Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries
(2022) In ESC Heart Failure 9(4). p.2528-2537- Abstract
Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive condition caused by deposition of transthyretin amyloid fibrils in the heart and is associated with poor quality of life and a shortened lifespan. This study aimed to describe the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using multiple national health registers in Denmark, Finland, Norway, and Sweden. Methods and results: Transthyretin amyloid cardiomyopathy patients were identified during 2008–2018 using a combination of diagnosis codes for amyloidosis and heart disease and were matched to patients with non-ATTR heart failure (HF). An identical study design was used in each country to facilitate comparison and aggregation of... (More)
Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive condition caused by deposition of transthyretin amyloid fibrils in the heart and is associated with poor quality of life and a shortened lifespan. This study aimed to describe the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using multiple national health registers in Denmark, Finland, Norway, and Sweden. Methods and results: Transthyretin amyloid cardiomyopathy patients were identified during 2008–2018 using a combination of diagnosis codes for amyloidosis and heart disease and were matched to patients with non-ATTR heart failure (HF). An identical study design was used in each country to facilitate comparison and aggregation of results. A total of 1930 ATTR-CM patients were identified from national health registers in the four countries. In 2018, prevalence of ATTR-CM per 100 000 inhabitants ranged from 1.4 in Denmark to 5.0 in Sweden; a steep increase over time was observed in Sweden and Norway. Median survival from diagnosis was 30 months for ATTR-CM patients and 67 months for matched HF patients. Survival was significantly lower for female than for male ATTR-CM patients (median survival: 22 and 36 months), while no significant difference was observed in the HF cohort. Conclusions: This study provides the first nationwide estimates of the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using identical study design across several countries. Findings corroborate previous case series showing high mortality in ATTR-CM, two-fold higher than for other HF patients and higher in women than men, highlighting the need for more precise and early diagnosis to reduce the disease burden.
(Less)
- author
- organization
- publishing date
- 2022-08
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Amyloidosis, ATTR-CM, Heart failure, Mortality, Prevalence, Red flags
- in
- ESC Heart Failure
- volume
- 9
- issue
- 4
- pages
- 10 pages
- publisher
- John Wiley & Sons Inc.
- external identifiers
-
- pmid:35560802
- scopus:85130067469
- ISSN
- 2055-5822
- DOI
- 10.1002/ehf2.13961
- language
- English
- LU publication?
- yes
- additional info
- Publisher Copyright: © 2022 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
- id
- e6049fe3-c13d-4006-93f1-58f4d97f970b
- date added to LUP
- 2022-08-19 15:21:50
- date last changed
- 2024-06-13 10:18:23
@article{e6049fe3-c13d-4006-93f1-58f4d97f970b,
abstract = {{<p>Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is a progressive condition caused by deposition of transthyretin amyloid fibrils in the heart and is associated with poor quality of life and a shortened lifespan. This study aimed to describe the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using multiple national health registers in Denmark, Finland, Norway, and Sweden. Methods and results: Transthyretin amyloid cardiomyopathy patients were identified during 2008–2018 using a combination of diagnosis codes for amyloidosis and heart disease and were matched to patients with non-ATTR heart failure (HF). An identical study design was used in each country to facilitate comparison and aggregation of results. A total of 1930 ATTR-CM patients were identified from national health registers in the four countries. In 2018, prevalence of ATTR-CM per 100 000 inhabitants ranged from 1.4 in Denmark to 5.0 in Sweden; a steep increase over time was observed in Sweden and Norway. Median survival from diagnosis was 30 months for ATTR-CM patients and 67 months for matched HF patients. Survival was significantly lower for female than for male ATTR-CM patients (median survival: 22 and 36 months), while no significant difference was observed in the HF cohort. Conclusions: This study provides the first nationwide estimates of the prevalence, clinical characteristics, and mortality of patients with ATTR-CM, using identical study design across several countries. Findings corroborate previous case series showing high mortality in ATTR-CM, two-fold higher than for other HF patients and higher in women than men, highlighting the need for more precise and early diagnosis to reduce the disease burden.</p>}},
author = {{Lauppe, Rosa and Liseth Hansen, Johan and Fornwall, Anna and Johansson, Katarina and Rozenbaum, Mark H. and Strand, Anne Mette and Väkeväinen, Merja and Kuusisto, Johanna and Gude, Einar and Smith, J. Gustav and Gustafsson, Finn}},
issn = {{2055-5822}},
keywords = {{Amyloidosis; ATTR-CM; Heart failure; Mortality; Prevalence; Red flags}},
language = {{eng}},
number = {{4}},
pages = {{2528--2537}},
publisher = {{John Wiley & Sons Inc.}},
series = {{ESC Heart Failure}},
title = {{Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries}},
url = {{http://dx.doi.org/10.1002/ehf2.13961}},
doi = {{10.1002/ehf2.13961}},
volume = {{9}},
year = {{2022}},
}