Light chain and transthyretin cardiac amyloidosis: Clinical characteristics, natural history and prognostic factors
Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases. We conducted a single-centre, retrospective review of all patients diagnosed with CA between...
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| Veröffentlicht in: | Medicina clinica 2021-04, Vol.156 (8), p.369 |
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| creator | Barge-Caballero, Gonzalo Vázquez-García, Raquel Barge-Caballero, Eduardo Couto-Mallón, David Paniagua-Martín, María J Barriales-Villa, Roberto Piñón-Esteban, Pablo Bouzas-Mosquera, Alberto Pombo-Otero, Jorge Debén-Ariznavarreta, Guillermo Vázquez-Rodríguez, José M Crespo-Leiro, María G |
| description | Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases.
We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied.
We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index |
| doi_str_mv | 10.1016/j.medcli.2020.04.031 |
| format | Article |
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We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied.
We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index < 1.5 mV (67.7%), with no differences between the two subtypes of CA. One-year, 3-year, and 5-year survival was 43.3%, 40.4% and 35.4%, respectively, in AC-AL patients, and 85.1%, 57.3% and 31.4% in AC-ATTR patients (p = 0.004). AL-CA subtype (HR 3.41; 95% CI 1.45-8.06; p = 0.005), previous admission for heart failure (HR 4.25; 95% CI 1.63-11.09; p = 0.003) and a NYHA class III-IV (HR 2.76; 95% CI; 1.09-7.03; p = 0.033) were independent predictors of mortality, while beta-blocker therapy was associated with longer survival (HR 0.23; 95% CI 0.09-0.59; p = 0.002).
Differences exist between the clinical presentation of AL-CA and ATTR-CA patients. Both diseases, particularly AL-CA, are associated with poor life prognosis.</description><identifier>EISSN: 1578-8989</identifier><identifier>DOI: 10.1016/j.medcli.2020.04.031</identifier><identifier>PMID: 32591181</identifier><language>eng</language><publisher>Spain</publisher><subject>Aged ; Amyloid Neuropathies, Familial - diagnosis ; Amyloid Neuropathies, Familial - epidemiology ; Cardiomyopathies - diagnosis ; Cardiomyopathies - epidemiology ; Female ; Humans ; Immunoglobulin Light-chain Amyloidosis ; Male ; Prealbumin - genetics ; Prognosis ; Retrospective Studies</subject><ispartof>Medicina clinica, 2021-04, Vol.156 (8), p.369</ispartof><rights>Copyright © 2020 Elsevier España, S.L.U. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>776</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/32591181$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Barge-Caballero, Gonzalo</creatorcontrib><creatorcontrib>Vázquez-García, Raquel</creatorcontrib><creatorcontrib>Barge-Caballero, Eduardo</creatorcontrib><creatorcontrib>Couto-Mallón, David</creatorcontrib><creatorcontrib>Paniagua-Martín, María J</creatorcontrib><creatorcontrib>Barriales-Villa, Roberto</creatorcontrib><creatorcontrib>Piñón-Esteban, Pablo</creatorcontrib><creatorcontrib>Bouzas-Mosquera, Alberto</creatorcontrib><creatorcontrib>Pombo-Otero, Jorge</creatorcontrib><creatorcontrib>Debén-Ariznavarreta, Guillermo</creatorcontrib><creatorcontrib>Vázquez-Rodríguez, José M</creatorcontrib><creatorcontrib>Crespo-Leiro, María G</creatorcontrib><title>Light chain and transthyretin cardiac amyloidosis: Clinical characteristics, natural history and prognostic factors</title><title>Medicina clinica</title><addtitle>Med Clin (Barc)</addtitle><description>Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases.
We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied.
We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index < 1.5 mV (67.7%), with no differences between the two subtypes of CA. One-year, 3-year, and 5-year survival was 43.3%, 40.4% and 35.4%, respectively, in AC-AL patients, and 85.1%, 57.3% and 31.4% in AC-ATTR patients (p = 0.004). AL-CA subtype (HR 3.41; 95% CI 1.45-8.06; p = 0.005), previous admission for heart failure (HR 4.25; 95% CI 1.63-11.09; p = 0.003) and a NYHA class III-IV (HR 2.76; 95% CI; 1.09-7.03; p = 0.033) were independent predictors of mortality, while beta-blocker therapy was associated with longer survival (HR 0.23; 95% CI 0.09-0.59; p = 0.002).
Differences exist between the clinical presentation of AL-CA and ATTR-CA patients. Both diseases, particularly AL-CA, are associated with poor life prognosis.</description><subject>Aged</subject><subject>Amyloid Neuropathies, Familial - diagnosis</subject><subject>Amyloid Neuropathies, Familial - epidemiology</subject><subject>Cardiomyopathies - diagnosis</subject><subject>Cardiomyopathies - epidemiology</subject><subject>Female</subject><subject>Humans</subject><subject>Immunoglobulin Light-chain Amyloidosis</subject><subject>Male</subject><subject>Prealbumin - genetics</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><issn>1578-8989</issn><fulltext>false</fulltext><rsrctype>article</rsrctype><creationdate>2021</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1UFtrwyAYlcFYS9d_MIY_YMn8NBrd2yi7QWAv23NRo40jTYLah_z7pbs8HTg3OAehGyAlEBD3X-XRtbYPJSWUlKQqCYMLtAZey0IqqVZom1IwBJSgNVfyCq0Y5QpAwhqlJhy6jG2nw4D10OIc9ZByN0eXF8bq2AZtsT7O_RjaMYX0gHd9GILV_TkVtc0uhpSDTXd40PkUF6FbiDHOP4VTHA_DeDZgv5jHmK7Rpdd9cts_3KDP56eP3WvRvL-87R6bYqIAudAeOFTCGuaBGEGYYcZWTNReUN56V0ujrDW8rlStOKsJN9SBEMK2XjAn6Qbd_vZOJ7NctJ9iOOo47__X02_NM2AC</recordid><startdate>20210423</startdate><enddate>20210423</enddate><creator>Barge-Caballero, Gonzalo</creator><creator>Vázquez-García, Raquel</creator><creator>Barge-Caballero, Eduardo</creator><creator>Couto-Mallón, David</creator><creator>Paniagua-Martín, María J</creator><creator>Barriales-Villa, Roberto</creator><creator>Piñón-Esteban, Pablo</creator><creator>Bouzas-Mosquera, Alberto</creator><creator>Pombo-Otero, Jorge</creator><creator>Debén-Ariznavarreta, Guillermo</creator><creator>Vázquez-Rodríguez, José M</creator><creator>Crespo-Leiro, María G</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope></search><sort><creationdate>20210423</creationdate><title>Light chain and transthyretin cardiac amyloidosis: Clinical characteristics, natural history and prognostic factors</title><author>Barge-Caballero, Gonzalo ; Vázquez-García, Raquel ; Barge-Caballero, Eduardo ; Couto-Mallón, David ; Paniagua-Martín, María J ; Barriales-Villa, Roberto ; Piñón-Esteban, Pablo ; Bouzas-Mosquera, Alberto ; Pombo-Otero, Jorge ; Debén-Ariznavarreta, Guillermo ; Vázquez-Rodríguez, José M ; Crespo-Leiro, María G</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p211t-af15146cb3f10b603b3bc4367f625dfe78b9ccb57497953705b2e1666cdf63e82</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2021</creationdate><topic>Aged</topic><topic>Amyloid Neuropathies, Familial - diagnosis</topic><topic>Amyloid Neuropathies, Familial - epidemiology</topic><topic>Cardiomyopathies - diagnosis</topic><topic>Cardiomyopathies - epidemiology</topic><topic>Female</topic><topic>Humans</topic><topic>Immunoglobulin Light-chain Amyloidosis</topic><topic>Male</topic><topic>Prealbumin - genetics</topic><topic>Prognosis</topic><topic>Retrospective Studies</topic><toplevel>peer_reviewed</toplevel><creatorcontrib>Barge-Caballero, Gonzalo</creatorcontrib><creatorcontrib>Vázquez-García, Raquel</creatorcontrib><creatorcontrib>Barge-Caballero, Eduardo</creatorcontrib><creatorcontrib>Couto-Mallón, David</creatorcontrib><creatorcontrib>Paniagua-Martín, María J</creatorcontrib><creatorcontrib>Barriales-Villa, Roberto</creatorcontrib><creatorcontrib>Piñón-Esteban, Pablo</creatorcontrib><creatorcontrib>Bouzas-Mosquera, Alberto</creatorcontrib><creatorcontrib>Pombo-Otero, Jorge</creatorcontrib><creatorcontrib>Debén-Ariznavarreta, Guillermo</creatorcontrib><creatorcontrib>Vázquez-Rodríguez, José M</creatorcontrib><creatorcontrib>Crespo-Leiro, María G</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><jtitle>Medicina clinica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>no_fulltext</fulltext></delivery><addata><au>Barge-Caballero, Gonzalo</au><au>Vázquez-García, Raquel</au><au>Barge-Caballero, Eduardo</au><au>Couto-Mallón, David</au><au>Paniagua-Martín, María J</au><au>Barriales-Villa, Roberto</au><au>Piñón-Esteban, Pablo</au><au>Bouzas-Mosquera, Alberto</au><au>Pombo-Otero, Jorge</au><au>Debén-Ariznavarreta, Guillermo</au><au>Vázquez-Rodríguez, José M</au><au>Crespo-Leiro, María G</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Light chain and transthyretin cardiac amyloidosis: Clinical characteristics, natural history and prognostic factors</atitle><jtitle>Medicina clinica</jtitle><addtitle>Med Clin (Barc)</addtitle><date>2021-04-23</date><risdate>2021</risdate><volume>156</volume><issue>8</issue><spage>369</spage><pages>369-</pages><eissn>1578-8989</eissn><abstract>Light-chain amyloidosis (AL-CA) and transthyretin amyloidosis (ATTR-CA) are the most common types of cardiac amyloidosis (CA). We sought to study the clinical characteristics and prognosis of both diseases.
We conducted a single-centre, retrospective review of all patients diagnosed with CA between 1998 and 2018. Clinical characteristics, complementary tests, survival and other adverse clinical events were studied.
We identified 105 patients with CA, 65 ATTR-CA and 40 AL-CA. Mean age was 74.4 years; 24.8% were women. In both groups, heart failure was the most frequent clinical presentation (55.2%). The most prevalent electrocardiographic findings were the pseudoinfarct pattern (68.5%) and a Sokolow-Lyon index < 1.5 mV (67.7%), with no differences between the two subtypes of CA. One-year, 3-year, and 5-year survival was 43.3%, 40.4% and 35.4%, respectively, in AC-AL patients, and 85.1%, 57.3% and 31.4% in AC-ATTR patients (p = 0.004). AL-CA subtype (HR 3.41; 95% CI 1.45-8.06; p = 0.005), previous admission for heart failure (HR 4.25; 95% CI 1.63-11.09; p = 0.003) and a NYHA class III-IV (HR 2.76; 95% CI; 1.09-7.03; p = 0.033) were independent predictors of mortality, while beta-blocker therapy was associated with longer survival (HR 0.23; 95% CI 0.09-0.59; p = 0.002).
Differences exist between the clinical presentation of AL-CA and ATTR-CA patients. Both diseases, particularly AL-CA, are associated with poor life prognosis.</abstract><cop>Spain</cop><pmid>32591181</pmid><doi>10.1016/j.medcli.2020.04.031</doi></addata></record> |
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| subjects | Aged Amyloid Neuropathies, Familial - diagnosis Amyloid Neuropathies, Familial - epidemiology Cardiomyopathies - diagnosis Cardiomyopathies - epidemiology Female Humans Immunoglobulin Light-chain Amyloidosis Male Prealbumin - genetics Prognosis Retrospective Studies |
| title | Light chain and transthyretin cardiac amyloidosis: Clinical characteristics, natural history and prognostic factors |
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