{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,10]],"date-time":"2026-11-10T19:12:30Z","timestamp":1794337950627,"version":"4.9.0"},"reference-count":98,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T00:00:00Z","timestamp":1751846400000},"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>Transthyretin-related (ATTR) amyloidosis is a progressive, multisystem disease caused by the extracellular deposition of misfolded transthyretin (TTR) monomers as insoluble amyloid fibrils. Clinical manifestations vary widely and may include cardiomyopathy (ATTR-CM), polyneuropathy (ATTR-PN), or mixed phenotypes. The condition is increasingly recognized as an underdiagnosed contributor to heart failure, particularly in elderly patients. ATTR amyloidosis exists in two major forms: hereditary (ATTRv), resulting from mutations in the TTR gene, and wild-type (ATTRwt), typically affecting men over 70 years of age. Advances in disease understanding have led to a paradigm shift in management, with the introduction of targeted therapies that slow disease progression and improve prognosis. First-generation therapies such as tafamidis have demonstrated survival benefits in ATTR-CM. More recently, second-generation agents\u2014such as the TTR stabilizer acoramidis and RNA silencers including vutrisiran and eplontersen\u2014have shown promising efficacy in clinical trials. Additional strategies under investigation include gene editing and monoclonal antibodies targeting TTR amyloid deposits. This review outlines current diagnostic strategies and therapeutic options for ATTR amyloidosis, emphasizing the need for early detection and individualized treatment approaches. The expanding therapeutic landscape highlights the importance of accurate phenotyping and timely intervention to optimize clinical outcomes.<\/jats:p>","DOI":"10.3390\/jcm14134785","type":"journal-article","created":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T08:58:23Z","timestamp":1751878703000},"page":"4785","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Transthyretin Amyloid Cardiomyopathy\u20142025 Update: Current Diagnostic Approaches and Emerging Therapeutic Options"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5243-8985","authenticated-orcid":false,"given":"Carsten","family":"Tsch\u00f6pe","sequence":"first","affiliation":[{"name":"Department of Cardiology, Angiology, and Intensive Medicine (CVK), German Heart Center at Charit\u00e9 (DHZC), Charit\u00e9\u2014University Medicine Berlin, 13353 Berlin, Germany"},{"name":"Berlin Institute of Health (BIH), Center for Regenerative Therapies (BCRT), Charit\u00e9\u2014University Medicine Berlin, 13353 Berlin, Germany"},{"name":"Amyloidosis Center, Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin (ACCB), Corporate Member of Freie Universit\u00e4t Berlin and Humboldt-Universit\u00e4t zu Berlin, Charit\u00e9platz 1, 10117 Berlin, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Berlin, 13353 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Elsanhoury","sequence":"additional","affiliation":[{"name":"Berlin Institute of Health (BIH), Center for Regenerative Therapies (BCRT), Charit\u00e9\u2014University Medicine Berlin, 13353 Berlin, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Berlin, 13353 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnt V.","family":"Kristen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Angiology and Respiratory Medicine, University Hospital Heidelberg, 69120 Heidelberg, Germany"},{"name":"Kardiovaskul\u00e4res Zentrum Darmstadt, 64287 Darmstadt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,7]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2641","DOI":"10.1016\/S0140-6736(15)01274-X","article-title":"Systemic amyloidosis","volume":"387","author":"Wechalekar","year":"2016","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1080\/13506129.2024.2405948","article-title":"Amyloid nomenclature 2024: Update, novel proteins, and recommendations by the International Society of Amyloidosis (ISA) Nomenclature Committee","volume":"31","author":"Buxbaum","year":"2024","journal-title":"Amyloid"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1146\/annurev-biochem-061516-045115","article-title":"Protein Misfolding, Amyloid Formation, and Human Disease: A Summary of Progress over the Last Decade","volume":"86","author":"Chiti","year":"2017","journal-title":"Annu. 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