Differences in the characteristics and contemporary cardiac outcomes of patients with light-chain versus transthyretin cardiac amyloidosis

To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients' contemporary cardiac outcomes. Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic...

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Veröffentlicht in:PloS one 2021-08, Vol.16 (8), p.e0255487-e0255487
Hauptverfasser: Itzhaki Ben Zadok, Osnat, Vaturi, Mordehay, Vaxman, Iuliana, Iakobishvili, Zaza, Rhurman-Shahar, Noa, Kornowski, Ran, Hamdan, Ashraf
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creator Itzhaki Ben Zadok, Osnat
Vaturi, Mordehay
Vaxman, Iuliana
Iakobishvili, Zaza
Rhurman-Shahar, Noa
Kornowski, Ran
Hamdan, Ashraf
description To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients' contemporary cardiac outcomes. Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients' cohort (years 2013-2020). Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p
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Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients' cohort (years 2013-2020). Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p&lt;0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm.sup.2 vs. 27 (IQR 23, 30)cm.sup.2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m.sup.2 vs. 82 (IQR 72, 98)grams/m.sup.2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). Despite the various similarities of AL-CA and ATTR-CA, these diseases have distinct baseline cardiovascular profiles and different heart failure course, thus merit tailored-cardiac management.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0255487</identifier><identifier>PMID: 34370783</identifier><language>eng</language><publisher>San Francisco: Public Library of Science</publisher><subject>Amyloid ; Amyloidosis ; Biology and Life Sciences ; Biopsy ; Cardiac arrhythmia ; Cardiology ; Cardiovascular disease ; Care and treatment ; Chains ; Congestive heart failure ; Coronary artery ; Coronary artery disease ; Diabetes mellitus ; Echocardiography ; Ejection fraction ; Heart failure ; Kidney diseases ; Laboratories ; Magnetic resonance ; Magnetic resonance imaging ; Medical prognosis ; Medicine ; Medicine and Health Sciences ; Normal distribution ; Patient outcomes ; Patients ; Pulmonary arteries ; Research and Analysis Methods ; Survival analysis ; Transthyretin ; Ventricle</subject><ispartof>PloS one, 2021-08, Vol.16 (8), p.e0255487-e0255487</ispartof><rights>COPYRIGHT 2021 Public Library of Science</rights><rights>2021 Itzhaki Ben Zadok et al. 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Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2021 Itzhaki Ben Zadok et al 2021 Itzhaki Ben Zadok et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c669t-991a770683e48f4d86bc2edf2da6386d7c6d2a55bba84315234c230595e2d1683</citedby><cites>FETCH-LOGICAL-c669t-991a770683e48f4d86bc2edf2da6386d7c6d2a55bba84315234c230595e2d1683</cites><orcidid>0000-0002-9259-4808</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8351987/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC8351987/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,315,728,781,785,865,886,2103,2929,23868,27926,27927,53793,53795</link.rule.ids></links><search><contributor>Lionetti, Vincenzo</contributor><creatorcontrib>Itzhaki Ben Zadok, Osnat</creatorcontrib><creatorcontrib>Vaturi, Mordehay</creatorcontrib><creatorcontrib>Vaxman, Iuliana</creatorcontrib><creatorcontrib>Iakobishvili, Zaza</creatorcontrib><creatorcontrib>Rhurman-Shahar, Noa</creatorcontrib><creatorcontrib>Kornowski, Ran</creatorcontrib><creatorcontrib>Hamdan, Ashraf</creatorcontrib><title>Differences in the characteristics and contemporary cardiac outcomes of patients with light-chain versus transthyretin cardiac amyloidosis</title><title>PloS one</title><description>To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients' contemporary cardiac outcomes. Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients' cohort (years 2013-2020). Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p&lt;0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm.sup.2 vs. 27 (IQR 23, 30)cm.sup.2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m.sup.2 vs. 82 (IQR 72, 98)grams/m.sup.2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). 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Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients' cohort (years 2013-2020). Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p&lt;0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm.sup.2 vs. 27 (IQR 23, 30)cm.sup.2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m.sup.2 vs. 82 (IQR 72, 98)grams/m.sup.2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). Despite the various similarities of AL-CA and ATTR-CA, these diseases have distinct baseline cardiovascular profiles and different heart failure course, thus merit tailored-cardiac management.</abstract><cop>San Francisco</cop><pub>Public Library of Science</pub><pmid>34370783</pmid><doi>10.1371/journal.pone.0255487</doi><tpages>e0255487</tpages><orcidid>https://orcid.org/0000-0002-9259-4808</orcidid><oa>free_for_read</oa></addata></record>
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subjects Amyloid
Amyloidosis
Biology and Life Sciences
Biopsy
Cardiac arrhythmia
Cardiology
Cardiovascular disease
Care and treatment
Chains
Congestive heart failure
Coronary artery
Coronary artery disease
Diabetes mellitus
Echocardiography
Ejection fraction
Heart failure
Kidney diseases
Laboratories
Magnetic resonance
Magnetic resonance imaging
Medical prognosis
Medicine
Medicine and Health Sciences
Normal distribution
Patient outcomes
Patients
Pulmonary arteries
Research and Analysis Methods
Survival analysis
Transthyretin
Ventricle
title Differences in the characteristics and contemporary cardiac outcomes of patients with light-chain versus transthyretin cardiac amyloidosis
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