{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T12:00:40Z","timestamp":1793016040171,"version":"4.5.0"},"reference-count":31,"publisher":"Oxford University Press (OUP)","issue":"27","license":[{"start":{"date-parts":[[2022,5,24]],"date-time":"2022-05-24T00:00:00Z","timestamp":1653350400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,7,14]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly diagnosed at an early stage of the disease natural history, defined as National Amyloidosis Centre (NAC) ATTR Stage I. The natural history of early-stage ATTR-CM remains poorly characterized.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods and results<\/jats:title>\n                  <jats:p>A retrospective multi-centre observational study of 879 patients with ATTR-CM, either wild-type TTR genotype or carrying the p.V142I TTR variant, and NAC ATTR Stage I biomarkers at the time of diagnosis who did not receive disease-modifying therapy for amyloidosis. Disease characteristics at diagnosis that were independently associated with mortality by Cox regression analysis were N-terminal pro-B-type natriuretic peptide (NT-proBNP), TTR genotype, and troponin T. Patients were categorized into NAC ATTR Stage Ia, defined as a furosemide equivalent diuretic requirement of &amp;lt;0.75\u2005mg\/kg and an NT-proBNP \u2264500\u2005ng\/L or \u22641000\u2005ng\/L in the presence of atrial fibrillation, and NAC ATTR Stage Ib comprising all remaining Stage I patients. Median estimated survival among the 88% NAC ATTR Stage Ib patients was 75 (95% CI 57\u201393) months compared with &amp;gt;100 months in the 12% with Stage Ia disease [hazard ratio for death 5.06 (95% confidence interval 1.23\u201320.87); P\u2009=\u20090.025] despite significant cardiovascular morbidity at the time of diagnosis which increased during follow-up, including among patients diagnosed in NAC ATTR Stage Ia. Estimated survival among UK NAC ATTR Stage Ia patients was comparable to UK general population controls (P\u2009=\u20090.297).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Patients with NAC ATTR Stage I ATTR-CM can be further stratified according to NT-proBNP concentration and diuretic requirement at diagnosis. Patients with Stage Ia ATTR-CM have significant cardiovascular morbidity despite good short- and mid-term survival.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/eurheartj\/ehac259","type":"journal-article","created":{"date-parts":[[2022,5,9]],"date-time":"2022-05-09T19:11:44Z","timestamp":1652123504000},"page":"2622-2632","source":"Crossref","is-referenced-by-count":70,"title":["Characteristics and natural history of early-stage cardiac transthyretin amyloidosis"],"prefix":"10.1093","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9335-5526","authenticated-orcid":false,"given":"Steven","family":"Law","sequence":"first","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1793-4099","authenticated-orcid":false,"given":"Melanie","family":"Bezard","sequence":"additional","affiliation":[{"name":"Referral 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