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Congenital Unilateral Pulmonary Vein Atresia

Radiologic Findings in Three Adult Patients

Laura E. Heyneman1, Robert L. Nolan1,2, J. Kevin Harrison3 and H. Page McAdams1

1 Department of Radiology, Box 3808, Duke University Medical Center, Rm. 2523 Blue Zone South, Durham, NC 27710.
2 Present address: Department of Radiology, Queen's University at Kingston, c/o Kingston General Hospital, Kingston, Ontario, K7L 2V7 Canada.
3 Division of Cardiology, Department of Medicine, Duke University Medical Center, Durham, NC 27710.



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  		Fig. 1A. —Congenital unilateral pulmonary vein atresia in 25-year-old woman with dyspnea and pulmonary artery hypertension. Posteroanterior chest radiograph shows small right hemithorax and diminutive right pulmonary artery.

 


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  		Fig. 1B. —Congenital unilateral pulmonary vein atresia in 25-year-old woman with dyspnea and pulmonary artery hypertension. Contrast-enhanced CT scan (mediastinal window settings) reveals small right pulmonary artery (black arrows). Note confluent hilar and mediastinal soft tissue in azygoesophageal recess (white arrows) and surrounding right main bronchus. No evidence of bronchial obstruction is seen.

 


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  		Fig. 2A. —Congenital unilateral pulmonary vein atresia in 43-year-old woman with dyspnea and hemoptysis. Contrast-enhanced CT scan (mediastinal window settings) shows smooth left atrial wall at expected location of right inferior pulmonary vein (small arrows). Confluent mediastinal soft tissue extends anteriorly from azygoesophageal recess to surround, but not narrow, right middle lobe bronchus (large arrow).

 


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  		Fig. 3A. —Congenital unilateral pulmonary vein atresia in 27-year-old woman with hemoptysis and hematemesis. Thin-section CT scan (1.5-mm collimation, mediastinal window settings) reveals apparent esophageal wall thickening (black arrows). Also note transpleural collateral vessels (white arrows). Abnormal subcarinal soft tissue was also seen on cephalad images (not shown).

 


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  		Fig. 1C. —Congenital unilateral pulmonary vein atresia in 25-year-old woman with dyspnea and pulmonary artery hypertension. Contrast-enhanced CT scan (lung window) shows ground-glass attenuation throughout right lung. Note interlobular septal thickening (arrows).

 


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  		Fig. 3B. —Congenital unilateral pulmonary vein atresia in 27-year-old woman with hemoptysis and hematemesis. Axial T1-weighted spin-echo MR image (TR/TE, 750/20) through lower hemithorax reveals multiple flow voids in paraesophageal location (arrows), consistent with paraesophageal varices and corresponding to apparent esophageal wall thickening on CT. On gradient-echo images (not shown), flow-related enhancement was present in subcarinal region. RV = right ventricle, LV = left ventricle, IVC = inferior vena cava.

 


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  		Fig. 2B. —Congenital unilateral pulmonary vein atresia in 43-year-old woman with dyspnea and hemoptysis. Image from venous phase of pulmonary angiography reveals absence of right pulmonary veins (arrows on left). Note normal left pulmonary veins (arrows on right). LA = left atrium, LUPV=left upper lobe pulmonary vein, LLPV=left lower lobe pulmonary vein.

 


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  		Fig. 3C. —Congenital unilateral pulmonary vein atresia in 27-year-old woman with hemoptysis and hematemesis. Image from venous phase of capillary wedge angiography reveals termination of left pulmonary veins at left hilum (open arrow) and opacification of paraesophageal plexus (solid arrows). (Reprinted with permission from [2])

 


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  		Fig. 2C. —Congenital unilateral pulmonary vein atresia in 43-year-old woman with dyspnea and hemoptysis. Posterior 99mTc macroaggregated albumin perfusion scan reveals absence of perfusion to right lung. Right lung ventilation was normal (not shown). L = left lung, R = right lung.

 

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