Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes

Purpose To characterise (1) the risk factors associated with inferior vena cava (IVC) atresia, (2) the radiographic and clinical presentations of deep vein thrombosis (DVT) in patients with IVC atresia, and (3) the treatment and outcome of DVT in patients with IVC atresia. Methods The electronic med...

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Veröffentlicht in:Cardiovascular and interventional radiology 2020, Vol.43 (1), p.37-45
Hauptverfasser: Mabud, Tarub S., Sailer, Anna M., Swee, Joshua K. Y., Tamboli, Mallika, Arendt, Victoria A., Jeon, Gyeong-Sik, An, Xiao, Cohn, David M., Kuo, William T., Hofmann, Lawrence V.
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container_issue 1
container_start_page 37
container_title Cardiovascular and interventional radiology
container_volume 43
creator Mabud, Tarub S.
Sailer, Anna M.
Swee, Joshua K. Y.
Tamboli, Mallika
Arendt, Victoria A.
Jeon, Gyeong-Sik
An, Xiao
Cohn, David M.
Kuo, William T.
Hofmann, Lawrence V.
description Purpose To characterise (1) the risk factors associated with inferior vena cava (IVC) atresia, (2) the radiographic and clinical presentations of deep vein thrombosis (DVT) in patients with IVC atresia, and (3) the treatment and outcome of DVT in patients with IVC atresia. Methods The electronic medical record was systematically reviewed for thrombotic risk factors in patients who presented with lower-extremity DVT ( n  = 409) at a single centre between 1996 and 2017. Patients with IVC atresia were identified based on imaging and chart review. Differences in demographics and thrombotic risk factors between patients with and without IVC atresia were statistically assessed. Extent and chronicity of DVT on imaging, clinical presentation, treatment, and outcomes were evaluated for all patients with IVC atresia. Results 4.2% of DVT patients (17/409) were found to have IVC atresia; mean age at diagnosis was 25.5 ± 9.4 years. The rate of heritable thrombophilia was significantly higher in patients with IVC atresia compared to patients without IVC atresia (52.9% vs. 17.9%, p  
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Y. ; Tamboli, Mallika ; Arendt, Victoria A. ; Jeon, Gyeong-Sik ; An, Xiao ; Cohn, David M. ; Kuo, William T. ; Hofmann, Lawrence V.</creator><creatorcontrib>Mabud, Tarub S. ; Sailer, Anna M. ; Swee, Joshua K. Y. ; Tamboli, Mallika ; Arendt, Victoria A. ; Jeon, Gyeong-Sik ; An, Xiao ; Cohn, David M. ; Kuo, William T. ; Hofmann, Lawrence V.</creatorcontrib><description>Purpose To characterise (1) the risk factors associated with inferior vena cava (IVC) atresia, (2) the radiographic and clinical presentations of deep vein thrombosis (DVT) in patients with IVC atresia, and (3) the treatment and outcome of DVT in patients with IVC atresia. Methods The electronic medical record was systematically reviewed for thrombotic risk factors in patients who presented with lower-extremity DVT ( n  = 409) at a single centre between 1996 and 2017. Patients with IVC atresia were identified based on imaging and chart review. Differences in demographics and thrombotic risk factors between patients with and without IVC atresia were statistically assessed. Extent and chronicity of DVT on imaging, clinical presentation, treatment, and outcomes were evaluated for all patients with IVC atresia. Results 4.2% of DVT patients (17/409) were found to have IVC atresia; mean age at diagnosis was 25.5 ± 9.4 years. The rate of heritable thrombophilia was significantly higher in patients with IVC atresia compared to patients without IVC atresia (52.9% vs. 17.9%, p  &lt; 0.0001). There were bilateral DVT in 70.6% of IVC atresia patients; DVT was chronic in 41.2% and acute on chronic in 58.8%. Pre-intervention Villalta scores were 13.9 ± 9.8 in the left limb and 8.5 ± 7.0 in the right limb. DVT in IVC atresia patients was typically treated with catheter-directed thrombolysis followed by stent placement, achieving complete or partial symptom resolution in 78.6% of cases. Conclusion Thrombotic risk factors such as heritable thrombophilia are associated with IVC atresia. IVC atresia patients can experience high burdens of lower-extremity thrombotic disease at a young age which benefit from endovascular treatment. Level of Evidence Level 4.</description><identifier>ISSN: 0174-1551</identifier><identifier>EISSN: 1432-086X</identifier><identifier>DOI: 10.1007/s00270-019-02353-z</identifier><identifier>PMID: 31650242</identifier><language>eng</language><publisher>New York: Springer US</publisher><subject>Adolescent ; Adult ; Cardiology ; Cardiovascular system ; Catheters ; Clinical Investigation ; Computed Tomography Angiography ; Demographics ; Demography ; Electronic health records ; Electronic medical records ; Female ; Humans ; Imaging ; Implants ; Lower Extremity - blood supply ; Magnetic Resonance Angiography ; Male ; Medical imaging ; Medical instruments ; Medicine ; Medicine &amp; Public Health ; Nuclear Medicine ; Patients ; Radiology ; Risk analysis ; Risk Factors ; Surgical implants ; Thromboembolism ; Thrombolysis ; Thrombolytic Therapy - methods ; Thrombophilia ; Thrombosis ; Treatment Outcome ; Ultrasound ; Vena Cava, Inferior - abnormalities ; Vena Cava, Inferior - diagnostic imaging ; Venous Interventions ; Venous Thrombosis - complications ; Venous Thrombosis - diagnostic imaging ; Venous Thrombosis - drug therapy ; Young Adult</subject><ispartof>Cardiovascular and interventional radiology, 2020, Vol.43 (1), p.37-45</ispartof><rights>Springer Science+Business Media, LLC, part of Springer Nature and the Cardiovascular and Interventional Radiological Society of Europe (CIRSE) 2019</rights><rights>CardioVascular and Interventional Radiology is a copyright of Springer, (2019). All Rights Reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c375t-a0af242624f690cdb07a171fec9a6a997466b170872a3f1deba84edfe855ff43</citedby><cites>FETCH-LOGICAL-c375t-a0af242624f690cdb07a171fec9a6a997466b170872a3f1deba84edfe855ff43</cites><orcidid>0000-0002-0192-2395</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00270-019-02353-z$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00270-019-02353-z$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/31650242$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mabud, Tarub S.</creatorcontrib><creatorcontrib>Sailer, Anna M.</creatorcontrib><creatorcontrib>Swee, Joshua K. Y.</creatorcontrib><creatorcontrib>Tamboli, Mallika</creatorcontrib><creatorcontrib>Arendt, Victoria A.</creatorcontrib><creatorcontrib>Jeon, Gyeong-Sik</creatorcontrib><creatorcontrib>An, Xiao</creatorcontrib><creatorcontrib>Cohn, David M.</creatorcontrib><creatorcontrib>Kuo, William T.</creatorcontrib><creatorcontrib>Hofmann, Lawrence V.</creatorcontrib><title>Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes</title><title>Cardiovascular and interventional radiology</title><addtitle>Cardiovasc Intervent Radiol</addtitle><addtitle>Cardiovasc Intervent Radiol</addtitle><description>Purpose To characterise (1) the risk factors associated with inferior vena cava (IVC) atresia, (2) the radiographic and clinical presentations of deep vein thrombosis (DVT) in patients with IVC atresia, and (3) the treatment and outcome of DVT in patients with IVC atresia. Methods The electronic medical record was systematically reviewed for thrombotic risk factors in patients who presented with lower-extremity DVT ( n  = 409) at a single centre between 1996 and 2017. Patients with IVC atresia were identified based on imaging and chart review. Differences in demographics and thrombotic risk factors between patients with and without IVC atresia were statistically assessed. Extent and chronicity of DVT on imaging, clinical presentation, treatment, and outcomes were evaluated for all patients with IVC atresia. Results 4.2% of DVT patients (17/409) were found to have IVC atresia; mean age at diagnosis was 25.5 ± 9.4 years. The rate of heritable thrombophilia was significantly higher in patients with IVC atresia compared to patients without IVC atresia (52.9% vs. 17.9%, p  &lt; 0.0001). There were bilateral DVT in 70.6% of IVC atresia patients; DVT was chronic in 41.2% and acute on chronic in 58.8%. Pre-intervention Villalta scores were 13.9 ± 9.8 in the left limb and 8.5 ± 7.0 in the right limb. DVT in IVC atresia patients was typically treated with catheter-directed thrombolysis followed by stent placement, achieving complete or partial symptom resolution in 78.6% of cases. Conclusion Thrombotic risk factors such as heritable thrombophilia are associated with IVC atresia. IVC atresia patients can experience high burdens of lower-extremity thrombotic disease at a young age which benefit from endovascular treatment. 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Y.</creator><creator>Tamboli, Mallika</creator><creator>Arendt, Victoria A.</creator><creator>Jeon, Gyeong-Sik</creator><creator>An, Xiao</creator><creator>Cohn, David M.</creator><creator>Kuo, William T.</creator><creator>Hofmann, Lawrence V.</creator><general>Springer US</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7RV</scope><scope>7TO</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-0192-2395</orcidid></search><sort><creationdate>2020</creationdate><title>Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes</title><author>Mabud, Tarub S. ; Sailer, Anna M. ; Swee, Joshua K. 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Y.</au><au>Tamboli, Mallika</au><au>Arendt, Victoria A.</au><au>Jeon, Gyeong-Sik</au><au>An, Xiao</au><au>Cohn, David M.</au><au>Kuo, William T.</au><au>Hofmann, Lawrence V.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes</atitle><jtitle>Cardiovascular and interventional radiology</jtitle><stitle>Cardiovasc Intervent Radiol</stitle><addtitle>Cardiovasc Intervent Radiol</addtitle><date>2020</date><risdate>2020</risdate><volume>43</volume><issue>1</issue><spage>37</spage><epage>45</epage><pages>37-45</pages><issn>0174-1551</issn><eissn>1432-086X</eissn><abstract>Purpose To characterise (1) the risk factors associated with inferior vena cava (IVC) atresia, (2) the radiographic and clinical presentations of deep vein thrombosis (DVT) in patients with IVC atresia, and (3) the treatment and outcome of DVT in patients with IVC atresia. Methods The electronic medical record was systematically reviewed for thrombotic risk factors in patients who presented with lower-extremity DVT ( n  = 409) at a single centre between 1996 and 2017. Patients with IVC atresia were identified based on imaging and chart review. Differences in demographics and thrombotic risk factors between patients with and without IVC atresia were statistically assessed. Extent and chronicity of DVT on imaging, clinical presentation, treatment, and outcomes were evaluated for all patients with IVC atresia. Results 4.2% of DVT patients (17/409) were found to have IVC atresia; mean age at diagnosis was 25.5 ± 9.4 years. The rate of heritable thrombophilia was significantly higher in patients with IVC atresia compared to patients without IVC atresia (52.9% vs. 17.9%, p  &lt; 0.0001). There were bilateral DVT in 70.6% of IVC atresia patients; DVT was chronic in 41.2% and acute on chronic in 58.8%. Pre-intervention Villalta scores were 13.9 ± 9.8 in the left limb and 8.5 ± 7.0 in the right limb. DVT in IVC atresia patients was typically treated with catheter-directed thrombolysis followed by stent placement, achieving complete or partial symptom resolution in 78.6% of cases. Conclusion Thrombotic risk factors such as heritable thrombophilia are associated with IVC atresia. IVC atresia patients can experience high burdens of lower-extremity thrombotic disease at a young age which benefit from endovascular treatment. Level of Evidence Level 4.</abstract><cop>New York</cop><pub>Springer US</pub><pmid>31650242</pmid><doi>10.1007/s00270-019-02353-z</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-0192-2395</orcidid></addata></record>
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subjects Adolescent
Adult
Cardiology
Cardiovascular system
Catheters
Clinical Investigation
Computed Tomography Angiography
Demographics
Demography
Electronic health records
Electronic medical records
Female
Humans
Imaging
Implants
Lower Extremity - blood supply
Magnetic Resonance Angiography
Male
Medical imaging
Medical instruments
Medicine
Medicine & Public Health
Nuclear Medicine
Patients
Radiology
Risk analysis
Risk Factors
Surgical implants
Thromboembolism
Thrombolysis
Thrombolytic Therapy - methods
Thrombophilia
Thrombosis
Treatment Outcome
Ultrasound
Vena Cava, Inferior - abnormalities
Vena Cava, Inferior - diagnostic imaging
Venous Interventions
Venous Thrombosis - complications
Venous Thrombosis - diagnostic imaging
Venous Thrombosis - drug therapy
Young Adult
title Inferior Vena Cava Atresia: Characterisation of Risk Factors, Treatment, and Outcomes
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