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Published in: Orphanet Journal of Rare Diseases 1/2022

Open Access 01-12-2022 | Cardiomyopathy | Research

Clinical manifestation, economic burden, and mortality in patients with transthyretin cardiac amyloidosis

Authors: Suk-Chan Jang, Jin Hyun Nam, Seung-Ah Lee, Dasom An, Hye-Lin Kim, Sun-Hong Kwon, Eui-Kyung Lee

Published in: Orphanet Journal of Rare Diseases | Issue 1/2022

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Abstract

Background

Transthyretin cardiac amyloidosis, also known as transthyretin cardiomyopathy (ATTR-CM) is a poorly-recognized disease with delayed diagnosis and poor prognosis. This nationwide population-based study aimed to identify disease manifestations, economic burden, and mortality of patients with ATTR-CM.

Methods

Data of newly diagnosed patients with ATTR-CM between 2013 and 2018 from the Korean National Health Insurance Service were used, covering the entire population. Patient characteristics included comorbidities, medical procedures, and medication. Healthcare resource utilization and medical costs were observed as measures of the economic burden. The Kaplan–Meier survival curve and years of potential life lost (YPLL) from the general population were estimated for disease burden with ATTR CM.

Results

A total of 175 newly diagnosed patients with ATTR-CM were identified. The most common cardiac manifestation was hypertension (51.3%), while the most common non-cardiac manifestation was musculoskeletal disease (68.0%). Mean medical costs at the post-cohort entry date were significantly higher than those at the pre-cohort entry date ($1,864 vs. $400 per patient per month (PPPM), p < 0.001). Of the total medical costs during the study period, the proportion of inpatients cost was 12.9 times higher than the outpatients cost ($1,730 and $134 PPPM, respectively). The median survival time was 3.53 years from the first diagnosis of ATTR-CM, and the mean (SD) YPLL was 13.0 (7.7).

Conclusions

Patients with ATTR-CM had short survival and high medical costs. To reduce the clinical and economic burdens, carefully examining manifestations of disease in patients can help with early diagnosis and treatment.
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Metadata
Title
Clinical manifestation, economic burden, and mortality in patients with transthyretin cardiac amyloidosis
Authors
Suk-Chan Jang
Jin Hyun Nam
Seung-Ah Lee
Dasom An
Hye-Lin Kim
Sun-Hong Kwon
Eui-Kyung Lee
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2022
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-022-02425-3

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