{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T06:52:31Z","timestamp":1781765551517,"version":"3.54.5"},"reference-count":24,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T00:00:00Z","timestamp":1781136000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Advances in diagnostic criteria for transthyretin cardiac amyloidosis (ATTR-CM) and expanded insurance coverage for bone scintigraphy have facilitated earlier detection of ATTR-CM. However, whether these changes have translated into improved clinical outcomes among patients receiving disease-modifying therapy remains uncertain, especially in non-high-volume centers. Methods: Consecutive patients with ATTR-CM who started disease-modifying therapy at our institute between May 2019 and March 2025 were retrospectively analyzed. Baseline characteristics and clinical outcomes were compared between the early period (2019\u20132021) and the late period (2021\u20132025). Results: A total of 31 patients (median age 77 years, 77% male) were included. Duration of heart failure was significantly shorter and the dose of loop diuretics at baseline was significantly lower in the late period (p &lt; 0.05 for both). The prevalence of National Amyloid Center (NAC) stage I at baseline tended to be higher in the late period (75.0% versus 53.5%, p = 0.273). The cumulative incidence of worsening heart failure hospitalization and all-cause death was significantly lower in the late period (6.3% versus 44.2%, p = 0.024) during a median follow-up of 5 years. NAC stage I at baseline was independently associated with the lower primary outcome with an adjusted hazard ratio of 0.10 (95% confidence interval 0.01\u20130.90, p = 0.040). Conclusions: Patients with ATTR-CM in the late group experienced more favorable clinical outcomes after disease-modifying therapy, probably due to earlier diagnosis and therapeutic intervention, although further studies are warranted to verify the hypothesis.<\/jats:p>","DOI":"10.3390\/jcm15124546","type":"journal-article","created":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T01:52:08Z","timestamp":1781229128000},"page":"4546","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Better Outcomes After Initiation of Disease-Modifying Therapy in Patients with Transthyretin Cardiac Amyloidosis"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9604-5603","authenticated-orcid":false,"given":"Makiko","family":"Nakamura","sequence":"first","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7294-7637","authenticated-orcid":false,"given":"Teruhiko","family":"Imamura","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4765-9593","authenticated-orcid":false,"given":"Masaki","family":"Nakagaito","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryuichi","family":"Ushijima","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Koichiro","family":"Kinugawa","sequence":"additional","affiliation":[{"name":"Second Department of Internal Medicine, University of Toyama, Toyama 930-0194, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,6,11]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1093\/eurheartj\/ehaf667","article-title":"Transthyretin amyloid cardiomyopathy: From cause to novel treatments","volume":"47","author":"Fontana","year":"2026","journal-title":"Eur. Heart J."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1554","DOI":"10.1161\/CIRCRESAHA.121.318187","article-title":"Pathophysiology and Therapeutic Approaches to Cardiac Amyloidosis","volume":"128","author":"Griffin","year":"2021","journal-title":"Circ. Res."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Cianci, V., Cianci, A., Sapienza, D., Crac\u00f2, A., German\u00e0, A., Ieni, A., Gualniera, P., Asmundo, A., and Mondello, C. (2024). Epidemiological Changes in Transthyretin Cardiac Amyloidosis: Evidence from In Vivo Data and Autoptic Series. J. Clin. Med., 13.","DOI":"10.3390\/jcm13175140"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2404","DOI":"10.1161\/CIRCULATIONAHA.116.021612","article-title":"Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis","volume":"133","author":"Gillmore","year":"2016","journal-title":"Circulation"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1002\/ehf2.13693","article-title":"99mTechnetium-pyrophosphate scintigraphy: A practical guide for early diagnosis of transthyretin amyloid cardiomyopathy","volume":"9","author":"Tahara","year":"2022","journal-title":"ESC Heart Fail."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1007\/s10741-024-10444-4","article-title":"Contextualizing the results of HELIOS-B in the broader landscape of clinical trials for the treatment of transthyretin cardiac amyloidosis","volume":"30","author":"Girard","year":"2025","journal-title":"Heart Fail. Rev."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1253\/circj.CJ-19-0811","article-title":"A Statement on the Appropriate Administration of Tafamidis in Patients With Transthyretin Cardiac Amyloidosis","volume":"84","author":"Endo","year":"2019","journal-title":"Circ. J."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1161\/CIRCULATIONAHA.119.045093","article-title":"Cost-Effectiveness of Tafamidis Therapy for Transthyretin Amyloid Cardiomyopathy","volume":"141","author":"Kazi","year":"2020","journal-title":"Circulation"},{"key":"ref_9","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_10","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.echo.2014.10.003","article-title":"Recommendations for cardiac chamber quantification by echocardiography in adults: An update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging","volume":"28","author":"Lang","year":"2015","journal-title":"J. Am. Soc. Echocardiogr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1554","DOI":"10.1093\/eurheartj\/ehab072","article-title":"Diagnosis and treatment of cardiac amyloidosis: A position statement of the ESC Working Group on Myocardial and Pericardial Diseases","volume":"42","author":"Rapezzi","year":"2021","journal-title":"Eur. Heart J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1253\/circrep.CR-25-0092","article-title":"Real-World Clinical Evidence with Tafamidis in Patients with Transthyretin Amyloid Cardiomyopathy\u2014A Contemporary Review","volume":"7","author":"Izumiya","year":"2025","journal-title":"Circ. Rep."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1896","DOI":"10.1002\/ehf2.14364","article-title":"Prevalence of transthyretin amyloidosis among heart failure patients with preserved ejection fraction in Japan","volume":"10","author":"Naito","year":"2023","journal-title":"ESC Heart Fail."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.1002\/ehf2.15112","article-title":"Prevalence of transthyretin cardiac amyloidosis in undifferentiated heart failure with preserved ejection fraction","volume":"12","author":"Healy","year":"2025","journal-title":"ESC Heart Fail."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/j.jacc.2025.04.055","article-title":"Impact of Vutrisiran on Cardiac Biomarkers in Patients With Transthyretin Amyloidosis With Cardiomyopathy From HELIOS-B","volume":"86","author":"Maurer","year":"2025","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"e034518","DOI":"10.1161\/JAHA.124.034518","article-title":"Cardiac Biomarker Change at 1 Year After Tafamidis Treatment and Clinical Outcomes in Patients With Transthyretin Amyloid Cardiomyopathy","volume":"13","author":"Kuyama","year":"2024","journal-title":"J. Am. Heart Assoc."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2411","DOI":"10.1016\/j.jacc.2024.03.429","article-title":"SGLT2 Inhibitor Therapy in Patients With Transthyretin Amyloid Cardiomyopathy","volume":"83","author":"Porcari","year":"2024","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1016\/j.jaccao.2020.06.007","article-title":"Diuretic Dose and NYHA Functional Class Are Independent Predictors of Mortality in Patients With Transthyretin Cardiac Amyloidosis","volume":"2","author":"Cheng","year":"2020","journal-title":"JACC Cardio Oncol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1016\/j.jacc.2024.11.015","article-title":"Outpatient Worsening Heart Failure in Patients With Transthyretin Amyloidosis With Cardiomyopathy in the HELIOS-B Trial","volume":"85","author":"Fontana","year":"2025","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1080\/13506129.2024.2376202","article-title":"Response to therapy with tafamidis 61\u2009mg in patients with cardiac transthyretin amyloidosis: Real-world experience since approval","volume":"31","author":"Meissner","year":"2024","journal-title":"Amyloid"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2319","DOI":"10.1002\/ehf2.14380","article-title":"Clinical characteristics, outcome, and therapeutic effect of tafamidis in wild-type transthyretin amyloid cardiomyopathy","volume":"10","author":"Takashio","year":"2023","journal-title":"ESC Heart Fail."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1016\/j.amjcard.2023.09.055","article-title":"Myocardial Work Appraisal in Transthyretin Cardiac Amyloidosis and Nonobstructive Hypertrophic Cardiomyopathy","volume":"208","author":"Trimarchi","year":"2023","journal-title":"Am. J. Cardiol."},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Pugliatti, P., Trimarchi, G., Barocelli, F., Pizzino, F., Di Spigno, F., Tedeschi, A., Piccione, M.C., Irrera, P., Aschieri, D., and Niccoli, G. (2024). Advancing Cardiac Amyloidosis Care Through Insights from Cardiopulmonary Exercise Testing. J. Clin. Med., 13.","DOI":"10.3390\/jcm13237285"},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"de Gregorio, C., Trimarchi, G., Faro, D.C., Poleggi, C., Teresi, L., De Gaetano, F., Zito, C., Lofrumento, F., Koniari, I., and Licordari, R. (2024). Systemic Vascular Resistance and Myocardial Work Analysis in Hypertrophic Cardiomyopathy and Transthyretin Cardiac Amyloidosis with Preserved Left Ventricular Ejection Fraction. J. Clin. Med., 13.","DOI":"10.20944\/preprints202403.0569.v1"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/12\/4546\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T06:13:44Z","timestamp":1781763224000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/12\/4546"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,11]]},"references-count":24,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2026,6]]}},"alternative-id":["jcm15124546"],"URL":"https:\/\/doi.org\/10.3390\/jcm15124546","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,11]]}}}