Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect
We sought to determine the predisposing factors of pulmonary vascular disease (PVD) in complete atrioventricular septal defect. Down’s syndrome is considered a risk factor for PVD, but the progression of PVD differs in each case. Morphometric analysis in autopsied hearts showed that Rastelli type A...
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| Veröffentlicht in: | The American journal of cardiology 2000-08, Vol.86 (4), p.434-437 |
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| creator | Suzuki, Kiyoshi Yamaki, Shigeo Mimori, Shigekazu Murakami, Yasuo Mori, Katsuhiko Takahashi, Yukihiro Kikuchi, Toshio |
| description | We sought to determine the predisposing factors of pulmonary vascular disease (PVD) in complete atrioventricular septal defect. Down’s syndrome is considered a risk factor for PVD, but the progression of PVD differs in each case. Morphometric analysis in autopsied hearts showed that Rastelli type A morphology had a narrower left ventricular outlet and a wider right ventricular outlet than did type C. In 81 consecutive patients with Down’s syndrome who underwent cardiac catheterization, we estimated the following variables: Rastelli subtypes, pulmonary vascular resistance, pulmonary-to-systemic flow ratio, patients’ age, and operative outcome. The hemodynamic variables in those |
| doi_str_mv | 10.1016/S0002-9149(00)00960-7 |
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Down’s syndrome is considered a risk factor for PVD, but the progression of PVD differs in each case. Morphometric analysis in autopsied hearts showed that Rastelli type A morphology had a narrower left ventricular outlet and a wider right ventricular outlet than did type C. In 81 consecutive patients with Down’s syndrome who underwent cardiac catheterization, we estimated the following variables: Rastelli subtypes, pulmonary vascular resistance, pulmonary-to-systemic flow ratio, patients’ age, and operative outcome. The hemodynamic variables in those <1 year old did not differ between the groups with type A and type C. However, all 5 patients with fatal pulmonary hypertension in early infancy had type A morphology. The lung histology revealed that 3 of these patients already had irreversible PVD. At ≥1 year old, those with type A showed a significantly higher pulmonary vascular resistance (p <0.001) and a lower pulmonary to systemic flow ratio (p <0.05) than did those <1 year old. In contrast, neither of these variables in the type C group differed between those ≥1 and <1 year old. Moreover, those with type A had a greater risk of being contraindicated for surgical repair (p <0.05). We suspect, therefore, that type A morphology is an independent risk factor for PVD in those with Down’s syndrome associated with this anomaly. This hemodynamic influence could become obvious once patients are ≥1 year old. It may also sometimes result in irreversible PVD even in early infancy.]]></description><identifier>ISSN: 0002-9149</identifier><identifier>EISSN: 1879-1913</identifier><identifier>DOI: 10.1016/S0002-9149(00)00960-7</identifier><identifier>PMID: 10946038</identifier><identifier>CODEN: AJCDAG</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Adolescent ; Age ; Autopsies ; Biological and medical sciences ; Biopsy ; Blood vessels ; Cardiac Catheterization ; Cardiology ; Cardiology. Vascular system ; Catheterization ; Child ; Child, Preschool ; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava ; Defects ; Down syndrome ; Down Syndrome - complications ; Downs syndrome ; Female ; Flow resistance ; Heart ; Heart diseases ; Heart Septal Defects, Ventricular - complications ; Heart Septal Defects, Ventricular - pathology ; Heart Septal Defects, Ventricular - surgery ; Hemodynamics ; Histology ; Humans ; Hypertension ; Hypertension, Pulmonary - classification ; Hypertension, Pulmonary - etiology ; Hypertension, Pulmonary - mortality ; Infant ; Intubation ; Lung diseases ; Male ; Medical sciences ; Morphology ; Patients ; Pulmonary arteries ; Pulmonary Circulation ; Risk Factors ; Statistical analysis ; Surgery ; Vascular diseases ; Vascular Resistance ; Veins & arteries ; Ventricle</subject><ispartof>The American journal of cardiology, 2000-08, Vol.86 (4), p.434-437</ispartof><rights>2000 Excerpta Medica Inc.</rights><rights>2000 INIST-CNRS</rights><rights>Copyright Elsevier Sequoia S.A. Aug 15, 2000</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c497t-7d69836b56f6998f5fb7effdb282a19c7c996de940cda7fdfaa5f1a98036b6973</citedby><cites>FETCH-LOGICAL-c497t-7d69836b56f6998f5fb7effdb282a19c7c996de940cda7fdfaa5f1a98036b6973</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0002-9149(00)00960-7$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3549,27923,27924,45994</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1470737$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/10946038$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Suzuki, Kiyoshi</creatorcontrib><creatorcontrib>Yamaki, Shigeo</creatorcontrib><creatorcontrib>Mimori, Shigekazu</creatorcontrib><creatorcontrib>Murakami, Yasuo</creatorcontrib><creatorcontrib>Mori, Katsuhiko</creatorcontrib><creatorcontrib>Takahashi, Yukihiro</creatorcontrib><creatorcontrib>Kikuchi, Toshio</creatorcontrib><title>Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect</title><title>The American journal of cardiology</title><addtitle>Am J Cardiol</addtitle><description><![CDATA[We sought to determine the predisposing factors of pulmonary vascular disease (PVD) in complete atrioventricular septal defect. Down’s syndrome is considered a risk factor for PVD, but the progression of PVD differs in each case. Morphometric analysis in autopsied hearts showed that Rastelli type A morphology had a narrower left ventricular outlet and a wider right ventricular outlet than did type C. In 81 consecutive patients with Down’s syndrome who underwent cardiac catheterization, we estimated the following variables: Rastelli subtypes, pulmonary vascular resistance, pulmonary-to-systemic flow ratio, patients’ age, and operative outcome. The hemodynamic variables in those <1 year old did not differ between the groups with type A and type C. However, all 5 patients with fatal pulmonary hypertension in early infancy had type A morphology. The lung histology revealed that 3 of these patients already had irreversible PVD. At ≥1 year old, those with type A showed a significantly higher pulmonary vascular resistance (p <0.001) and a lower pulmonary to systemic flow ratio (p <0.05) than did those <1 year old. In contrast, neither of these variables in the type C group differed between those ≥1 and <1 year old. Moreover, those with type A had a greater risk of being contraindicated for surgical repair (p <0.05). We suspect, therefore, that type A morphology is an independent risk factor for PVD in those with Down’s syndrome associated with this anomaly. This hemodynamic influence could become obvious once patients are ≥1 year old. It may also sometimes result in irreversible PVD even in early infancy.]]></description><subject>Adolescent</subject><subject>Age</subject><subject>Autopsies</subject><subject>Biological and medical sciences</subject><subject>Biopsy</subject><subject>Blood vessels</subject><subject>Cardiac Catheterization</subject><subject>Cardiology</subject><subject>Cardiology. Vascular system</subject><subject>Catheterization</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava</subject><subject>Defects</subject><subject>Down syndrome</subject><subject>Down Syndrome - complications</subject><subject>Downs syndrome</subject><subject>Female</subject><subject>Flow resistance</subject><subject>Heart</subject><subject>Heart diseases</subject><subject>Heart Septal Defects, Ventricular - complications</subject><subject>Heart Septal Defects, Ventricular - pathology</subject><subject>Heart Septal Defects, Ventricular - surgery</subject><subject>Hemodynamics</subject><subject>Histology</subject><subject>Humans</subject><subject>Hypertension</subject><subject>Hypertension, Pulmonary - classification</subject><subject>Hypertension, Pulmonary - etiology</subject><subject>Hypertension, Pulmonary - mortality</subject><subject>Infant</subject><subject>Intubation</subject><subject>Lung diseases</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Morphology</subject><subject>Patients</subject><subject>Pulmonary arteries</subject><subject>Pulmonary Circulation</subject><subject>Risk Factors</subject><subject>Statistical analysis</subject><subject>Surgery</subject><subject>Vascular diseases</subject><subject>Vascular Resistance</subject><subject>Veins & arteries</subject><subject>Ventricle</subject><issn>0002-9149</issn><issn>1879-1913</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNqFkc2KFDEUhYMoTjv6CEpQEV2U3nSlkspKZJwfYUBB3QkhndxghqpKT1LVw-x8DV_PJzE11agI4upy4TuHwzmEPGTwkgETrz4CwLpSjKvnAC8AlIBK3iIr1kpVMcXq22T1Czkg93K-KC9jjbhLDhgoLqBuV-TLh6nr42DSNd2ZbKfOJOpCRpORhoG-jVfDj2_fM83Xg0uxR3oVxq_Uxn7b4YjUjCnEHQ7lLNqM29F01KFHO94nd7zpMj7Y30Py-eT409FZdf7-9N3Rm_PKciXHSjqh2lpsGuGFUq1v_Eai926zbteGKSutUsKh4mCdkd55YxrPjGqhiISS9SF5tvhuU7ycMI-6D9li15kB45S1ZLKppZzBx3-BF3FKQ8mm1zXUjWw4K9CTf0LARcN5q1ShmoWyKeac0OttCn3pUTPQ80L6ZiE9168B9M1Ceo7waO8-bXp0f6iWSQrwdA-UPUznkxlsyL85LkHWs8_rBcNS7C5g0tkGHCy6kEr12sXwnyQ_AcYarj8</recordid><startdate>20000815</startdate><enddate>20000815</enddate><creator>Suzuki, Kiyoshi</creator><creator>Yamaki, Shigeo</creator><creator>Mimori, Shigekazu</creator><creator>Murakami, Yasuo</creator><creator>Mori, Katsuhiko</creator><creator>Takahashi, Yukihiro</creator><creator>Kikuchi, Toshio</creator><general>Elsevier Inc</general><general>Elsevier</general><general>Elsevier Limited</general><scope>IQODW</scope><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>7TS</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>8G5</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>M2O</scope><scope>M7Z</scope><scope>MBDVC</scope><scope>NAPCQ</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>Q9U</scope><scope>7X8</scope></search><sort><creationdate>20000815</creationdate><title>Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect</title><author>Suzuki, Kiyoshi ; Yamaki, Shigeo ; Mimori, Shigekazu ; Murakami, Yasuo ; Mori, Katsuhiko ; Takahashi, Yukihiro ; Kikuchi, Toshio</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c497t-7d69836b56f6998f5fb7effdb282a19c7c996de940cda7fdfaa5f1a98036b6973</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2000</creationdate><topic>Adolescent</topic><topic>Age</topic><topic>Autopsies</topic><topic>Biological and medical sciences</topic><topic>Biopsy</topic><topic>Blood vessels</topic><topic>Cardiac Catheterization</topic><topic>Cardiology</topic><topic>Cardiology. Vascular system</topic><topic>Catheterization</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava</topic><topic>Defects</topic><topic>Down syndrome</topic><topic>Down Syndrome - complications</topic><topic>Downs syndrome</topic><topic>Female</topic><topic>Flow resistance</topic><topic>Heart</topic><topic>Heart diseases</topic><topic>Heart Septal Defects, Ventricular - complications</topic><topic>Heart Septal Defects, Ventricular - pathology</topic><topic>Heart Septal Defects, Ventricular - surgery</topic><topic>Hemodynamics</topic><topic>Histology</topic><topic>Humans</topic><topic>Hypertension</topic><topic>Hypertension, Pulmonary - classification</topic><topic>Hypertension, Pulmonary - etiology</topic><topic>Hypertension, Pulmonary - mortality</topic><topic>Infant</topic><topic>Intubation</topic><topic>Lung diseases</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Morphology</topic><topic>Patients</topic><topic>Pulmonary arteries</topic><topic>Pulmonary Circulation</topic><topic>Risk Factors</topic><topic>Statistical analysis</topic><topic>Surgery</topic><topic>Vascular diseases</topic><topic>Vascular Resistance</topic><topic>Veins & arteries</topic><topic>Ventricle</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Suzuki, Kiyoshi</creatorcontrib><creatorcontrib>Yamaki, Shigeo</creatorcontrib><creatorcontrib>Mimori, Shigekazu</creatorcontrib><creatorcontrib>Murakami, Yasuo</creatorcontrib><creatorcontrib>Mori, Katsuhiko</creatorcontrib><creatorcontrib>Takahashi, Yukihiro</creatorcontrib><creatorcontrib>Kikuchi, Toshio</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Nursing & Allied Health Database</collection><collection>Physical Education Index</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Technology Research Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Research Library (Alumni Edition)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>Research Library Prep</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Research Library</collection><collection>Biochemistry Abstracts 1</collection><collection>Research Library (Corporate)</collection><collection>Nursing & Allied Health Premium</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>ProQuest Central Basic</collection><collection>MEDLINE - Academic</collection><jtitle>The American journal of cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Suzuki, Kiyoshi</au><au>Yamaki, Shigeo</au><au>Mimori, Shigekazu</au><au>Murakami, Yasuo</au><au>Mori, Katsuhiko</au><au>Takahashi, Yukihiro</au><au>Kikuchi, Toshio</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect</atitle><jtitle>The American journal of cardiology</jtitle><addtitle>Am J Cardiol</addtitle><date>2000-08-15</date><risdate>2000</risdate><volume>86</volume><issue>4</issue><spage>434</spage><epage>437</epage><pages>434-437</pages><issn>0002-9149</issn><eissn>1879-1913</eissn><coden>AJCDAG</coden><abstract><![CDATA[We sought to determine the predisposing factors of pulmonary vascular disease (PVD) in complete atrioventricular septal defect. Down’s syndrome is considered a risk factor for PVD, but the progression of PVD differs in each case. Morphometric analysis in autopsied hearts showed that Rastelli type A morphology had a narrower left ventricular outlet and a wider right ventricular outlet than did type C. In 81 consecutive patients with Down’s syndrome who underwent cardiac catheterization, we estimated the following variables: Rastelli subtypes, pulmonary vascular resistance, pulmonary-to-systemic flow ratio, patients’ age, and operative outcome. The hemodynamic variables in those <1 year old did not differ between the groups with type A and type C. However, all 5 patients with fatal pulmonary hypertension in early infancy had type A morphology. The lung histology revealed that 3 of these patients already had irreversible PVD. At ≥1 year old, those with type A showed a significantly higher pulmonary vascular resistance (p <0.001) and a lower pulmonary to systemic flow ratio (p <0.05) than did those <1 year old. In contrast, neither of these variables in the type C group differed between those ≥1 and <1 year old. Moreover, those with type A had a greater risk of being contraindicated for surgical repair (p <0.05). We suspect, therefore, that type A morphology is an independent risk factor for PVD in those with Down’s syndrome associated with this anomaly. This hemodynamic influence could become obvious once patients are ≥1 year old. It may also sometimes result in irreversible PVD even in early infancy.]]></abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>10946038</pmid><doi>10.1016/S0002-9149(00)00960-7</doi><tpages>4</tpages></addata></record> |
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| subjects | Adolescent Age Autopsies Biological and medical sciences Biopsy Blood vessels Cardiac Catheterization Cardiology Cardiology. Vascular system Catheterization Child Child, Preschool Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava Defects Down syndrome Down Syndrome - complications Downs syndrome Female Flow resistance Heart Heart diseases Heart Septal Defects, Ventricular - complications Heart Septal Defects, Ventricular - pathology Heart Septal Defects, Ventricular - surgery Hemodynamics Histology Humans Hypertension Hypertension, Pulmonary - classification Hypertension, Pulmonary - etiology Hypertension, Pulmonary - mortality Infant Intubation Lung diseases Male Medical sciences Morphology Patients Pulmonary arteries Pulmonary Circulation Risk Factors Statistical analysis Surgery Vascular diseases Vascular Resistance Veins & arteries Ventricle |
| title | Pulmonary vascular disease in Down’s syndrome with complete atrioventricular septal defect |
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