{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T23:49:49Z","timestamp":1793317789220,"version":"4.5.0"},"reference-count":25,"publisher":"Oxford University Press (OUP)","issue":"14","license":[{"start":{"date-parts":[[2020,1,17]],"date-time":"2020-01-17T00:00:00Z","timestamp":1579219200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"British Heart Foundation Intermediate Clinical Research Fellowship","award":["FS\/18\/21\/33447"],"award-info":[{"award-number":["FS\/18\/21\/33447"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,4,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an increasingly recognized cause of heart failure. We sought to characterize the structural and functional echocardiographic phenotype across the spectrum of wild-type (wtATTR-CM) and hereditary (hATTR-CM) transthyretin cardiomyopathy and the echocardiographic features predicting prognosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>We studied 1240 patients with ATTR-CM who underwent prospective protocolized evaluations comprising full echocardiographic assessment and survival between 2000 and 2019, comprising 766 with wtATTR-CM and 474 with hATTR-CM, of whom 314 had the V122I variant and 127 the T60A variant. At diagnosis, patients with V122I-hATTR-CM had the most severe degree of systolic and diastolic dysfunction across all echocardiographic parameters and patients with T60AhATTR-CM the least; patients with wtATTR-CM had intermediate features. Stroke volume index, right atrial area index, longitudinal strain, and E\/e\u2019 were all independently associated with mortality (P &amp;lt; 0.05 for all). Severe aortic stenosis (AS) was also independently associated with prognosis, conferring a significantly shorter survival (median survival 22 vs. 53 months, P = 0.001).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>The three distinct genotypes present with varying degrees of severity. Echocardiography indicates a complex pathophysiology in which both systolic and diastolic function are independently associated with mortality. The presence of severe AS was independently associated with significantly reduced patient survival.<\/jats:p>\n                    <jats:p\/>\n                  <\/jats:sec>","DOI":"10.1093\/eurheartj\/ehz905","type":"journal-article","created":{"date-parts":[[2019,12,7]],"date-time":"2019-12-07T07:12:32Z","timestamp":1575702752000},"page":"1439-1447","source":"Crossref","is-referenced-by-count":176,"title":["Echocardiographic phenotype and prognosis in transthyretin cardiac amyloidosis"],"prefix":"10.1093","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4524-1436","authenticated-orcid":false,"given":"Liza","family":"Chacko","sequence":"first","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2449-7843","authenticated-orcid":false,"given":"Raffaele","family":"Martone","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"},{"name":"Department of Heart, Lung and Vessels, Tuscan Regional Amyloid Center, Careggi University Hospital , Largo Brambilla 3, Florence 50134, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5821-7371","authenticated-orcid":false,"given":"Francesco","family":"Bandera","sequence":"additional","affiliation":[{"name":"Heart Failure Unit, Cardiology University Department, IRCCS Policlinico San Donato , Piazza Malan, 1, San Donato Milanese, Milan 20097, Italy"},{"name":"Department for Biomedical Sciences for Health, University of Milano, Via Luigi Mangiagalli , 31, Milan 20133, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thirusha","family":"Lane","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3595-6988","authenticated-orcid":false,"given":"Ana","family":"Martinez-Naharro","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Boldrini","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tamer","family":"Rezk","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol","family":"Whelan","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Quarta","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorota","family":"Rowczenio","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janet A","family":"Gilbertson","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8747-0387","authenticated-orcid":false,"given":"Tanakal","family":"Wongwarawipat","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Lachmann","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashutosh","family":"Wechalekar","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sajitha","family":"Sachchithanantham","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shameem","family":"Mahmood","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9549-7176","authenticated-orcid":false,"given":"Rossella","family":"Marcucci","sequence":"additional","affiliation":[{"name":"Department of Experimental and Clinical Medicine, Careggi University Hospital, University of Florence , Largo Brambilla 3, Florence 50134, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Knight","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3466-306X","authenticated-orcid":false,"given":"David","family":"Hutt","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Campus , Rowland Hill Street, London NW3 2PF, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9763-1436","authenticated-orcid":false,"given":"James","family":"Moon","sequence":"additional","affiliation":[{"name":"Barts Heart Centre, The Cardiovascular Magnetic Resonance Imaging Unit, and the Inherited Cardiovascular Diseases Unit, St Bartholomew's Hospital , West Smithfield, London EC1A 7BE, UK"},{"name":"Institute of Cardiovascular Science, University College London , Gower Street, London WC1E 6BT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5236-4741","authenticated-orcid":false,"given":"Aviva","family":"Petrie","sequence":"additional","affiliation":[{"name":"Biostatistics Unit, UCL Eastman Dental Institute, University College London , 256 Grays Inn Road, London WC1X 8LD, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1622-0732","authenticated-orcid":false,"given":"Francesco","family":"Cappelli","sequence":"additional","affiliation":[{"name":"Department of Heart, Lung and Vessels, Tuscan Regional Amyloid Center, Careggi University Hospital , Largo Brambilla 3, Florence 50134, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Guazzi","sequence":"additional","affiliation":[{"name":"Heart Failure Unit, Cardiology University Department, IRCCS Policlinico San Donato , Piazza Malan, 1, San Donato Milanese, Milan 20097, Italy"},{"name":"Department for Biomedical Sciences for Health, University of Milano, Via Luigi Mangiagalli , 31, Milan 20133, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip N","family":"Hawkins","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, Division of Medicine, University College London, Royal 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