{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T21:52:06Z","timestamp":1790459526558,"version":"4.1.0"},"reference-count":58,"publisher":"Oxford University Press (OUP)","issue":"45","license":[{"start":{"date-parts":[[2019,5,20]],"date-time":"2019-05-20T00:00:00Z","timestamp":1558310400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Transthyretin (TTR) is a tetrameric protein synthesized mostly by the liver. As a result of gene mutations or as an ageing-related phenomenon, TTR molecules may misfold and deposit in the heart and in other organs as amyloid fibrils. Cardiac involvement in TTR-related amyloidosis (ATTR) manifests typically as left ventricular pseudohypertrophy and\/or heart failure with preserved ejection fraction. ATTR is an underdiagnosed disorder as well as a crucial determinant of morbidity and mortality, thus justifying the current quest for a safe and effective treatment. Therapies targeting cardiac damage and its direct consequences may yield limited benefit, mostly related to dyspnoea relief through diuretics. For many years, liver or combined heart and liver transplantation have been the only available treatments for patients with mutations causing ATTR, including those with cardiac involvement. The therapeutic options now include several pharmacological agents that inhibit hepatic synthesis of TTR, stabilize the tetramer, or disrupt fibrils. Following the positive results of a phase 3 trial on tafamidis, and preliminary findings on patisiran and inotersen in patients with ATTR-related neuropathy and cardiac involvement, we provide an update on this rapidly evolving field, together with practical recommendations on the management of cardiac involvement.<\/jats:p>\n               <jats:p\/>","DOI":"10.1093\/eurheartj\/ehz298","type":"journal-article","created":{"date-parts":[[2019,4,23]],"date-time":"2019-04-23T19:14:30Z","timestamp":1556046870000},"page":"3699-3706","source":"Crossref","is-referenced-by-count":143,"title":["Treatment of cardiac transthyretin amyloidosis: an update"],"prefix":"10.1093","volume":"40","author":[{"given":"Michele","family":"Emdin","sequence":"first","affiliation":[{"name":"Institute of Life Sciences, Scuola Superiore Sant\u2019Anna , Piazza Martiri della Libert\u00e0 33, Pisa, Italy"},{"name":"Cardiology Department, Fondazione Toscana Gabriele Monasterio , Via G. Moruzzi 1, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Aimo","sequence":"additional","affiliation":[{"name":"Cardiology Division, University Hospital of Pisa , via Paradisa 2, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Rapezzi","sequence":"additional","affiliation":[{"name":"Cardiology Division, University Hospital of Bologna , via Massarenti 9, Bologna, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9233-9831","authenticated-orcid":false,"given":"Marianna","family":"Fontana","sequence":"additional","affiliation":[{"name":"Institute of Cardiovascular Science, University College London , 62 Huntley St, Fitzrovia, London, UK"},{"name":"National Amyloidosis Centre, University College London, Royal Free Hospital , Gower Street, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0276-8331","authenticated-orcid":false,"given":"Federico","family":"Perfetto","sequence":"additional","affiliation":[{"name":"Regional Amyloid Centre, Azienda Ospedaliero Universitaria Careggi , Largo Piero Palagi 1, Florence, Italy"},{"name":"Department of Internal and Experimental Medicine, University of Florence , Largo Piero Palagi 1, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petar M","family":"Seferovi\u0107","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Belgrade University School of Medicine , Dr Subotica 8, Belgrade, Serbia"},{"name":"Cardiology Department, University Institute for Cardiovascular Diseases , Heroja Milana Tepi\u0107a 1, Belgrade, Serbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9042-340X","authenticated-orcid":false,"given":"Andrea","family":"Barison","sequence":"additional","affiliation":[{"name":"Institute of Life Sciences, Scuola Superiore Sant\u2019Anna , Piazza Martiri della Libert\u00e0 33, Pisa, Italy"},{"name":"Cardiology Department, Fondazione Toscana Gabriele Monasterio , Via G. Moruzzi 1, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Castiglione","sequence":"additional","affiliation":[{"name":"Institute of Life Sciences, Scuola Superiore Sant\u2019Anna , Piazza Martiri della Libert\u00e0 33, Pisa, Italy"},{"name":"Cardiology Division, University Hospital of Pisa , via Paradisa 2, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Vergaro","sequence":"additional","affiliation":[{"name":"Institute of Life Sciences, Scuola Superiore Sant\u2019Anna , Piazza Martiri della Libert\u00e0 33, Pisa, Italy"},{"name":"Cardiology Department, Fondazione Toscana Gabriele Monasterio , Via G. Moruzzi 1, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Giannoni","sequence":"additional","affiliation":[{"name":"Institute of Life Sciences, Scuola Superiore Sant\u2019Anna , Piazza Martiri della Libert\u00e0 33, Pisa, Italy"},{"name":"Cardiology Department, Fondazione Toscana Gabriele Monasterio , Via G. Moruzzi 1, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudio","family":"Passino","sequence":"additional","affiliation":[{"name":"Institute of Life Sciences, Scuola Superiore Sant\u2019Anna , Piazza Martiri della Libert\u00e0 33, Pisa, Italy"},{"name":"Cardiology Department, Fondazione Toscana Gabriele Monasterio , Via G. Moruzzi 1, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giampaolo","family":"Merlini","sequence":"additional","affiliation":[{"name":"Amyloidosis Research and Treatment Centre, Fondazione IRCCS Policlinico San Matteo , Viale Camillo Golgi 19, Pavia, Italy"},{"name":"Department of Molecular Medicine, University of Pavia , Via C Forlanini 6, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,5,20]]},"reference":[{"key":"2022102609520037500_ehz298-B1","doi-asserted-by":"crossref","first-page":"28932","DOI":"10.1074\/jbc.M115.659912","article-title":"Uncovering the mechanism of aggregation of human transthyretin","volume":"290","author":"Saelices","year":"2015","journal-title":"J Biol Chem"},{"key":"2022102609520037500_ehz298-B2","doi-asserted-by":"crossref","first-page":"2817","DOI":"10.1073\/pnas.0400062101","article-title":"Tissue damage in the amyloidoses: transthyretin monomers and 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