Clinical characteristics and outcome of end stage hypertrophic cardiomyopathy: Role of age and heart failure phenotypes
A minority of patients with hypertrophic cardiomyopathy (HCM) presents advanced heart failure (HF) during their clinical course, in the context of left ventricular (LV) remodeling with reduced LV ejection fraction (LVEF), or of severe diastolic dysfunction without impaired LVEF. Aim of this study wa...
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| Veröffentlicht in: | International journal of cardiology 2024-04, Vol.400, p.131784-131784, Article 131784 |
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| creator | Musumeci, Beatrice Tini, Giacomo Biagini, Elena Merlo, Marco Calore, Chiara Ammirati, Enrico Zampieri, Mattia Russo, Domitilla Grilli, Giulia Santolamazza, Caterina Vio, Riccardo Rubino, Marta Ditaranto, Raffaello Del Franco, Annamaria Sormani, Paola Parisi, Vanda Monda, Emanuele Francia, Pietro Cipriani, Alberto Limongelli, Giuseppe Sinagra, Gianfranco Olivotto, Iacopo Boni, Luca Autore, Camillo |
| description | A minority of patients with hypertrophic cardiomyopathy (HCM) presents advanced heart failure (HF) during their clinical course, in the context of left ventricular (LV) remodeling with reduced LV ejection fraction (LVEF), or of severe diastolic dysfunction without impaired LVEF. Aim of this study was to describe a multicentric end stage (ES) HCM population and analyze clinical course and outcome among its different phenotypes.
Data of all HCM patients from 7 Italian referral centres were retrospectively evaluated. ES was diagnosed in presence of: LVEF |
| doi_str_mv | 10.1016/j.ijcard.2024.131784 |
| format | Article |
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Data of all HCM patients from 7 Italian referral centres were retrospectively evaluated. ES was diagnosed in presence of: LVEF <50% (ES-rEF) or NYHA functional class ≥II with severe diastolic dysfunction (ES-pEF). Outcomes were: HCM-related and all-cause mortality; combined arrhythmic events; advanced HF treatments.
Study population included 331 ES patients; 87% presented ES-rEF and 13% ES-pEF. At ES recognition, patients with ES-pEF were more commonly females, had more frequently NYHA III/IV, atrial fibrillation and greater maximal LV wall thickness. Over a median follow-up of 5.6 years, 83 (25%) patients died, 46 (15%) experienced arrhythmic events and (26%) 85 received advanced HF treatments. Incidence of HCM-related and all-cause mortality, and of combined arrhythmic events did not differ in ES-pEF and ES-rEF patients, but ES-pEF patients were less likely to receive advanced HF treatments. Older age at ES recognition was an independent predictor of increased HCM-related mortality (p = 0.01) and reduced access to advanced HF treatments (p < 0.0001).
Two different HCM-ES phenotypes can be recognized, with ES-pEF showing distinctive features at ES recognition and receiving less frequently advanced HF treatments. Older age at ES recognition has a major impact on outcomes.
•End stage (ES) hypertrophic cardiomyopathy presents with reduced LV ejection fraction (ES-rEF), or with severe diastolic dysfunction and preserved EF (ES-pEF).•Prognosis is similar in ES-rEF and ES-pEF.•Patients with ES-pEF patients or older at ES recognition are less likely to receive advanced heart failure treatments.</description><identifier>ISSN: 0167-5273</identifier><identifier>EISSN: 1874-1754</identifier><identifier>DOI: 10.1016/j.ijcard.2024.131784</identifier><identifier>PMID: 38242504</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Advanced heart failure ; End stage ; Hypertrophic cardiomyopathy ; Left ventricular remodeling</subject><ispartof>International journal of cardiology, 2024-04, Vol.400, p.131784-131784, Article 131784</ispartof><rights>2024 Elsevier B.V.</rights><rights>Copyright © 2024. Published by Elsevier B.V.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c311t-4bd3fe4d488936317e214edf32aeab70f818956c197b2d9a0eea4603f365bbf23</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.ijcard.2024.131784$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38242504$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Musumeci, Beatrice</creatorcontrib><creatorcontrib>Tini, Giacomo</creatorcontrib><creatorcontrib>Biagini, Elena</creatorcontrib><creatorcontrib>Merlo, Marco</creatorcontrib><creatorcontrib>Calore, Chiara</creatorcontrib><creatorcontrib>Ammirati, Enrico</creatorcontrib><creatorcontrib>Zampieri, Mattia</creatorcontrib><creatorcontrib>Russo, Domitilla</creatorcontrib><creatorcontrib>Grilli, Giulia</creatorcontrib><creatorcontrib>Santolamazza, Caterina</creatorcontrib><creatorcontrib>Vio, Riccardo</creatorcontrib><creatorcontrib>Rubino, Marta</creatorcontrib><creatorcontrib>Ditaranto, Raffaello</creatorcontrib><creatorcontrib>Del Franco, Annamaria</creatorcontrib><creatorcontrib>Sormani, Paola</creatorcontrib><creatorcontrib>Parisi, Vanda</creatorcontrib><creatorcontrib>Monda, Emanuele</creatorcontrib><creatorcontrib>Francia, Pietro</creatorcontrib><creatorcontrib>Cipriani, Alberto</creatorcontrib><creatorcontrib>Limongelli, Giuseppe</creatorcontrib><creatorcontrib>Sinagra, Gianfranco</creatorcontrib><creatorcontrib>Olivotto, Iacopo</creatorcontrib><creatorcontrib>Boni, Luca</creatorcontrib><creatorcontrib>Autore, Camillo</creatorcontrib><title>Clinical characteristics and outcome of end stage hypertrophic cardiomyopathy: Role of age and heart failure phenotypes</title><title>International journal of cardiology</title><addtitle>Int J Cardiol</addtitle><description>A minority of patients with hypertrophic cardiomyopathy (HCM) presents advanced heart failure (HF) during their clinical course, in the context of left ventricular (LV) remodeling with reduced LV ejection fraction (LVEF), or of severe diastolic dysfunction without impaired LVEF. Aim of this study was to describe a multicentric end stage (ES) HCM population and analyze clinical course and outcome among its different phenotypes.
Data of all HCM patients from 7 Italian referral centres were retrospectively evaluated. ES was diagnosed in presence of: LVEF <50% (ES-rEF) or NYHA functional class ≥II with severe diastolic dysfunction (ES-pEF). Outcomes were: HCM-related and all-cause mortality; combined arrhythmic events; advanced HF treatments.
Study population included 331 ES patients; 87% presented ES-rEF and 13% ES-pEF. At ES recognition, patients with ES-pEF were more commonly females, had more frequently NYHA III/IV, atrial fibrillation and greater maximal LV wall thickness. Over a median follow-up of 5.6 years, 83 (25%) patients died, 46 (15%) experienced arrhythmic events and (26%) 85 received advanced HF treatments. Incidence of HCM-related and all-cause mortality, and of combined arrhythmic events did not differ in ES-pEF and ES-rEF patients, but ES-pEF patients were less likely to receive advanced HF treatments. Older age at ES recognition was an independent predictor of increased HCM-related mortality (p = 0.01) and reduced access to advanced HF treatments (p < 0.0001).
Two different HCM-ES phenotypes can be recognized, with ES-pEF showing distinctive features at ES recognition and receiving less frequently advanced HF treatments. Older age at ES recognition has a major impact on outcomes.
•End stage (ES) hypertrophic cardiomyopathy presents with reduced LV ejection fraction (ES-rEF), or with severe diastolic dysfunction and preserved EF (ES-pEF).•Prognosis is similar in ES-rEF and ES-pEF.•Patients with ES-pEF patients or older at ES recognition are less likely to receive advanced heart failure treatments.</description><subject>Advanced heart failure</subject><subject>End stage</subject><subject>Hypertrophic cardiomyopathy</subject><subject>Left ventricular remodeling</subject><issn>0167-5273</issn><issn>1874-1754</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNp9kE2L1TAUhoM4ONfRfyCSpZte89UmdSHIxS8YEIaZdUiTE5tL29QkVe6_t7WjS1eHA897Ph6EXlFypIQ2b8_HcLYmuSMjTBwpp1KJJ-hAlRQVlbV4ig4rJquaSX6Nnud8JoSItlXP0DVXTLCaiAP6dRrCFKwZsO1NMrZACrkEm7GZHI5LsXEEHD2Gtc3FfAfcX2ZIJcW5DxZvF4Q4XuJsSn95h-_i8AffwG1CDyYV7E0YlgR47mGKZc3nF-jKmyHDy8d6gx4-fbw_faluv33-evpwW1lOaalE57gH4YRSLW_WH4FRAc5zZsB0knhFVVs3lrayY641BMCIhnDPm7rrPOM36M0-d07xxwK56DFkC8NgJohL1qylqiatFHJFxY7aFHNO4PWcwmjSRVOiN-X6rHflelOud-Vr7PXjhqUbwf0L_XW8Au93ANY_fwZIOtsAkwUXEtiiXQz_3_AbOF6WEw</recordid><startdate>20240401</startdate><enddate>20240401</enddate><creator>Musumeci, Beatrice</creator><creator>Tini, Giacomo</creator><creator>Biagini, Elena</creator><creator>Merlo, Marco</creator><creator>Calore, Chiara</creator><creator>Ammirati, Enrico</creator><creator>Zampieri, Mattia</creator><creator>Russo, Domitilla</creator><creator>Grilli, Giulia</creator><creator>Santolamazza, Caterina</creator><creator>Vio, Riccardo</creator><creator>Rubino, Marta</creator><creator>Ditaranto, Raffaello</creator><creator>Del Franco, Annamaria</creator><creator>Sormani, Paola</creator><creator>Parisi, Vanda</creator><creator>Monda, Emanuele</creator><creator>Francia, Pietro</creator><creator>Cipriani, Alberto</creator><creator>Limongelli, Giuseppe</creator><creator>Sinagra, Gianfranco</creator><creator>Olivotto, Iacopo</creator><creator>Boni, Luca</creator><creator>Autore, Camillo</creator><general>Elsevier B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20240401</creationdate><title>Clinical characteristics and outcome of end stage hypertrophic cardiomyopathy: Role of age and heart failure phenotypes</title><author>Musumeci, Beatrice ; 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Aim of this study was to describe a multicentric end stage (ES) HCM population and analyze clinical course and outcome among its different phenotypes.
Data of all HCM patients from 7 Italian referral centres were retrospectively evaluated. ES was diagnosed in presence of: LVEF <50% (ES-rEF) or NYHA functional class ≥II with severe diastolic dysfunction (ES-pEF). Outcomes were: HCM-related and all-cause mortality; combined arrhythmic events; advanced HF treatments.
Study population included 331 ES patients; 87% presented ES-rEF and 13% ES-pEF. At ES recognition, patients with ES-pEF were more commonly females, had more frequently NYHA III/IV, atrial fibrillation and greater maximal LV wall thickness. Over a median follow-up of 5.6 years, 83 (25%) patients died, 46 (15%) experienced arrhythmic events and (26%) 85 received advanced HF treatments. Incidence of HCM-related and all-cause mortality, and of combined arrhythmic events did not differ in ES-pEF and ES-rEF patients, but ES-pEF patients were less likely to receive advanced HF treatments. Older age at ES recognition was an independent predictor of increased HCM-related mortality (p = 0.01) and reduced access to advanced HF treatments (p < 0.0001).
Two different HCM-ES phenotypes can be recognized, with ES-pEF showing distinctive features at ES recognition and receiving less frequently advanced HF treatments. Older age at ES recognition has a major impact on outcomes.
•End stage (ES) hypertrophic cardiomyopathy presents with reduced LV ejection fraction (ES-rEF), or with severe diastolic dysfunction and preserved EF (ES-pEF).•Prognosis is similar in ES-rEF and ES-pEF.•Patients with ES-pEF patients or older at ES recognition are less likely to receive advanced heart failure treatments.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>38242504</pmid><doi>10.1016/j.ijcard.2024.131784</doi><tpages>1</tpages></addata></record> |
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| subjects | Advanced heart failure End stage Hypertrophic cardiomyopathy Left ventricular remodeling |
| title | Clinical characteristics and outcome of end stage hypertrophic cardiomyopathy: Role of age and heart failure phenotypes |
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