{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,6,18]],"date-time":"2025-06-18T04:11:42Z","timestamp":1750219902764,"version":"3.41.0"},"reference-count":29,"publisher":"Public Library of Science (PLoS)","issue":"6","license":[{"start":{"date-parts":[[2025,6,17]],"date-time":"2025-06-17T00:00:00Z","timestamp":1750118400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS One"],"abstract":"<jats:sec>\n<jats:title>Background<\/jats:title>\n<jats:p>Cardiac amyloidosis (CA) is a potentially fatal systemic disease that has received increasing attention in recent years. However, there is no data on its epidemiology in French Guiana. This study aimed to evaluate the epidemiological characteristics of cardiac amyloidosis and describe the regional diagnostic pathways in French Guiana.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>We performed a multicenter retrospective study of Guianese patients with confirmed or suspected cardiac amyloidosis who were followed up in hospitals in French Guiana by private cardiologists.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>A total of 47 patients were included. The study population was predominantly male (n\u2009=\u200929, 61.7%). The mean age of the population was 72.8 years (SD\u2009=\u200912.2). Most patients were from the island of Cayenne (n\u2009=\u200934, 72.3%), had at least one cardiovascular risk factor (n\u2009=\u200932, 68.1%), and more than half had extracardiac amyloid involvement (n\u2009=\u200925, 53.2%). More than half of the patients were hospitalized at a reference center outside French Guiana (29, 61.7%), mainly at Henri Mondor Hospital (n\u2009=\u200920, 69%) and Martinique (n\u2009=\u20096, 20.7%). Bone scintigraphy was performed in 27 patients (57.5%) and hyperfixation was observed in 25 patients (93%). Anatomopathological examinations were performed in 33 patients (70.2%). Amyloid typing of the biopsied tissue revealed predominantly ATTR (n\u2009=\u200914, 62.6%), AL amyloidosis (n\u2009=\u20091, 4.5%), and AA amyloidosis (4.5%). Among the ATTR amyloidoses, we found mainly ATTRm (n\u2009=\u200922, 75.8%). Genetic mutation testing was performed in approximately half of the patients (n\u2009=\u200925, 51.1%), mostly for the VAL122ILE mutation (n\u2009=\u200921, 84%), and in one case for the IL107VAL mutation (4%). Of the patients with ATTR amyloidosis, 22 (75.9%) were treated with tafamidis. Of the included patients, 18 (38.3%) died. The median overall survival (OS) was 38 months. Survival analysis from the date of diagnosis showed a probability of survival at 30 days, one year, 1.5 years and 4 years of 97% (95% confidence interval [CI]: 90\u2013100), 68% (95%CI 55\u201384)), 64% (95%CI 51\u201379)), 32% (95%CI 29\u201341)), respectively.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusion<\/jats:title>\n<jats:p>This study provides the first information on the diagnostic pathway for cardiac amyloidosis in French Guiana. The increasing proportion of undiagnosed patients has led us to create a French Guianese Amyloidosis Team to simplify the diagnostic pathway by focusing on cardiac MRI and biopsy, which can be performed locally. This is particularly important, as current and future therapeutic advances mean that more effective treatments are on the horizon.<\/jats:p>\n<\/jats:sec>","DOI":"10.1371\/journal.pone.0324955","type":"journal-article","created":{"date-parts":[[2025,6,17]],"date-time":"2025-06-17T17:34:38Z","timestamp":1750181678000},"page":"e0324955","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":0,"title":["A comprehensive evaluation of cardiac amyloidosis epidemiology and diagnostics in French Guiana"],"prefix":"10.1371","volume":"20","author":[{"given":"Baptiste","family":"Desjardins","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K\u00e9nol","family":"Franck","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6202-5762","authenticated-orcid":true,"given":"Nathalie","family":"Deschamps","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean-Franky","family":"Alexis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cyrille","family":"Mathien","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franck","family":"Boteko","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grace \u00e0 Dieu","family":"Yabeta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thibaud","family":"Damy","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jocelyn","family":"Inamo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0624-4180","authenticated-orcid":true,"given":"Narcisse","family":"Elenga","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2025,6,17]]},"reference":[{"issue":"4","key":"pone.0324955.ref001","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1159\/000506696","article-title":"The Pathology of Amyloidosis in Classification: A Review","volume":"143","author":"MM Picken","year":"2020","journal-title":"Acta Haematol"},{"issue":"9","key":"pone.0324955.ref002","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCHEARTFAILURE.119.006075","article-title":"Expert Consensus Recommendations for the Suspicion and Diagnosis of Transthyretin Cardiac Amyloidosis","volume":"12","author":"MS Maurer","year":"2019","journal-title":"Circ Heart Fail"},{"key":"pone.0324955.ref003"},{"issue":"24","key":"pone.0324955.ref004","doi-asserted-by":"crossref","first-page":"2404","DOI":"10.1161\/CIRCULATIONAHA.116.021612","article-title":"Nonbiopsy Diagnosis of Cardiac Transthyretin Amyloidosis","volume":"133","author":"JD Gillmore","year":"2016","journal-title":"Circulation"},{"issue":"1","key":"pone.0324955.ref005","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1016\/j.tcm.2017.07.004","article-title":"Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment","volume":"28","author":"OK Siddiqi","year":"2018","journal-title":"Trends Cardiovasc Med"},{"issue":"1","key":"pone.0324955.ref006","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1186\/s12968-017-0376-0","article-title":"Patterns of CMR measured longitudinal strain and its association with late gadolinium enhancement in patients with cardiac amyloidosis and its mimics","volume":"19","author":"LK Williams","year":"2017","journal-title":"J Cardiovasc Magn Reson"},{"issue":"5","key":"pone.0324955.ref007","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1097\/RLU.0000000000000741","article-title":"Etiology of amyloidosis determines myocardial 99mTc-DPD uptake in amyloidotic cardiomyopathy","volume":"40","author":"S Longhi","year":"2015","journal-title":"Clin Nucl Med"},{"issue":"1","key":"pone.0324955.ref008","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCHEARTFAILURE.120.008193","article-title":"Long-Term Survival With Tafamidis in Patients With Transthyretin Amyloid Cardiomyopathy","volume":"15","author":"P Elliott","year":"2022","journal-title":"Circ Heart Fail"},{"key":"pone.0324955.ref009","doi-asserted-by":"crossref","first-page":"131804","DOI":"10.1016\/j.ijcard.2024.131804","article-title":"Hospitalization-based epidemiology of systemic and cardiac amyloidosis in the Veneto Region, Italy","volume":"400","author":"L De Michieli","year":"2024","journal-title":"Int J Cardiol"},{"issue":"6","key":"pone.0324955.ref010","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCHEARTFAILURE.118.005407","article-title":"Epidemiology of Cardiac Amyloidosis-Associated Heart Failure Hospitalizations Among Fee-for-Service Medicare Beneficiaries in the United States","volume":"12","author":"LG Gilstrap","year":"2019","journal-title":"Circ Heart Fail"},{"issue":"3","key":"pone.0324955.ref011","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1007\/s00392-022-02114-y","article-title":"Epidemiology of cardiac amyloidosis in Germany: a retrospective analysis from 2009 to 2018","volume":"112","author":"S Ney","year":"2023","journal-title":"Clin Res Cardiol"},{"issue":"4","key":"pone.0324955.ref012","doi-asserted-by":"crossref","first-page":"1592","DOI":"10.1007\/s12350-022-03005-5","article-title":"Cardiac amyloidosis in Latin America: Gaps and opportunities to increase awareness of the disease. Findings from the AMILO-LATAM research group","volume":"30","author":"F Mut","year":"2023","journal-title":"J Nucl Cardiol"},{"key":"pone.0324955.ref013","author":"13- French Guiana Identifier","year":"2023"},{"issue":"12","key":"pone.0324955.ref014","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1097\/MNM.0000000000001630","article-title":"Current status of nuclear cardiology practice in Latin America and the Caribbean, in the era of multimodality cardiac imaging approach: 2022 update","volume":"43","author":"A Peix","year":"2022","journal-title":"Nucl Med Commun"},{"issue":"4","key":"pone.0324955.ref015","first-page":"451","article-title":"Clinical characterization of patients with cardiac amyloidosis in a referral center of Colombia","volume":"94","author":"P Ortiz","year":"2024","journal-title":"Arch Cardiol Mex"},{"issue":"1","key":"pone.0324955.ref016","doi-asserted-by":"crossref","DOI":"10.1136\/bmjph-2024-001047","article-title":"Epidemiological perspectives of amyloidosis in Argentina: a cohort study analysing incidence and mortality patterns among a population affiliated to a medical care programme","volume":"3","author":"ML Posadas-Mart\u00ednez","year":"2025","journal-title":"BMJ Public Health"},{"issue":"2","key":"pone.0324955.ref017","first-page":"422","article-title":"Clinical, Laboratory, and Imaging Profile in Patients with Systemic Amyloidosis in a Brazilian Cardiology Referral Center","volume":"118","author":"F Fernandes","year":"2022","journal-title":"Arq Bras Cardiol"},{"issue":"2","key":"pone.0324955.ref018","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/j.jacc.2016.03.596","article-title":"Genotype and Phenotype of Transthyretin Cardiac Amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey)","volume":"68","author":"MS Maurer","year":"2016","journal-title":"J Am Coll Cardiol"},{"issue":"6","key":"pone.0324955.ref019","doi-asserted-by":"crossref","DOI":"10.2174\/011573403X305835240715092532","article-title":"Cardiac Amyloidosis in Venezuela: A Pending Issue","volume":"20","author":"J Salazar","year":"2024","journal-title":"Curr Cardiol Rev"},{"issue":"7","key":"pone.0324955.ref020","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1038\/gim.2016.200","article-title":"Transthyretin V122I (pV142I)* cardiac amyloidosis: an age-dependent autosomal dominant cardiomyopathy too common to be overlooked as a cause of significant heart disease in elderly African Americans","volume":"19","author":"JN Buxbaum","year":"2017","journal-title":"Genet Med"},{"key":"pone.0324955.ref021","doi-asserted-by":"crossref","first-page":"878292","DOI":"10.3389\/fneur.2022.878292","article-title":"Cerebral Ischemic Events: An Overlooked Complication of Transthyretin Cardiac Amyloidosis in Afro-Caribbean Patients","volume":"13","author":"R Banydeen","year":"2022","journal-title":"Front Neurol"},{"issue":"1","key":"pone.0324955.ref022","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1186\/s13023-023-02933-w","article-title":"Incidence and survival of transthyretin amyloid cardiomyopathy from a French nationwide study of in- and out-patient databases","volume":"18","author":"T Damy","year":"2023","journal-title":"Orphanet J Rare Dis"},{"issue":"3","key":"pone.0324955.ref023","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1080\/13506129.2016.1221815","article-title":"Identification of prognostic markers in transthyretin and AL cardiac amyloidosis","volume":"23","author":"T Damy","year":"2016","journal-title":"Amyloid"},{"issue":"2","key":"pone.0324955.ref024","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.113.000098","article-title":"Senile systemic amyloidosis: clinical features at presentation and outcome","volume":"2","author":"JH Pinney","year":"2013","journal-title":"J Am Heart Assoc"},{"issue":"10","key":"pone.0324955.ref025","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0274578","article-title":"Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina","volume":"17","author":"ML Posadas-Martinez","year":"2022","journal-title":"PLoS One"},{"issue":"11","key":"pone.0324955.ref026","doi-asserted-by":"crossref","first-page":"1007","DOI":"10.1056\/NEJMoa1805689","article-title":"Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy","volume":"379","author":"MS Maurer","year":"2018","journal-title":"N Engl J Med"},{"issue":"17","key":"pone.0324955.ref027","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1056\/NEJMoa2300757","article-title":"Patisiran Treatment in Patients with Transthyretin Cardiac Amyloidosis","volume":"389","author":"MS Maurer","year":"2023","journal-title":"N Engl J Med"},{"issue":"1","key":"pone.0324955.ref028","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1056\/NEJMoa2409134","article-title":"Vutrisiran in Patients with Transthyretin Amyloidosis with Cardiomyopathy","volume":"392","author":"M Fontana","year":"2025","journal-title":"N Engl J Med"},{"issue":"11","key":"pone.0324955.ref029","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1038\/s41569-024-01086-y","article-title":"Benefit of vutrisiran in transthyretin amyloidosis with cardiomyopathy","volume":"21","author":"GB Lim","year":"2024","journal-title":"Nat Rev Cardiol"}],"container-title":["PLOS One"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0324955","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,6,17]],"date-time":"2025-06-17T17:34:49Z","timestamp":1750181689000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0324955"}},"subtitle":[],"editor":[{"given":"Gbolahan Deji","family":"Olatunji","sequence":"first","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2025,6,17]]},"references-count":29,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2025,6,17]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0324955","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,6,17]]}}}