{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T19:31:29Z","timestamp":1793043089916,"version":"4.5.0"},"reference-count":20,"publisher":"BMJ","issue":"1","license":[{"start":{"date-parts":[[2025,4,4]],"date-time":"2025-04-04T00:00:00Z","timestamp":1743724800000},"content-version":"unspecified","delay-in-days":93,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Alexion, AstraZeneca Rare Disease","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2025,3,12]]},"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Patients with light chain (AL) amyloidosis, a rare life-threatening disease, often go through a lengthy diagnostic journey. We qualitatively and quantitatively characterised the diagnostic pathway and the impact of delayed diagnosis on health outcomes among patients with AL amyloidosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We conducted a retrospective cohort analysis of adults (age \u226518 years) with \u22652 AL amyloidosis diagnoses (index date: first diagnosis date) using the IQVIA PharMetrics Plus US claims data from 1 January 2016 to 31 December 2022. We stratified patients based on time from first cardiovascular (CV) manifestation onset to diagnosis. Patients were categorised as having a delayed diagnosis if the first CV manifestation occurred &gt;1 year prior to diagnosis, and patients were categorised as without a delayed diagnosis if the first CV manifestation occurred &lt;1 year prior to diagnosis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Our study included 470 patients (mean age 61.8 years, 60% males). In the 24 months before diagnosis, CV manifestations occurred in 86% of patients and renal manifestations in 74%. Patients most frequently visited on average four different cardiologists. Patients were most frequently diagnosed by haematologists\/oncologists (49.8%). Patients with a delayed diagnosis (179\/470, 38.1%) were twice as likely as those without a delayed diagnosis to have CV-related emergency room visits (adjusted OR: 1.98; 95% CI: 1.21 to 3.24; p&lt;0.010). Patients with a delayed diagnosis were one and a half times more likely than those without a delayed diagnosis to have CV-related inpatient hospitalisations (adjusted OR: 1.65; 95%\u2009CI: 1.1 to 2.46; p=0.020).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>This claims database study suggests that patients with delayed diagnosis experienced more CV-related emergency room visits and inpatient hospitalisations, underscoring the need for timely diagnosis of AL amyloidosis and the role of cardiologists in the diagnostic pathway.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/openhrt-2024-003124","type":"journal-article","created":{"date-parts":[[2025,4,4]],"date-time":"2025-04-04T09:57:26Z","timestamp":1743760646000},"page":"e003124","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["Diagnostic pathways, cardiac manifestations and outcomes in light chain amyloidosis: analysis of a US claims database"],"prefix":"10.1136","volume":"12","author":[{"given":"Genevieve","family":"Lyons","sequence":"first","affiliation":[{"name":"Global Health Economics and Outcomes Research, Alexion Pharmaceuticals Inc, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey","family":"Thompson","sequence":"additional","affiliation":[{"name":"Global Health Economics and Outcomes Research, Alexion Pharmaceuticals Inc, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Lousada","sequence":"additional","affiliation":[{"name":"Amyloidosis Research Consortium Inc, Newton, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Catini","sequence":"additional","affiliation":[{"name":"Global Medical Affairs, Alexion Pharmaceuticals Inc, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richa","family":"Manwani","sequence":"additional","affiliation":[{"name":"Clinical Development, Alexion Pharmaceuticals Inc, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mathew S","family":"Maurer","sequence":"additional","affiliation":[{"name":"Cardiac Amyloidosis Program, Centre for Advanced Cardiac Care, New York-Presbyterian\/Columbia University Irving Medical Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,4,4]]},"reference":[{"key":"2025081611590173000_12.1.e003124.1","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/j.jaccao.2022.08.009","article-title":"AL Amyloidosis for Cardiologists: Awareness, Diagnosis, and Future Prospects: JACC: CardioOncology State-of-the-Art Review","volume":"4","author":"Wechalekar","year":"2022","journal-title":"JACC CardioOncol"},{"key":"2025081611590173000_12.1.e003124.2","doi-asserted-by":"crossref","DOI":"10.1038\/s41408-021-00486-4","article-title":"Comprehensive Review of AL amyloidosis: some practical recommendations","volume":"11","author":"Al Hamed","year":"2021","journal-title":"Blood Cancer J"},{"key":"2025081611590173000_12.1.e003124.3","doi-asserted-by":"crossref","first-page":"388","DOI":"10.1159\/000508287","article-title":"Prognosis and Staging of AL Amyloidosis","volume":"143","author":"Dittrich","year":"2020","journal-title":"Acta Haematol"},{"key":"2025081611590173000_12.1.e003124.4","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1007\/s40271-017-0273-5","article-title":"Light Chain (AL) Amyloidosis: The Journey to Diagnosis","volume":"11","author":"McCausland","year":"2018","journal-title":"Patient"},{"key":"2025081611590173000_12.1.e003124.5","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2022.1008449","article-title":"Understanding AL amyloidosis with a little help from in vivo models","volume":"13","author":"Martinez-Rivas","year":"2022","journal-title":"Front Immunol"},{"key":"2025081611590173000_12.1.e003124.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.blre.2023.101040"},{"key":"2025081611590173000_12.1.e003124.7","doi-asserted-by":"crossref","DOI":"10.3390\/cells10030545","article-title":"Daratumumab in the Treatment of Light-Chain (AL) Amyloidosis","volume":"10","author":"Palladini","year":"2021","journal-title":"Cells"},{"key":"2025081611590173000_12.1.e003124.8","doi-asserted-by":"crossref","first-page":"83","DOI":"10.6004\/jnccn.2022.7077","article-title":"Immunoglobulin Light Chain Amyloidosis: Diagnosis and Risk Assessment","volume":"21","author":"Zanwar","year":"2023","journal-title":"J Natl Compr Canc Netw"},{"key":"2025081611590173000_12.1.e003124.9","doi-asserted-by":"crossref","DOI":"10.1097\/HS9.0000000000000454","article-title":"Systemic AL Amyloidosis: Current Approaches to Diagnosis and Management","volume":"4","author":"Fotiou","year":"2020","journal-title":"Hemasphere"},{"key":"2025081611590173000_12.1.e003124.10","doi-asserted-by":"publisher","DOI":"10.1007\/s12325-015-0250-0"},{"key":"2025081611590173000_12.1.e003124.11","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2020006913"},{"key":"2025081611590173000_12.1.e003124.12","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1080\/13506129.2022.2093635","article-title":"Guidelines for non-transplant chemotherapy for treatment of systemic AL amyloidosis: EHA-ISA working group","volume":"30","author":"Wechalekar","year":"2023","journal-title":"Amyloid"},{"key":"2025081611590173000_12.1.e003124.13","doi-asserted-by":"crossref","first-page":"103435","DOI":"10.1016\/j.critrevonc.2021.103435","article-title":"Progress in research: Daratumumab improves treatment outcomes of patients with AL amyloidosis","volume":"165","author":"Hassan","year":"2021","journal-title":"Crit Rev Oncol Hematol"},{"key":"2025081611590173000_12.1.e003124.14","doi-asserted-by":"crossref","first-page":"98","DOI":"10.3390\/hemato3010008","article-title":"Monitoring Patients with Light Chain (AL) Amyloidosis during and after Therapy: Response Assessment and Identification of Relapse","volume":"3","author":"Milani","year":"2022","journal-title":"Hemato"},{"key":"2025081611590173000_12.1.e003124.15","unstructured":"IQVIA . 2022. Available: https:\/\/www.iqvia.com\/locations\/united-states\/library\/fact-sheets\/iqvia-pharmetrics-plus [Accessed 08 Mar 2024]."},{"key":"2025081611590173000_12.1.e003124.16","doi-asserted-by":"crossref","first-page":"1740","DOI":"10.1002\/ehf2.13851","article-title":"Clinical features and predictors of atrial fibrillation in patients with light-chain or transthyretin cardiac amyloidosis","volume":"9","author":"Papathanasiou","year":"2022","journal-title":"ESC Heart Fail"},{"key":"2025081611590173000_12.1.e003124.17","doi-asserted-by":"crossref","DOI":"10.2196\/44420","article-title":"Patient Journey Toward a Diagnosis of Light Chain Amyloidosis in a National Sample: Cross-Sectional Web-Based Study","volume":"7","author":"Dou","year":"2023","journal-title":"JMIR Form Res"},{"key":"2025081611590173000_12.1.e003124.18","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1111\/ejh.13472","article-title":"Patient outcomes in light chain (AL) amyloidosis: The clock is ticking from symptoms to diagnosis","volume":"105","author":"Schulman","year":"2020","journal-title":"Eur J Haematol"},{"key":"2025081611590173000_12.1.e003124.19","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1007\/s41669-019-0122-7","article-title":"Associations between Health-Related Quality of Life and Self-Reported Emergency Room Department Visits and Inpatient Hospitalizations: Insights from a Secondary Data Analysis of Patients with Light-Chain (AL) Amyloidosis","volume":"3","author":"McCausland","year":"2019","journal-title":"Pharmacoecon Open"},{"key":"2025081611590173000_12.1.e003124.20","doi-asserted-by":"crossref","first-page":"549","DOI":"10.2217\/cer-2017-0100","article-title":"Healthcare resource utilization and costs in amyloid light-chain amyloidosis: a real-world study using US claims data","volume":"7","author":"Quock","year":"2018","journal-title":"J Comp Eff Res"}],"container-title":["Open Heart"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/openhrt-2024-003124","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,16]],"date-time":"2025-08-16T18:59:09Z","timestamp":1755370749000},"score":1,"resource":{"primary":{"URL":"https:\/\/openheart.bmj.com\/lookup\/doi\/10.1136\/openhrt-2024-003124"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":20,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,4,4]]},"published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1136\/openhrt-2024-003124"],"URL":"https:\/\/doi.org\/10.1136\/openhrt-2024-003124","relation":{},"ISSN":["2053-3624"],"issn-type":[{"value":"2053-3624","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1]]}}}