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Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis

Background Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional...

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Published in:Pediatric radiology 2023-08, Vol.53 (9), p.1874-1884
Main Authors: O’Callaghan, Barry, Zablah, Jenny E., Weinman, Jason P., Englund, Erin K., Morgan, Gareth J., Ivy, D. Dunbar, Frank, Benjamin S., Mong, David Andrew, Malone, LaDonna J., Browne, Lorna P.
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container_title Pediatric radiology
container_volume 53
creator O’Callaghan, Barry
Zablah, Jenny E.
Weinman, Jason P.
Englund, Erin K.
Morgan, Gareth J.
Ivy, D. Dunbar
Frank, Benjamin S.
Mong, David Andrew
Malone, LaDonna J.
Browne, Lorna P.
description Background Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. Objective To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. Materials and methods A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (
doi_str_mv 10.1007/s00247-023-05673-y
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Dunbar ; Frank, Benjamin S. ; Mong, David Andrew ; Malone, LaDonna J. ; Browne, Lorna P.</creator><creatorcontrib>O’Callaghan, Barry ; Zablah, Jenny E. ; Weinman, Jason P. ; Englund, Erin K. ; Morgan, Gareth J. ; Ivy, D. Dunbar ; Frank, Benjamin S. ; Mong, David Andrew ; Malone, LaDonna J. ; Browne, Lorna P.</creatorcontrib><description>Background Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. Objective To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. Materials and methods A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (&lt; 18 years) who had a history of prematurity (&lt; 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients’ CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. Results A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1–4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased ( n  = 13/17, 35%, n  = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. Conclusion Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.</description><identifier>ISSN: 1432-1998</identifier><identifier>ISSN: 0301-0449</identifier><identifier>EISSN: 1432-1998</identifier><identifier>DOI: 10.1007/s00247-023-05673-y</identifier><identifier>PMID: 37106091</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer Berlin Heidelberg</publisher><subject>Angiography ; Chest ; Computed tomography ; Correlation ; Dysplasia ; Heart diseases ; Hypertension ; Imaging ; Lung diseases ; Medicine ; Medicine &amp; Public Health ; Neonates ; Neuroradiology ; Nuclear Medicine ; Oncology ; Original Article ; Parenchyma ; Patients ; Pediatrics ; Phenotypes ; Pulmonary hypertension ; Radiology ; Soft tissues ; Stenosis ; Ultrasound ; Veins ; Veins &amp; arteries</subject><ispartof>Pediatric radiology, 2023-08, Vol.53 (9), p.1874-1884</ispartof><rights>The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</rights><rights>2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c326t-4121effcd95a4558e1586f70fe10d78d5717b4833151c0fb44017e597f6fbe5e3</cites><orcidid>0000-0002-8080-5276</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37106091$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>O’Callaghan, Barry</creatorcontrib><creatorcontrib>Zablah, Jenny E.</creatorcontrib><creatorcontrib>Weinman, Jason P.</creatorcontrib><creatorcontrib>Englund, Erin K.</creatorcontrib><creatorcontrib>Morgan, Gareth J.</creatorcontrib><creatorcontrib>Ivy, D. Dunbar</creatorcontrib><creatorcontrib>Frank, Benjamin S.</creatorcontrib><creatorcontrib>Mong, David Andrew</creatorcontrib><creatorcontrib>Malone, LaDonna J.</creatorcontrib><creatorcontrib>Browne, Lorna P.</creatorcontrib><title>Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis</title><title>Pediatric radiology</title><addtitle>Pediatr Radiol</addtitle><addtitle>Pediatr Radiol</addtitle><description>Background Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. Objective To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. Materials and methods A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (&lt; 18 years) who had a history of prematurity (&lt; 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients’ CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. Results A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1–4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased ( n  = 13/17, 35%, n  = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. Conclusion Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.</description><subject>Angiography</subject><subject>Chest</subject><subject>Computed tomography</subject><subject>Correlation</subject><subject>Dysplasia</subject><subject>Heart diseases</subject><subject>Hypertension</subject><subject>Imaging</subject><subject>Lung diseases</subject><subject>Medicine</subject><subject>Medicine &amp; Public Health</subject><subject>Neonates</subject><subject>Neuroradiology</subject><subject>Nuclear Medicine</subject><subject>Oncology</subject><subject>Original Article</subject><subject>Parenchyma</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Phenotypes</subject><subject>Pulmonary hypertension</subject><subject>Radiology</subject><subject>Soft tissues</subject><subject>Stenosis</subject><subject>Ultrasound</subject><subject>Veins</subject><subject>Veins &amp; arteries</subject><issn>1432-1998</issn><issn>0301-0449</issn><issn>1432-1998</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><recordid>eNp9kMFO3DAQhi1UBHTbF-gBWeqFS8pMHMfJEa2grYTUC5yNNxnvZpXYwU6o9u0x7BaqHnryWP7mH8_H2BeEbwigLiNAXqgMcpGBLJXIdkfsDAuRZ1jX1Ye_6lP2McYtAAiJ4oSdCoVQQo1n7GHph3GeqOWTH_w6mHHTNXw0gVyz2Q2m5_3s1tx2ru3cOvLO8THQYKY5ULpY46bIf3fTho9zP3hnwo4_UaLiRM7HLn5ix9b0kT4fzgW7v7m-W_7Ibn99_7m8us0akZdTVmCOZG3T1tIUUlaEsiqtAksIrapaqVCtikoIlNiAXRUFoCJZK1vaFUkSC3axzx2Df5wpTnroYkN9bxz5Oeq8AlWjwBIS-vUfdOvn4NLvElXUqEqJMlH5nmqCjzGQ1WPohrSfRtAv_vXev07-9at_vUtN54foeTVQ-9byR3gCxB6I6cmtKbzP_k_sM8KakfQ</recordid><startdate>20230801</startdate><enddate>20230801</enddate><creator>O’Callaghan, Barry</creator><creator>Zablah, Jenny E.</creator><creator>Weinman, Jason P.</creator><creator>Englund, Erin K.</creator><creator>Morgan, Gareth J.</creator><creator>Ivy, D. 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Dunbar</au><au>Frank, Benjamin S.</au><au>Mong, David Andrew</au><au>Malone, LaDonna J.</au><au>Browne, Lorna P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis</atitle><jtitle>Pediatric radiology</jtitle><stitle>Pediatr Radiol</stitle><addtitle>Pediatr Radiol</addtitle><date>2023-08-01</date><risdate>2023</risdate><volume>53</volume><issue>9</issue><spage>1874</spage><epage>1884</epage><pages>1874-1884</pages><issn>1432-1998</issn><issn>0301-0449</issn><eissn>1432-1998</eissn><abstract>Background Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. Objective To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. Materials and methods A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (&lt; 18 years) who had a history of prematurity (&lt; 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients’ CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. Results A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1–4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased ( n  = 13/17, 35%, n  = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. Conclusion Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>37106091</pmid><doi>10.1007/s00247-023-05673-y</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0002-8080-5276</orcidid></addata></record>
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subjects Angiography
Chest
Computed tomography
Correlation
Dysplasia
Heart diseases
Hypertension
Imaging
Lung diseases
Medicine
Medicine & Public Health
Neonates
Neuroradiology
Nuclear Medicine
Oncology
Original Article
Parenchyma
Patients
Pediatrics
Phenotypes
Pulmonary hypertension
Radiology
Soft tissues
Stenosis
Ultrasound
Veins
Veins & arteries
title Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis
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