{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T14:26:35Z","timestamp":1791728795664,"version":"4.3.4"},"reference-count":24,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T00:00:00Z","timestamp":1764892800000},"content-version":"vor","delay-in-days":863,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T00:00:00Z","timestamp":1690329600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,11,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>The value of disease-modifying therapies (such as tafamidis) in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) and severe heart failure symptoms has been debated. This study assessed long-term all-cause survival in patients with New York Heart Association (NYHA) class III symptoms in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT) long-term extension (LTE) study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>At the baseline of ATTR-ACT, 55\/176 (31.3%) patients receiving tafamidis 80\u2009mg and 63\/177 (35.6%) receiving placebo had NYHA class III symptoms. After 30\u2009months of treatment, patients could join an ongoing LTE study to receive open-label tafamidis. In an interim analysis of the LTE study (August 2021), all-cause mortality was lower among patients with NYHA class III symptoms who received continuous tafamidis in ATTR-ACT and the LTE study (hazard ratio 0.64; 95% confidence interval 0.41\u20130.99; median follow-up: 60\u2009months), as compared with those who received placebo in ATTR-ACT and tafamidis in the LTE study (median follow-up: 56\u2009months). Similar findings were observed in patients with NYHA class I\/II symptoms at baseline (0.50; 0.35\u20130.73; tafamidis 80\u2009mg n\u2009=\u2009121; placebo n\u2009=\u2009114; median follow-up of 61 and 60\u2009months, respectively).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>We observed reduced all-cause mortality with continuous tafamidis treatment compared with delayed tafamidis treatment (placebo then tafamidis) in patients with NYHA class III symptoms at baseline over a median follow-up of \u223c5\u2009years. These findings demonstrate the value of tafamidis treatment in patients with ATTR-CM and severe heart failure symptoms, and emphasize the importance of early treatment.<\/jats:p>\n                    <jats:p>Clinical Trial Registrations: ClinicalTrials.gov \u00a0NCT01994889 and NCT02791230.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ejhf.2974","type":"journal-article","created":{"date-parts":[[2023,7,12]],"date-time":"2023-07-12T02:14:00Z","timestamp":1689128040000},"page":"2060-2064","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":67,"title":["Improved Long-Term Survival with Tafamidis Treatment in Patients with Transthyretin Amyloid Cardiomyopathy and Severe Heart Failure Symptoms"],"prefix":"10.1093","volume":"25","author":[{"given":"Perry","family":"Elliott","sequence":"first","affiliation":[{"name":"University College London , London 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