UMMS Affiliation

Department of Radiology; UMass Memorial Children's Medical Center

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Cardiovascular Diseases | Diagnosis | Pathological Conditions, Signs and Symptoms | Radiology | Respiratory Tract Diseases


(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0-4 = no disease-atresia) and lung segments (0-3 = unaffected-severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score > 11, lung cysts, and total lung severity score > 6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity.


computed tomography, perihilar induration, pulmonary cysts, pulmonary vein atresia, pulmonary vein compression, pulmonary vein stenosis, pulmonary venous collaterals

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Copyright © 2021 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (

DOI of Published Version



Sena L, Callahan R, Sleeper LA, Beroukhim RS. Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis. Children (Basel). 2021 May 17;8(5):402. doi: 10.3390/children8050402. PMID: 34067561; PMCID: PMC8155841. Link to article on publisher's site

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Children (Basel, Switzerland)

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Creative Commons Attribution 4.0 License
This work is licensed under a Creative Commons Attribution 4.0 License.