{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T12:54:21Z","timestamp":1779195261895,"version":"3.51.4"},"reference-count":30,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circ: Cardiovascular Imaging"],"published-print":{"date-parts":[[2013,9]]},"abstract":"<jats:sec>\n            <jats:title>Background\u2014<\/jats:title>\n            <jats:p>Transthyretin familial amyloid polyneuropathy is a hereditary form of amyloidosis characterized by sensorimotor and autonomic neuropathy, cardiac conduction defects, and infiltrative cardiomyopathy. Previous studies have suggested that myocardial sympathetic denervation assessed by 123-iodine metaiodobenzylguanidine (MIBG) imaging occurs early in disease progression. However, its prognostic significance was never evaluated. We aimed to study the long-term prognostic value of myocardial sympathetic denervation detected by MIBG imaging in transthyretin familial amyloid polyneuropathy.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Methods and Results\u2014<\/jats:title>\n            <jats:p>\n              A total of 143 individuals with V30M transthyretin mutation underwent Holter, ambulatory blood pressure monitoring, echocardiography, and MIBG imaging. Time to all-cause death was compared with late heart-to-mediastinum MIBG uptake ratio (H\/M; either in relation to the estimated lower limit of normal [1.60] or as a continuous variable) using Cox proportional hazards regression. Multivariable analyses were performed to test the prognostic accuracy of clinical, neurological, and cardiovascular parameters. During a median follow-up of 5.5 years, 32 (22%) patients died. Five-year mortality rate was 42% for late H\/M &lt;1.60 and 7% for late H\/M \u22651.60 (hazard ratio, 7.19;\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001). Late H\/M was identified as an independent prognostic predictor. Fifty-three patients were submitted to liver transplantation. In comparison with neurophysiological score\u2013matched controls, transplanted patients had lower long-term mortality (hazard ratio, 0.32;\n              <jats:italic>P<\/jats:italic>\n              =0.012). Patients with late H\/M&lt;1.60 were at higher risk of unfavorable outcome but seemed to have benefited from liver transplantation.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions\u2014<\/jats:title>\n            <jats:p>Cardiac sympathetic denervation as assessed by MIBG imaging is a useful prognostic marker in transthyretin familial amyloid polyneuropathy.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/circimaging.112.000367","type":"journal-article","created":{"date-parts":[[2013,7,6]],"date-time":"2013-07-06T02:18:20Z","timestamp":1373077100000},"page":"627-636","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":95,"title":["Reduced Myocardial 123-Iodine Metaiodobenzylguanidine Uptake"],"prefix":"10.1161","volume":"6","author":[{"given":"Maria C. Azevedo","family":"Coutinho","sequence":"first","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nuno","family":"Cortez-Dias","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guilhermina","family":"Cantinho","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabel","family":"Concei\u00e7\u00e3o","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ant\u00f3nio","family":"Oliveira","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Armando","family":"Bordalo e S\u00e1","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susana","family":"Gon\u00e7alves","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana G.","family":"Almeida","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mamede","family":"de Carvalho","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ant\u00f3nio Nunes","family":"Diogo","sequence":"additional","affiliation":[{"name":"From the Departments of Cardiology (M.C.A.C., N.C.-D., A.B.e.S., S.G., A.G.A., A.N.D.) and Neurosciences (I.C., M.d.C.), Santa Maria University Hospital, Lisbon, Portugal; University Clinic of Cardiology (M.C.A.C., N.C.-D., A.O., A.G.A., A.N.D.), Translational Clinical Physiology Unit, Instituto de Medicina Molecular (M.C.A.C., I.C., M.d.C.), and Institute of Nuclear Medicine (G.C.), Lisbon Medical School, University of Lisbon, Portugal; and Programme for Advanced Medical Education (Funda\u00e7\u00e3o..."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20821"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ahj.2012.04.015"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI111390"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.75.9.4499"},{"key":"e_1_3_3_6_2","first-page":"88","volume-title":"Amyloid and amyloidosis","author":"Coutinho P","year":"1980","unstructured":"Coutinho P, Martins da Silva A, Lopes Lima J. Forty years of experience with type I amyloid neuropathy: review of 438 cases. In: , Glenner GG, Costa PP, de Freitas F, eds. Amyloid and amyloidosis. Amsterdam: Excerpta Medica; 1980:88\u201398."},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0447.1951.tb09673.x"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320270213"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2012.673184"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2011.574354072"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-6143.2007.01969.x"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/00007890-200203150-00015"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.3109\/13506129.2011.574354064"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1136\/pgmj.62.728.601"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.49.2703"},{"key":"e_1_3_3_16_2","first-page":"201","article-title":"Early detection of sympathetic myocardial denervation in patients with familial amyloid polyneuropathy type I.","volume":"23","author":"Coutinho CA","year":"2004","unstructured":"Coutinho CA, Concei\u00e7\u00e3o I, Almeida A, Cantinho G, Sargento L, Vagueiro MC. 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[Familial amyloidotic polyneuropathy \u2013Portuguese type\u2013 clinical and neurophysiologic course]. Acta Med Port. 1993;6:15\u201317.","journal-title":"Acta Med Port"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1994.tb02719.x"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.eurheartj.a014868"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.acvd.2012.03.004"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.hjh.0000163132.84890.c4"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.acvd.2012.03.004"},{"key":"e_1_3_3_28_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.HYP.35.4.892"},{"key":"e_1_3_3_29_2","first-page":"423","article-title":"Familial amyloidotic polyneuropathy in northern Sweden. A cross-sectional and longitudinal study of cardiac function with echocardiography.","volume":"47","author":"Olofsson BO","year":"1988","unstructured":"Olofsson BO, Backman C, Boman K. Familial amyloidotic polyneuropathy in northern Sweden. A cross-sectional and longitudinal study of cardiac function with echocardiography. Arctic Med Res. 1988;47(suppl 1):423\u2013425.","journal-title":"Arctic Med Res"},{"key":"e_1_3_3_30_2","doi-asserted-by":"publisher","DOI":"10.1097\/00007890-200203150-00015"},{"key":"e_1_3_3_31_2","doi-asserted-by":"publisher","DOI":"10.1097\/01.md.0000232559.22098.c3"}],"container-title":["Circulation: Cardiovascular Imaging"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/CIRCIMAGING.112.000367","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,13]],"date-time":"2024-05-13T01:42:04Z","timestamp":1715564524000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/CIRCIMAGING.112.000367"}},"subtitle":["A Prognostic Marker in Familial Amyloid Polyneuropathy"],"short-title":[],"issued":{"date-parts":[[2013,9]]},"references-count":30,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2013,9]]}},"alternative-id":["10.1161\/CIRCIMAGING.112.000367"],"URL":"https:\/\/doi.org\/10.1161\/circimaging.112.000367","relation":{},"ISSN":["1941-9651","1942-0080"],"issn-type":[{"value":"1941-9651","type":"print"},{"value":"1942-0080","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,9]]},"assertion":[{"value":"2012-12-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-07-01","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-07-05","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}