{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T08:39:54Z","timestamp":1789634394912,"version":"build-2803163510"},"reference-count":39,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2013,3,12]]},"abstract":"\n Background<\/jats:title>\n \n Cardiac amyloidosis is a fatal disease whose prognosis and treatment rely on identification of the amyloid type. In our aging population transthyretin amyloidosis (\n ATTR<\/jats:styled-content>\n wt) is common and must be differentiated from other amyloid types. We report the clinical presentation, natural history, and prognostic features of\n ATTR<\/jats:styled-content>\n wt compared with cardiac\u2010isolated\n AL<\/jats:styled-content>\n amyloidosis and calculate the probability of disease diagnosis of\n ATTR<\/jats:styled-content>\n wt from baseline factors.\n <\/jats:p>\n <\/jats:sec>\n \n Methods and Results<\/jats:title>\n \n All patients with biopsy\u2010proven\n ATTR<\/jats:styled-content>\n wt (102 cases) and isolated cardiac\n AL<\/jats:styled-content>\n (36 cases) seen from 2002 to 2011 at the\n UK<\/jats:styled-content>\n National Amyloidosis Center were included. Median survival from the onset of symptoms was 6.07\u00a0years in the\n ATTR<\/jats:styled-content>\n wt group and 1.7\u00a0years in the\n AL<\/jats:styled-content>\n group. Positive troponin, a pacemaker, and increasing New York Heart Association (NYHA) class were associated with worse survival in\n ATTR<\/jats:styled-content>\n wt patients on univariate analysis. All patients with isolated cardiac\n AL<\/jats:styled-content>\n and 24.1% of patients with\n ATTR<\/jats:styled-content>\n wt had evidence of a plasma cell dyscrasia. Older age and lower\n N<\/jats:styled-content>\n \u2010terminal pro\u2010\n B<\/jats:styled-content>\n \u2010type natriuretic peptide (\n NT<\/jats:styled-content>\n pro\u2010\n BNP<\/jats:styled-content>\n ) were factors significantly associated with\n ATTR<\/jats:styled-content>\n wt. Patients aged 70\u00a0years and younger with an\n NT<\/jats:styled-content>\n pro\u2010\n BNP<\/jats:styled-content>\n <183\u00a0pmol\/L were more likely to have\n ATTR<\/jats:styled-content>\n wt, as were patients older than 70\u00a0years with an\n NT<\/jats:styled-content>\n pro\u2010\n BNP<\/jats:styled-content>\n <1420\u00a0pmol\/L.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n \n Factors at baseline associated with a worse outcome in\n ATTR<\/jats:styled-content>\n wt are positive troponin T, a pacemaker, and\n NYHA<\/jats:styled-content>\n class\n IV<\/jats:styled-content>\n symptoms. The age of the patient at diagnosis and\n NT<\/jats:styled-content>\n pro\u2010\n BNP<\/jats:styled-content>\n level can aid in distinguishing\n ATTR<\/jats:styled-content>\n wt from\n AL<\/jats:styled-content>\n amyloidosis.\n <\/jats:p>\n <\/jats:sec>","DOI":"10.1161\/jaha.113.000098","type":"journal-article","created":{"date-parts":[[2013,5,24]],"date-time":"2013-05-24T16:49:31Z","timestamp":1369414171000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":309,"title":["Senile Systemic Amyloidosis: Clinical Features at Presentation and Outcome"],"prefix":"10.1161","volume":"2","author":[{"given":"Jennifer H.","family":"Pinney","sequence":"first","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"},{"name":"UCL Centre for Nephrology, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol J.","family":"Whelan","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aviva","family":"Petrie","sequence":"additional","affiliation":[{"name":"Biostatistics Unit, UCL Eastman Dental Institute, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jason","family":"Dungu","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"},{"name":"St George's Hospital University of London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanjay M.","family":"Banypersad","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"},{"name":"The Heart Hospital UCLH United Kingdom, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prayman","family":"Sattianayagam","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashutosh","family":"Wechalekar","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon D. J.","family":"Gibbs","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher P.","family":"Venner","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nancy","family":"Wassef","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carolyn A.","family":"McCarthy","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janet A.","family":"Gilbertson","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorota","family":"Rowczenio","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip N.","family":"Hawkins","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian D.","family":"Gillmore","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"},{"name":"UCL Centre for Nephrology, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen J.","family":"Lachmann","sequence":"additional","affiliation":[{"name":"National Amyloidosis Centre, UCL Medical School, Royal Free Hospital, London, UK"},{"name":"UCL Centre for Nephrology, UCL Medical School, Royal Free Hospital, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_4_2_2","first-page":"45","article-title":"Primary systemic amyloidosis: clinical and laboratory features in 474 cases","volume":"32","author":"Kyle RA","year":"1995","unstructured":"Kyle RA, Gertz MA. 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