Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden
(2021) In Open Heart 8(2).- Abstract
Objective Transthyretin amyloid cardiomyopathy (ATTR-CM) is a rare, progressive and fatal condition caused by deposition of transthyretin amyloid fibrils in the heart. This study aims to identify all patients diagnosed with ATTR-CM in Sweden, estimate the prevalence of ATTR-CM, describe patient characteristics and mortality, assess the importance of early symptoms (red flags) for identification of ATTR-CM, and compare with patients with heart failure (HF). Methods This retrospective study combined multiple national health registers covering all specialist visits and prescriptions for the entire population of Sweden. Between January 2008 and December 2018, patients with ATTR-CM were identified retrospectively based on a combination of... (More)
Objective Transthyretin amyloid cardiomyopathy (ATTR-CM) is a rare, progressive and fatal condition caused by deposition of transthyretin amyloid fibrils in the heart. This study aims to identify all patients diagnosed with ATTR-CM in Sweden, estimate the prevalence of ATTR-CM, describe patient characteristics and mortality, assess the importance of early symptoms (red flags) for identification of ATTR-CM, and compare with patients with heart failure (HF). Methods This retrospective study combined multiple national health registers covering all specialist visits and prescriptions for the entire population of Sweden. Between January 2008 and December 2018, patients with ATTR-CM were identified retrospectively based on a combination of diagnosis codes and compared with matched, all-cause non-ATTR HF patients. Results Overall, a total of 994 patients diagnosed with ATTR-CM were identified, with an average age at diagnosis of 73 years, and 30% of whom were female. The prevalence of diagnosed ATTR-CM cases in 2018 was 5.0 per 100 000. The median survival from diagnosis was 37.6 months (CI 33.8 to 43.8), with a lower median survival in women (27.9 months, CI 23.3 to 33.8) compared with men (43.5 months, CI 37.6 to 49.6). Patients with ATTR-CM demonstrated reduced survival compared with patients with HF (p<0.001). Compared with patients with HF, clinical identification of carpal tunnel syndrome, spinal stenosis, and atrioventricular and left bundle branch block can facilitate earlier diagnosis of ATTR-CM. Conclusions This study provides the first nationwide estimates of ATTR-CM prevalence and risk factors. The results reinforce the severity of the disease and the importance of earlier diagnosis, especially for female patients, in order to allow effective treatment and prevention of disease progression.
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- author
- Lauppe, Rosa Elisabeth ; Liseth Hansen, Johan ; Gerdesköld, Christian ; Rozenbaum, Mark H. ; Strand, Anne Mette ; Vakevainen, Merja ; Kuusisto, Johanna ; Gude, Einar ; Gustafsson, Finn and Gustav Smith, J.
- publishing date
- 2021-10-13
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Cardiomyopathies, Epidemiology, Heart failure, Risk factors
- in
- Open Heart
- volume
- 8
- issue
- 2
- article number
- e001755
- publisher
- BMJ Publishing Group
- external identifiers
-
- scopus:85117798568
- pmid:34645699
- ISSN
- 2398-595X
- DOI
- 10.1136/openhrt-2021-001755
- language
- English
- LU publication?
- no
- additional info
- Publisher Copyright: © Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
- id
- 8011a45f-22b5-4c98-bd91-0c8f53d96daa
- date added to LUP
- 2021-11-23 15:25:18
- date last changed
- 2024-06-15 21:20:06
@article{8011a45f-22b5-4c98-bd91-0c8f53d96daa,
abstract = {{<p>Objective Transthyretin amyloid cardiomyopathy (ATTR-CM) is a rare, progressive and fatal condition caused by deposition of transthyretin amyloid fibrils in the heart. This study aims to identify all patients diagnosed with ATTR-CM in Sweden, estimate the prevalence of ATTR-CM, describe patient characteristics and mortality, assess the importance of early symptoms (red flags) for identification of ATTR-CM, and compare with patients with heart failure (HF). Methods This retrospective study combined multiple national health registers covering all specialist visits and prescriptions for the entire population of Sweden. Between January 2008 and December 2018, patients with ATTR-CM were identified retrospectively based on a combination of diagnosis codes and compared with matched, all-cause non-ATTR HF patients. Results Overall, a total of 994 patients diagnosed with ATTR-CM were identified, with an average age at diagnosis of 73 years, and 30% of whom were female. The prevalence of diagnosed ATTR-CM cases in 2018 was 5.0 per 100 000. The median survival from diagnosis was 37.6 months (CI 33.8 to 43.8), with a lower median survival in women (27.9 months, CI 23.3 to 33.8) compared with men (43.5 months, CI 37.6 to 49.6). Patients with ATTR-CM demonstrated reduced survival compared with patients with HF (p<0.001). Compared with patients with HF, clinical identification of carpal tunnel syndrome, spinal stenosis, and atrioventricular and left bundle branch block can facilitate earlier diagnosis of ATTR-CM. Conclusions This study provides the first nationwide estimates of ATTR-CM prevalence and risk factors. The results reinforce the severity of the disease and the importance of earlier diagnosis, especially for female patients, in order to allow effective treatment and prevention of disease progression.</p>}},
author = {{Lauppe, Rosa Elisabeth and Liseth Hansen, Johan and Gerdesköld, Christian and Rozenbaum, Mark H. and Strand, Anne Mette and Vakevainen, Merja and Kuusisto, Johanna and Gude, Einar and Gustafsson, Finn and Gustav Smith, J.}},
issn = {{2398-595X}},
keywords = {{Cardiomyopathies; Epidemiology; Heart failure; Risk factors}},
language = {{eng}},
month = {{10}},
number = {{2}},
publisher = {{BMJ Publishing Group}},
series = {{Open Heart}},
title = {{Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden}},
url = {{http://dx.doi.org/10.1136/openhrt-2021-001755}},
doi = {{10.1136/openhrt-2021-001755}},
volume = {{8}},
year = {{2021}},
}