{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T21:07:38Z","timestamp":1792789658228,"version":"4.5.0"},"reference-count":13,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T00:00:00Z","timestamp":1700697600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"M.H.\u2019s Term Chair for Amyloid Heart Disease"},{"name":"Karos Research Award for training in clinical investigation"},{"name":"Ionis Therapeutics Inc."}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Transthyretin cardiac amyloidosis (ATTR-CM) is classically thought of as a progressive disease with preserved systolic function. The longitudinal clinical trajectories of ATTR-CM with impaired left ventricular ejection fraction (LVEF) remain unclear. Methods: This is a single-center retrospective cohort study of consecutive patients with ATTR-CM who underwent two or more echocardiograms with baseline LVEF &lt; 50%. Patients were stratified according to the presence of \u22655% change in LVEF. A Cox proportional hazard model examined hazard of a composite outcome of death, transplant, or LVAD insertion over the two years following diagnosis. Results: In our study cohort of 179 patients, 62 patients (34.6%) experienced an increase in LVEF while 33 (18.4%) experienced a decrease in LVEF. After adjusting for covariates, patients with a decrease in EF experienced increased hazard of death (HR 2.15, 95% CI 1.05\u20134.40, p = 0.038) compared to those with stable or an increase in LVEF. Changes in LVEF corresponded with significant differences in NT proBNP trajectories, but initial biomarker levels or clinical staging were not predictive of LVEF trajectory. Conclusions: in ATTR-CM patients with impaired LVEF, over a third demonstrated improved LVEF over time, while those with a decrease in LVEF had worse long-term outcomes.<\/jats:p>","DOI":"10.3390\/jcm12237250","type":"journal-article","created":{"date-parts":[[2023,11,23]],"date-time":"2023-11-23T03:45:56Z","timestamp":1700711156000},"page":"7250","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Changes in Left Ventricular Ejection Fraction and Clinical Trajectories of Transthyretin Cardiac Amyloidosis with Systolic Dysfunction"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1896-6423","authenticated-orcid":false,"given":"Joshua","family":"Saef","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"},{"name":"Joe DiMaggio Children\u2019s Hospital and the Memorial Healthcare Total Heart Center, Hollywood, FL 33021, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Trejeeve","family":"Martyn","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anusha","family":"Ray Dey","sequence":"additional","affiliation":[{"name":"Department of Medical Science, Brown University, Providence, RI 02912, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rola","family":"Khedraki","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Scripps Clinic and Research Foundation, La Jolla, CA 92037, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lauren","family":"Ives","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3941-9653","authenticated-orcid":false,"given":"Patrick","family":"Collier","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wael A.","family":"Jaber","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerry D.","family":"Estep","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic Florida, Weston, FL 33331, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mazen","family":"Hanna","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8335-735X","authenticated-orcid":false,"given":"Wai Hong Wilson","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,11,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"27","DOI":"10.14797\/mdcvj.1163","article-title":"Opportunities for Earlier Diagnosis and Treatment of Cardiac Amyloidosis","volume":"18","author":"Martyn","year":"2022","journal-title":"Methodist DeBakey Cardiovasc. J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1161\/CIRCULATIONAHA.118.038169","article-title":"Natural History, Quality of Life, and Outcome in Cardiac Transthyretin Amyloidosis","volume":"140","author":"Lane","year":"2019","journal-title":"Circulation"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2872","DOI":"10.1016\/j.jacc.2019.04.003","article-title":"Transthyretin Amyloid Cardiomyopathy: JACC State-of-the-Art Review","volume":"73","author":"Ruberg","year":"2019","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1016\/j.jchf.2022.06.006","article-title":"Racial and Genetic Differences in Presentation of Transthyretin Amyloid Cardiomyopathy with Impaired Left Ventricular Function","volume":"10","author":"Martyn","year":"2022","journal-title":"JACC Heart Fail."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e006075","DOI":"10.1161\/CIRCHEARTFAILURE.119.006075","article-title":"Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis","volume":"12","author":"Maurer","year":"2019","journal-title":"Circ. Heart Fail."},{"key":"ref_6","first-page":"e895","article-title":"2022 AHA\/ACC\/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology\/American Heart Association Joint Committee on Clinical Practice Guidelines","volume":"145","author":"Heidenreich","year":"2022","journal-title":"Circulation"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"e022859","DOI":"10.1161\/JAHA.121.022859","article-title":"Lack of Association between Neurohormonal Blockade and Survival in Transthyretin Cardiac Amyloidosis","volume":"10","author":"Cheng","year":"2021","journal-title":"J. Am. Heart Assoc."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"2893","DOI":"10.1093\/eurheartj\/ehad347","article-title":"Conventional heart failure therapy in cardiac ATTR amyloidosis","volume":"44","author":"Ioannou","year":"2023","journal-title":"Eur. Heart J."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1056\/NEJMoa1409077","article-title":"Angiotensin-neprilysin inhibition versus enalapril in heart failure","volume":"371","author":"McMurray","year":"2014","journal-title":"N. Engl. J. Med."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1995","DOI":"10.1056\/NEJMoa1911303","article-title":"Dapagliflozin in Patients with Heart Failure and Reduced Ejection Fraction","volume":"381","author":"McMurray","year":"2019","journal-title":"N. Engl. J. Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2799","DOI":"10.1093\/eurheartj\/ehx589","article-title":"A new staging system for cardiac transthyretin amyloidosis","volume":"39","author":"Gillmore","year":"2018","journal-title":"Eur. Heart J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1056\/NEJMoa1805689","article-title":"Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy","volume":"379","author":"Maurer","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"S23","DOI":"10.1016\/j.amjcard.2022.10.014","article-title":"Overview of Current and Emerging Therapies for Amyloid Transthyretin Cardiomyopathy","volume":"185","author":"Maurer","year":"2022","journal-title":"Am. J. Cardiol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/23\/7250\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T21:27:59Z","timestamp":1760131679000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/23\/7250"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11,23]]},"references-count":13,"journal-issue":{"issue":"23","published-online":{"date-parts":[[2023,12]]}},"alternative-id":["jcm12237250"],"URL":"https:\/\/doi.org\/10.3390\/jcm12237250","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,11,23]]}}}