ArticleEJHaem2026
Real-World Diagnostic Workup of Patients Suspected for Light Chain Amyloidosis and Wild-Type Transthyretin Amyloid Cardiomyopathy: A Retrospective Cohort Study Using US Electronic Health Records.
Article in EJHaem, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Introduction: Light chain (AL) amyloidosis and transthyretin amyloid cardiomyopathy (ATTR-CM) are the most common types of cardiac amyloidosis. Despite similar manifestations, prognosis and treatments are distinct, emphasizing the importance of accurate and timely diagnosis. This retrospective cohort study assessed real-world diagnostic workups of adult patients suspected of having AL amyloidosis, wild-type ATTR-CM (ATTRwt-CM), or both (AL amyloidosis + ATTRwt-CM). Methods: Data were extracted from a large electronic health record and integrated claims-clinical database (January 2017-June 2023). Workups within 24 months before the first recorded diagnosis were assessed in cohorts with AL amyloidosis (International Classification of Diseases, Tenth Revision code: E85.81 only), ATTRwt-CM (E85.82 only), or AL amyloidosis + ATTRwt-CM (E85.81 and E85.82). Results: Of 1653, 1055, and 59 patients in the AL amyloidosis, ATTRwt-CM, and AL amyloidosis + ATTRwt-CM cohorts, respectively, 53%, 61%, and 66% received any type of AL amyloidosis or ATTRwt-CM test. Across respective cohorts, 42%, 40%, and 49% received an AL amyloidosis workup, and 17%, 15%, and 17% underwent complete monoclonal protein testing (MPT) for AL amyloidosis assessment. In the ATTRwt-CM and AL amyloidosis + ATTRwt-CM cohorts, 50% and 46% received an ATTRwt-CM workup, and 9% and 5% underwent complete MPT before or ≤ 7 days after Conclusions: Notable proportions of patients suspected of having AL amyloidosis, ATTRwt-CM, and AL amyloidosis + ATTRwt-CM did not undergo adequate guideline-recommended diagnostic testing. Due to clinical urgency, improving disease and diagnostic awareness among clinicians is necessary for early, accurate diagnosis and treatment. Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.
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