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Left atrial structural and functional remodelling in Fabry disease and cardiac amyloidosis: A comparative analysis
Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analy...
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| Published in: | International journal of cardiology 2024-05, Vol.402, p.131891-131891, Article 131891 |
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| container_title | International journal of cardiology |
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| creator | Meucci, Maria Chiara Lillo, Rosa Mango, Federica Marsilia, Mario Iannaccone, Giulia Tusa, Filippo Luigetti, Marco Biagini, Elena Massetti, Massimo Lanza, Gaetano Antonio Lombardo, Antonella Graziani, Francesca |
| description | Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analysis of LA remodelling between the two diseases.
In this prospective study, a total of 114 patients (31 FD and 83 TTR CA) were included; all of them had left ventricular hypertrophy (LVH), defined as left ventricular (LV) wall thickness ≥ 12 mm. Despite similar degree of LVH, patients with TTR CA showed worse LV systolic and diastolic function. LA maximal volume index was not significantly different between the two groups (p = 0.084), while patients with TTR CA showed larger LA minimal volume index (p = 0.001). Moreover, all phases of LA mechanics were more impaired in the TTR CA group vs FD (reservoir: 6.9[4.2–15.5] vs 19.0[15.5–29.5], p |
| doi_str_mv | 10.1016/j.ijcard.2024.131891 |
| format | article |
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In this prospective study, a total of 114 patients (31 FD and 83 TTR CA) were included; all of them had left ventricular hypertrophy (LVH), defined as left ventricular (LV) wall thickness ≥ 12 mm. Despite similar degree of LVH, patients with TTR CA showed worse LV systolic and diastolic function. LA maximal volume index was not significantly different between the two groups (p = 0.084), while patients with TTR CA showed larger LA minimal volume index (p = 0.001). Moreover, all phases of LA mechanics were more impaired in the TTR CA group vs FD (reservoir: 6.9[4.2–15.5] vs 19.0[15.5–29.5], p < 0.001). After excluding patients with atrial fibrillation (AF), these differences remained clearly significant. In multivariable regression analyses, LA reservoir strain showed an independent correlation with TTR CA, controlling for demographic characteristics, AF and LV systolic and diastolic performance (p ≤ 0.001), whereas LV global longitudinal strain did not. Finally, among echocardiographic parameters, LA function demonstrated the highest accuracy in discriminating the two diseases.
TTR CA is characterized by a more advanced LA structural and functional remodelling in comparison to patients with FD and similar degree of LVH. The association between TTR CA and LA dysfunction remains consistent after adjustment for potential confounders.
[Display omitted]
•LA remodelling was compared between patients with FD and TTR CA.•The two study groups had a similar degree of LV hypertrophy.•TTR CA patients showed larger LAVmin, whereas there were no differences in LAVImax.•TTR CA patients were characterized by worse LA functional parameters.•This association was confirmed after adjustment for potential confounders.</description><identifier>ISSN: 0167-5273</identifier><identifier>EISSN: 1874-1754</identifier><identifier>DOI: 10.1016/j.ijcard.2024.131891</identifier><identifier>PMID: 38382852</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Amyloidosis ; Cardiomyopathy ; Fabry disease ; Left atrial remodelling ; Speckle-tracking echocardiography</subject><ispartof>International journal of cardiology, 2024-05, Vol.402, p.131891-131891, Article 131891</ispartof><rights>2024 Elsevier B.V.</rights><rights>Copyright © 2024 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c362t-76cd1a5af59cc6938c61356d90184f1adebf4fb5d4437a6d5d2ee68bf0d494373</citedby><cites>FETCH-LOGICAL-c362t-76cd1a5af59cc6938c61356d90184f1adebf4fb5d4437a6d5d2ee68bf0d494373</cites></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/38382852$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Meucci, Maria Chiara</creatorcontrib><creatorcontrib>Lillo, Rosa</creatorcontrib><creatorcontrib>Mango, Federica</creatorcontrib><creatorcontrib>Marsilia, Mario</creatorcontrib><creatorcontrib>Iannaccone, Giulia</creatorcontrib><creatorcontrib>Tusa, Filippo</creatorcontrib><creatorcontrib>Luigetti, Marco</creatorcontrib><creatorcontrib>Biagini, Elena</creatorcontrib><creatorcontrib>Massetti, Massimo</creatorcontrib><creatorcontrib>Lanza, Gaetano Antonio</creatorcontrib><creatorcontrib>Lombardo, Antonella</creatorcontrib><creatorcontrib>Graziani, Francesca</creatorcontrib><title>Left atrial structural and functional remodelling in Fabry disease and cardiac amyloidosis: A comparative analysis</title><title>International journal of cardiology</title><addtitle>Int J Cardiol</addtitle><description>Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analysis of LA remodelling between the two diseases.
In this prospective study, a total of 114 patients (31 FD and 83 TTR CA) were included; all of them had left ventricular hypertrophy (LVH), defined as left ventricular (LV) wall thickness ≥ 12 mm. Despite similar degree of LVH, patients with TTR CA showed worse LV systolic and diastolic function. LA maximal volume index was not significantly different between the two groups (p = 0.084), while patients with TTR CA showed larger LA minimal volume index (p = 0.001). Moreover, all phases of LA mechanics were more impaired in the TTR CA group vs FD (reservoir: 6.9[4.2–15.5] vs 19.0[15.5–29.5], p < 0.001). After excluding patients with atrial fibrillation (AF), these differences remained clearly significant. In multivariable regression analyses, LA reservoir strain showed an independent correlation with TTR CA, controlling for demographic characteristics, AF and LV systolic and diastolic performance (p ≤ 0.001), whereas LV global longitudinal strain did not. Finally, among echocardiographic parameters, LA function demonstrated the highest accuracy in discriminating the two diseases.
TTR CA is characterized by a more advanced LA structural and functional remodelling in comparison to patients with FD and similar degree of LVH. The association between TTR CA and LA dysfunction remains consistent after adjustment for potential confounders.
[Display omitted]
•LA remodelling was compared between patients with FD and TTR CA.•The two study groups had a similar degree of LV hypertrophy.•TTR CA patients showed larger LAVmin, whereas there were no differences in LAVImax.•TTR CA patients were characterized by worse LA functional parameters.•This association was confirmed after adjustment for potential confounders.</description><subject>Amyloidosis</subject><subject>Cardiomyopathy</subject><subject>Fabry disease</subject><subject>Left atrial remodelling</subject><subject>Speckle-tracking echocardiography</subject><issn>0167-5273</issn><issn>1874-1754</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNp9kE1v1DAQQK0K1G4L_wAhH7lksWMncTggVVVLkVbiAmdrYo-RV0m82E6l_fd1msKRk8ejN1-PkA-c7Tnj7efj3h8NRLuvWS33XHDV8wuy46qTFe8a-YbsCtZVTd2JK3Kd0pExJvteXZIroYSqVVPvSDygyxRy9DDSlONi8hJLCLOlbplN9mEu34hTsDiOfv5N_UwfYIhnan1CSPjCrpt4MBSm8xi8DcmnL_SWmjCdIEL2TysG47nk35G3DsaE71_fG_Lr4f7n3WN1-PHt-93toTKirXPVtcZyaMA1vTFtL5RpuWha2zOupONgcXDSDY2VUnTQ2sbWiK0aHLOyLylxQz5tfU8x_FkwZT35ZMoRMGNYkq57wWQnOF9RuaEmhpQiOn2KfoJ41pzp1bY-6s22Xm3rzXYp-_g6YRkmtP-K_uotwNcNwHLnk8eok_E4G7Q-osnaBv__Cc_uTJRU</recordid><startdate>20240501</startdate><enddate>20240501</enddate><creator>Meucci, Maria Chiara</creator><creator>Lillo, Rosa</creator><creator>Mango, Federica</creator><creator>Marsilia, Mario</creator><creator>Iannaccone, Giulia</creator><creator>Tusa, Filippo</creator><creator>Luigetti, Marco</creator><creator>Biagini, Elena</creator><creator>Massetti, Massimo</creator><creator>Lanza, Gaetano Antonio</creator><creator>Lombardo, Antonella</creator><creator>Graziani, Francesca</creator><general>Elsevier B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20240501</creationdate><title>Left atrial structural and functional remodelling in Fabry disease and cardiac amyloidosis: A comparative analysis</title><author>Meucci, Maria Chiara ; Lillo, Rosa ; Mango, Federica ; Marsilia, Mario ; Iannaccone, Giulia ; Tusa, Filippo ; Luigetti, Marco ; Biagini, Elena ; Massetti, Massimo ; Lanza, Gaetano Antonio ; Lombardo, Antonella ; Graziani, Francesca</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c362t-76cd1a5af59cc6938c61356d90184f1adebf4fb5d4437a6d5d2ee68bf0d494373</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Amyloidosis</topic><topic>Cardiomyopathy</topic><topic>Fabry disease</topic><topic>Left atrial remodelling</topic><topic>Speckle-tracking echocardiography</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Meucci, Maria Chiara</creatorcontrib><creatorcontrib>Lillo, Rosa</creatorcontrib><creatorcontrib>Mango, Federica</creatorcontrib><creatorcontrib>Marsilia, Mario</creatorcontrib><creatorcontrib>Iannaccone, Giulia</creatorcontrib><creatorcontrib>Tusa, Filippo</creatorcontrib><creatorcontrib>Luigetti, Marco</creatorcontrib><creatorcontrib>Biagini, Elena</creatorcontrib><creatorcontrib>Massetti, Massimo</creatorcontrib><creatorcontrib>Lanza, Gaetano Antonio</creatorcontrib><creatorcontrib>Lombardo, Antonella</creatorcontrib><creatorcontrib>Graziani, Francesca</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>International journal of cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Meucci, Maria Chiara</au><au>Lillo, Rosa</au><au>Mango, Federica</au><au>Marsilia, Mario</au><au>Iannaccone, Giulia</au><au>Tusa, Filippo</au><au>Luigetti, Marco</au><au>Biagini, Elena</au><au>Massetti, Massimo</au><au>Lanza, Gaetano Antonio</au><au>Lombardo, Antonella</au><au>Graziani, Francesca</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Left atrial structural and functional remodelling in Fabry disease and cardiac amyloidosis: A comparative analysis</atitle><jtitle>International journal of cardiology</jtitle><addtitle>Int J Cardiol</addtitle><date>2024-05-01</date><risdate>2024</risdate><volume>402</volume><spage>131891</spage><epage>131891</epage><pages>131891-131891</pages><artnum>131891</artnum><issn>0167-5273</issn><eissn>1874-1754</eissn><abstract>Fabry disease (FD) and transthyretin cardiac amyloidosis (TTR CA) are cardiomyopathies with hypertrophic phenotype that share several features, including left atrial (LA) enlargement and dysfunction, but direct comparative data are lacking. Aim of the present study was to perform a comparative analysis of LA remodelling between the two diseases.
In this prospective study, a total of 114 patients (31 FD and 83 TTR CA) were included; all of them had left ventricular hypertrophy (LVH), defined as left ventricular (LV) wall thickness ≥ 12 mm. Despite similar degree of LVH, patients with TTR CA showed worse LV systolic and diastolic function. LA maximal volume index was not significantly different between the two groups (p = 0.084), while patients with TTR CA showed larger LA minimal volume index (p = 0.001). Moreover, all phases of LA mechanics were more impaired in the TTR CA group vs FD (reservoir: 6.9[4.2–15.5] vs 19.0[15.5–29.5], p < 0.001). After excluding patients with atrial fibrillation (AF), these differences remained clearly significant. In multivariable regression analyses, LA reservoir strain showed an independent correlation with TTR CA, controlling for demographic characteristics, AF and LV systolic and diastolic performance (p ≤ 0.001), whereas LV global longitudinal strain did not. Finally, among echocardiographic parameters, LA function demonstrated the highest accuracy in discriminating the two diseases.
TTR CA is characterized by a more advanced LA structural and functional remodelling in comparison to patients with FD and similar degree of LVH. The association between TTR CA and LA dysfunction remains consistent after adjustment for potential confounders.
[Display omitted]
•LA remodelling was compared between patients with FD and TTR CA.•The two study groups had a similar degree of LV hypertrophy.•TTR CA patients showed larger LAVmin, whereas there were no differences in LAVImax.•TTR CA patients were characterized by worse LA functional parameters.•This association was confirmed after adjustment for potential confounders.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>38382852</pmid><doi>10.1016/j.ijcard.2024.131891</doi><tpages>1</tpages></addata></record> |
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| subjects | Amyloidosis Cardiomyopathy Fabry disease Left atrial remodelling Speckle-tracking echocardiography |
| title | Left atrial structural and functional remodelling in Fabry disease and cardiac amyloidosis: A comparative analysis |
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