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논문 기본 정보

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학술저널
저자정보
Kim Darae (Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.) Youn Jong-Chan (Division of Cardiology, Department of Internal Medicine, Seoul St. Mary’s Hospital, Catholic Research Institute for Intractable Cardiovascular Disease, College of Medicine, The Catholic Univ) Lee Hye Won (Department of Cardiology, Medical Research Institute, Pusan National University Hospital, Pusan National University College of Medicine, Busan, Korea.) Oh Jaewon (Division of Cardiology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.) Son Jung-Woo (Division of Cardiology, Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, Korea.) Cho Hyun-Jai (Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.) Lee Seul (Medical Affairs, Pfizer Pharmaceuticals Korea Ltd., Seoul, Korea.) Shah Nishant R. (Division of Cardiology, Department of Medicine, Warren Alpert Medical School of Brown University, Providence, RI, USA.) Kittleson Michelle M. (Department of Cardiology, Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA.) Jeon Eun-Seok (Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.)
저널정보
대한의학회 Journal of Korean Medical Science Journal of Korean Medical Science Vol.39 No.19
발행연도
2024.5
수록면
1 - 12 (12page)
DOI
10.3346/jkms.2024.39.e163

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초록· 키워드

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Background: Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is an under-recognized cause of heart failure (HF) with clinical phenotypes that vary across regions and genotypes. We sought to characterize the clinical characteristics of ATTR-CM in Asia. Methods: Data from a nationwide cohort of patients with ATTR-CM from six major tertiary centres in South Korea were analysed between 2010 and 2021. All patients underwent clinical evaluation, biochemical laboratory tests, echocardiography, and transthyretin (TTR) genotyping at the time of diagnosis. The study population comprised 105 Asian ATTR-CM patients (mean age: 69 years; male: 65.7%, wild-type ATTR-CM: 41.9%). Results: Among our cohort, 18% of the patients had a mean left ventricular (LV) wall thickness < 12 mm. The diagnosis of ATTR-CM increased notably during the study period (8 [7.6%] during 2010–2013 vs. 22 [21.0%] during 2014–2017 vs. 75 [71.4%] during 2018–2021). Although the duration between symptom onset and diagnosis did not differ, the proportion of patients with HF presenting mild symptoms increased during the study period (25% NYHA class I/II between 2010–2013 to 77% between 2018–2021). In contrast to other international registry data, male predominance was less prominent in wild-type ATTR-CM (68.2%). The distribution of TTR variants was also different from Western countries and from Japan. Asp38Ala was the most common mutation. Conclusion: A nationwide cohort of ATTR-CM exhibited less male predominance, a proportion of patients without increased LV wall thickness, and distinct characteristics of genetic mutations, compared to cohorts in other parts of the world. Our results highlight the ethnic variation in ATTR-CM and may contribute to improving the screening process for ATTR-CM in the Asian population.
#Cardiac Amyloidosis #Diagnosis #Nationwide Registry #Transthyretin

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