abnormalities. [1][2][3][4][5][6][7][8] Imaging findings including multidetector computed tomography (MDCT) examination and differential diagnoses are discussed.…”

Asymptomatic Congenital Atresia of the Left Inferior Pulmonary Vein: A Case Report

“…1,2 Symptoms generally occur in early childhood and include recurrent pulmonary infections (34%), hemoptysis (24%) and exercise intolerance, 1 but the malformation can also be asymptomatic. 5,10,11 Diagnosis is drawn from different radiological exams. Thoracic radiography can evidence a small hemithorax with a small hilum, a small ipsilateral pulmonary artery, and deviation of the mediastum on the affected side.…”

A Case of Pulmonary Varix Associated with Superior Pulmonary Vein Occlusion

“…The occlusion of the right superior lobe branch of the pulmonary vein, clarified by multiplanar reformation of multislice CT, suggested that it was due to hypoplasia or occlusion of the branch, which caused vascular advancement as a collateral pathway simultaneously with the development of the interlobular region and secondarily formed the pulmonary varix, rather than due to abnormal distribution in the embryonic development of the pulmonary vein. Only a few cases of the pulmonary vein passing through the interlobular fissure have been reported by Kumazoe et al 4) and Hanson et al 5) The diagnosis of an abnormal blood vessel passing through the lung parenchyma and then thoracic cavity is important because it may cause hemothorax. 6,7) Only one case of varix accompanied by pulmonary venous occlusion diagnosed by angiography has previously been reported, 8) and no case directly imaged by multislice CT has been reported.…”