ArticleJACC. Asia2026
Patient Characteristics and Diagnosis of Transthyretin Amyloid Cardiomyopathy in Japan: Baseline Analysis of J-COSSA.
Article in JACC. Asia, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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34 authors.
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Abstract
backgroundLarge-scale, nationwide data for patients with transthyretin amyloid cardiomyopathy (ATTR-CM) in Japan are limited.
objectivesThis multicenter observational, retrospective cohort study (UMIN000056734) assessed the clinical characteristics and diagnostic landscape of patients with ATTR-CM in Japan.
methodsA subcohort of patients with biopsy-proven ATTR-CM initiated on tafamidis from the J-COSSA (Japan Cohort Study of Systemic Amyloidosis) was evaluated (120 sites; April 2020 to January 2024). Baseline characteristics and investigations for the diagnosis of ATTR-CM were recorded.
resultsOverall, 1,506 patients with ATTR-CM (median [range, IQR] age, 78 [48-94, 74-82] years [n = 1,391]; men 88.9% [1,337 of 1,504]) were registered: wild-type transthyretin amyloidosis (ATTRwt), 97.9% (1,475 of 1,506); hereditary (variant) transthyretin amyloidosis (ATTRv), 2.0% (30 of 1,506); New York Heart Association (NYHA) functional class I or II, 86.6% (747 of 863); interventricular septum thickness, 15.3 ± 2.7 mm; left ventricular posterior wall thickness, 14.9 ± 2.8 mm; M protein test availability, 838 of 994 (84.3%; 30 [3.6%] tested positive); and endomyocardial biopsy, 858 (83.4% of 1,029 biopsy records). Among patients with evaluable records, technetium-99m (
conclusionsThis nationwide study clarified the clinical characteristics and diagnostic landscape of patients with biopsy-proven ATTR-CM in Japan. The results indicate that noninvasive testing is implemented for early ATTR-CM diagnosis in clinical practice and reiterate the importance of biomarkers and imaging data for early disease diagnosis.
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