Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition
Transthyretin V30M (ATTR V30M) amyloidosis is a phenotypically diverse disease with symptoms ranging from predominant neuropathy to exclusive cardiac manifestations. The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish ATTR V30M patients -Type A consis...
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| description | Transthyretin V30M (ATTR V30M) amyloidosis is a phenotypically diverse disease with symptoms ranging from predominant neuropathy to exclusive cardiac manifestations. The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish ATTR V30M patients -Type A consisting of a mixture of truncated and full length ATTR fibrils and type B fibrils consisting of full length fibrils, and to estimate the severity of cardiac dysfunction in relation to fibril composition and sex.
Echocardiographic data were analysed in 107 Swedish ATTR V30M patients with their fibril composition determined as either type A or type B. Measurements of left ventricular (LV) dimensions and evaluation of systolic and diastolic function including speckle tracking derived strain were performed. Patients were grouped according to fibril type and sex. Multivariate linear regression was utilised to determine factors of significant impact on LV thickness.
There was no significant difference in proportions of the two types of fibrils between men and women. In patients with type A fibrils, women had significantly lower median septal (p = 0.007) and posterior wall thicknesses (p = 0.010), lower median LV mass indexed to height (p = 0.008), and higher septal strain (p = 0.037), as compared to males. These differences were not apparent in patients with type B fibrils. Multiple linear regression analysis revealed that fibril type, sex and age all had significant impact on LV septal thickness.
This study demonstrates a clear difference between sexes in the severity of amyloid heart disease in ATTR V30M amyloidosis patients. Even though type A fibrils were associated with more advanced amyloid heart disease compared to type B, women with type A fibrils generally developed less cardiac infiltration than men. The differences may explain the better outcome for liver transplanted late-onset female patients compared to males. |
| doi_str_mv | 10.1371/journal.pone.0143456 |
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Echocardiographic data were analysed in 107 Swedish ATTR V30M patients with their fibril composition determined as either type A or type B. Measurements of left ventricular (LV) dimensions and evaluation of systolic and diastolic function including speckle tracking derived strain were performed. Patients were grouped according to fibril type and sex. Multivariate linear regression was utilised to determine factors of significant impact on LV thickness.
There was no significant difference in proportions of the two types of fibrils between men and women. In patients with type A fibrils, women had significantly lower median septal (p = 0.007) and posterior wall thicknesses (p = 0.010), lower median LV mass indexed to height (p = 0.008), and higher septal strain (p = 0.037), as compared to males. These differences were not apparent in patients with type B fibrils. Multiple linear regression analysis revealed that fibril type, sex and age all had significant impact on LV septal thickness.
This study demonstrates a clear difference between sexes in the severity of amyloid heart disease in ATTR V30M amyloidosis patients. Even though type A fibrils were associated with more advanced amyloid heart disease compared to type B, women with type A fibrils generally developed less cardiac infiltration than men. The differences may explain the better outcome for liver transplanted late-onset female patients compared to males.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0143456</identifier><identifier>PMID: 26600306</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Adult ; Age ; Aged ; Aged, 80 and over ; Amyloid ; Amyloid - metabolism ; Amyloid Neuropathies, Familial - metabolism ; Amyloidosis ; ATTR ; cardiac amyloidosis ; Cardiology ; Cardiomyopathies - metabolism ; Cardiomyopathy ; Cardiovascular disease ; Care and treatment ; Clinical medicine ; Coronary artery disease ; Data processing ; Echocardiography ; Elderly ; Female ; fibril ; Fibrils ; gender ; Health aspects ; Heart ; Heart diseases ; Humans ; Hypertension ; Infiltration ; Liver ; Liver transplantation ; Liver transplants ; Male ; Males ; Medical prognosis ; Middle Aged ; Mutation ; Myocardial diseases ; Neuropathy ; Patients ; Physiology ; Prealbumin - metabolism ; Public health ; Regression analysis ; Risk factors ; Sex ; Sex Factors ; Transthyretin ; Ventricle</subject><ispartof>PloS one, 2015-11, Vol.10 (11), p.e0143456-e0143456</ispartof><rights>COPYRIGHT 2015 Public Library of Science</rights><rights>2015 Arvidsson et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2015 Arvidsson et al 2015 Arvidsson et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c833t-43fb680d1f6912ce5c28d66338b64906a41b5a9d29dee583e18540353e742ab33</citedby><cites>FETCH-LOGICAL-c833t-43fb680d1f6912ce5c28d66338b64906a41b5a9d29dee583e18540353e742ab33</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4658178/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4658178/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,552,727,780,784,864,885,2102,2928,23866,27924,27925,53791,53793,79600,79601</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26600306$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-113831$$DView record from Swedish Publication Index$$Hfree_for_read</backlink><backlink>$$Uhttps://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-272284$$DView record from Swedish Publication Index$$Hfree_for_read</backlink></links><search><contributor>Gasset, Maria</contributor><creatorcontrib>Arvidsson, Sandra</creatorcontrib><creatorcontrib>Pilebro, Björn</creatorcontrib><creatorcontrib>Westermark, Per</creatorcontrib><creatorcontrib>Lindqvist, Per</creatorcontrib><creatorcontrib>Suhr, Ole B</creatorcontrib><title>Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>Transthyretin V30M (ATTR V30M) amyloidosis is a phenotypically diverse disease with symptoms ranging from predominant neuropathy to exclusive cardiac manifestations. The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish ATTR V30M patients -Type A consisting of a mixture of truncated and full length ATTR fibrils and type B fibrils consisting of full length fibrils, and to estimate the severity of cardiac dysfunction in relation to fibril composition and sex.
Echocardiographic data were analysed in 107 Swedish ATTR V30M patients with their fibril composition determined as either type A or type B. Measurements of left ventricular (LV) dimensions and evaluation of systolic and diastolic function including speckle tracking derived strain were performed. Patients were grouped according to fibril type and sex. Multivariate linear regression was utilised to determine factors of significant impact on LV thickness.
There was no significant difference in proportions of the two types of fibrils between men and women. In patients with type A fibrils, women had significantly lower median septal (p = 0.007) and posterior wall thicknesses (p = 0.010), lower median LV mass indexed to height (p = 0.008), and higher septal strain (p = 0.037), as compared to males. These differences were not apparent in patients with type B fibrils. Multiple linear regression analysis revealed that fibril type, sex and age all had significant impact on LV septal thickness.
This study demonstrates a clear difference between sexes in the severity of amyloid heart disease in ATTR V30M amyloidosis patients. Even though type A fibrils were associated with more advanced amyloid heart disease compared to type B, women with type A fibrils generally developed less cardiac infiltration than men. 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metabolism</subject><subject>Public health</subject><subject>Regression analysis</subject><subject>Risk factors</subject><subject>Sex</subject><subject>Sex Factors</subject><subject>Transthyretin</subject><subject>Ventricle</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>D8T</sourceid><sourceid>DOA</sourceid><recordid>eNqNk1Fv0zAUhSMEYmPwDxBYQkIg0WLHseO8IFWFsUpDk9joq-U4N62nJA52Auu_x23TqUF7mPKQ6N7vHNsnvlH0muApoSn5fGt716hq2toGppgkNGH8SXRKMhpPeIzp06Pvk-iF97cYMyo4fx6dxJxjTDE_jdys3lTWFGiuXGFsvbGt6tYbZBp0AQ4K0ym3QTdONT6UHXShsaT4Bxp01huPJmhRt0p3yJboGu6QaopDH52b3JkKzW3dBrYztnkZPStV5eHV8D6Lfp1_u5lfTC6vvi_ms8uJFpR2k4SWORe4ICXPSKyB6VgUnFMqcp5kmKuE5ExlRZwVAExQIIIlmDIKaRKrnNKz6O3et62sl0NcXpKUsmCTEhyIxZ4orLqVrTN1OKy0yshdwbqVVK4zugKJCyHiLFMsK3WCdSbCZnKNQQMlSlMSvD7tvfxfaPt85PbVLGc7t76XcRrHIgn45BF43UtCqNjZfxkO0-c1FBqazqlqJBt3GrOWK_tHJpwJkopg8GEwcPZ3D76TtfEaqko1YPtdLJynmSDbtd79hz4c3kCtVMjHNKUN6-qtqZwlgcIxYSxQ0weo8BRQGx2ubmlCfST4OBIEpoO7bqV67-Xi-ufj2avlmH1_xK5BVd3a26rf3kg_BpM9qJ313kF5HzLBcjt5hzTkdvLkMHlB9ub4B92LDqNG_wGBcSgS</recordid><startdate>20151123</startdate><enddate>20151123</enddate><creator>Arvidsson, Sandra</creator><creator>Pilebro, Björn</creator><creator>Westermark, Per</creator><creator>Lindqvist, Per</creator><creator>Suhr, Ole B</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</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>IOV</scope><scope>ISR</scope><scope>3V.</scope><scope>7QG</scope><scope>7QL</scope><scope>7QO</scope><scope>7RV</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TG</scope><scope>7TM</scope><scope>7U9</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>D1I</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB.</scope><scope>KB0</scope><scope>KL.</scope><scope>L6V</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>M7S</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PATMY</scope><scope>PDBOC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PTHSS</scope><scope>PYCSY</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope><scope>ADHXS</scope><scope>ADTPV</scope><scope>AOWAS</scope><scope>D8T</scope><scope>D93</scope><scope>ZZAVC</scope><scope>ACNBI</scope><scope>DF2</scope><scope>DOA</scope></search><sort><creationdate>20151123</creationdate><title>Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition</title><author>Arvidsson, Sandra ; Pilebro, Björn ; Westermark, Per ; Lindqvist, Per ; Suhr, Ole B</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c833t-43fb680d1f6912ce5c28d66338b64906a41b5a9d29dee583e18540353e742ab33</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Adult</topic><topic>Age</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Amyloid</topic><topic>Amyloid - metabolism</topic><topic>Amyloid Neuropathies, Familial - metabolism</topic><topic>Amyloidosis</topic><topic>ATTR</topic><topic>cardiac amyloidosis</topic><topic>Cardiology</topic><topic>Cardiomyopathies - metabolism</topic><topic>Cardiomyopathy</topic><topic>Cardiovascular disease</topic><topic>Care and treatment</topic><topic>Clinical medicine</topic><topic>Coronary artery disease</topic><topic>Data processing</topic><topic>Echocardiography</topic><topic>Elderly</topic><topic>Female</topic><topic>fibril</topic><topic>Fibrils</topic><topic>gender</topic><topic>Health aspects</topic><topic>Heart</topic><topic>Heart diseases</topic><topic>Humans</topic><topic>Hypertension</topic><topic>Infiltration</topic><topic>Liver</topic><topic>Liver transplantation</topic><topic>Liver transplants</topic><topic>Male</topic><topic>Males</topic><topic>Medical prognosis</topic><topic>Middle Aged</topic><topic>Mutation</topic><topic>Myocardial diseases</topic><topic>Neuropathy</topic><topic>Patients</topic><topic>Physiology</topic><topic>Prealbumin - 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Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>SWEPUB Umeå universitet full text</collection><collection>SwePub</collection><collection>SwePub Articles</collection><collection>SWEPUB Freely available online</collection><collection>SWEPUB Umeå universitet</collection><collection>SwePub Articles full text</collection><collection>SWEPUB Uppsala universitet full text</collection><collection>SWEPUB Uppsala universitet</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Arvidsson, Sandra</au><au>Pilebro, Björn</au><au>Westermark, Per</au><au>Lindqvist, Per</au><au>Suhr, Ole B</au><au>Gasset, Maria</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2015-11-23</date><risdate>2015</risdate><volume>10</volume><issue>11</issue><spage>e0143456</spage><epage>e0143456</epage><pages>e0143456-e0143456</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>Transthyretin V30M (ATTR V30M) amyloidosis is a phenotypically diverse disease with symptoms ranging from predominant neuropathy to exclusive cardiac manifestations. The aims of this study were to determine the dispersion of the two types of fibrils found in Swedish ATTR V30M patients -Type A consisting of a mixture of truncated and full length ATTR fibrils and type B fibrils consisting of full length fibrils, and to estimate the severity of cardiac dysfunction in relation to fibril composition and sex.
Echocardiographic data were analysed in 107 Swedish ATTR V30M patients with their fibril composition determined as either type A or type B. Measurements of left ventricular (LV) dimensions and evaluation of systolic and diastolic function including speckle tracking derived strain were performed. Patients were grouped according to fibril type and sex. Multivariate linear regression was utilised to determine factors of significant impact on LV thickness.
There was no significant difference in proportions of the two types of fibrils between men and women. In patients with type A fibrils, women had significantly lower median septal (p = 0.007) and posterior wall thicknesses (p = 0.010), lower median LV mass indexed to height (p = 0.008), and higher septal strain (p = 0.037), as compared to males. These differences were not apparent in patients with type B fibrils. Multiple linear regression analysis revealed that fibril type, sex and age all had significant impact on LV septal thickness.
This study demonstrates a clear difference between sexes in the severity of amyloid heart disease in ATTR V30M amyloidosis patients. Even though type A fibrils were associated with more advanced amyloid heart disease compared to type B, women with type A fibrils generally developed less cardiac infiltration than men. The differences may explain the better outcome for liver transplanted late-onset female patients compared to males.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>26600306</pmid><doi>10.1371/journal.pone.0143456</doi><oa>free_for_read</oa></addata></record> |
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| recordid | cdi_plos_journals_1735663710 |
| source | MEDLINE; DOAJ Directory of Open Access Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals; SWEPUB Freely available online; Public Library of Science (PLoS); PubMed Central; Free Full-Text Journals in Chemistry |
| subjects | Adult Age Aged Aged, 80 and over Amyloid Amyloid - metabolism Amyloid Neuropathies, Familial - metabolism Amyloidosis ATTR cardiac amyloidosis Cardiology Cardiomyopathies - metabolism Cardiomyopathy Cardiovascular disease Care and treatment Clinical medicine Coronary artery disease Data processing Echocardiography Elderly Female fibril Fibrils gender Health aspects Heart Heart diseases Humans Hypertension Infiltration Liver Liver transplantation Liver transplants Male Males Medical prognosis Middle Aged Mutation Myocardial diseases Neuropathy Patients Physiology Prealbumin - metabolism Public health Regression analysis Risk factors Sex Sex Factors Transthyretin Ventricle |
| title | Amyloid Cardiomyopathy in Hereditary Transthyretin V30M Amyloidosis - Impact of Sex and Amyloid Fibril Composition |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-06T23%3A06%3A01IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Amyloid%20Cardiomyopathy%20in%20Hereditary%20Transthyretin%20V30M%20Amyloidosis%20-%20Impact%20of%20Sex%20and%20Amyloid%20Fibril%20Composition&rft.jtitle=PloS%20one&rft.au=Arvidsson,%20Sandra&rft.date=2015-11-23&rft.volume=10&rft.issue=11&rft.spage=e0143456&rft.epage=e0143456&rft.pages=e0143456-e0143456&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0143456&rft_dat=%3Cgale_plos_%3EA435602155%3C/gale_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1735663710&rft_id=info:pmid/26600306&rft_galeid=A435602155&rft_doaj_id=oai_doaj_org_article_0d88299a59fc40c9828dbc0ece31ac31&rfr_iscdi=true |