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Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden

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Lauppe, RE, Liseth Hansen, J, Gerdesköld, C, Rozenbaum, MH, Strand, AM, Vakevainen, M, Kuusisto, J, Gude, E, Gustafsson, F & Smith, JG 2021, 'Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden', Open Heart, vol. 8, no. 2, e001755, pp. e001755. https://doi.org/10.1136/openhrt-2021-001755

APA

Lauppe, R. E., Liseth Hansen, J., Gerdesköld, C., Rozenbaum, M. H., Strand, A. M., Vakevainen, M., Kuusisto, J., Gude, E., Gustafsson, F., & Smith, J. G. (2021). Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden. Open Heart, 8(2), e001755. [e001755]. https://doi.org/10.1136/openhrt-2021-001755

CBE

Lauppe RE, Liseth Hansen J, Gerdesköld C, Rozenbaum MH, Strand AM, Vakevainen M, Kuusisto J, Gude E, Gustafsson F, Smith JG. 2021. Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden. Open Heart. 8(2):e001755. https://doi.org/10.1136/openhrt-2021-001755

MLA

Vancouver

Lauppe RE, Liseth Hansen J, Gerdesköld C, Rozenbaum MH, Strand AM, Vakevainen M et al. Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden. Open Heart. 2021 Oct;8(2):e001755. e001755. https://doi.org/10.1136/openhrt-2021-001755

Author

Lauppe, Rosa Elisabeth ; Liseth Hansen, Johan ; Gerdesköld, Christian ; Rozenbaum, Mark H ; Strand, Anne Mette ; Vakevainen, Merja ; Kuusisto, Johanna ; Gude, Einar ; Gustafsson, Finn ; Smith, J Gustav. / Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden. In: Open Heart. 2021 ; Vol. 8, No. 2. pp. e001755.

Bibtex

@article{a0f31c8ec24b4dcd89cafebe3094b90f,
title = "Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden",
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 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.",
author = "Lauppe, {Rosa Elisabeth} and {Liseth Hansen}, Johan and Christian Gerdesk{\"o}ld and Rozenbaum, {Mark H} and Strand, {Anne Mette} and Merja Vakevainen and Johanna Kuusisto and Einar Gude and Finn Gustafsson and Smith, {J Gustav}",
note = "{\textcopyright} 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.",
year = "2021",
month = oct,
doi = "10.1136/openhrt-2021-001755",
language = "English",
volume = "8",
pages = "e001755",
journal = "Open Heart",
issn = "2053-3624",
publisher = "BMJ Publishing Group",
number = "2",

}

RIS

TY - JOUR

T1 - Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden

AU - Lauppe, Rosa Elisabeth

AU - Liseth Hansen, Johan

AU - Gerdesköld, Christian

AU - Rozenbaum, Mark H

AU - Strand, Anne Mette

AU - Vakevainen, Merja

AU - Kuusisto, Johanna

AU - Gude, Einar

AU - Gustafsson, Finn

AU - Smith, J Gustav

N1 - © 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.

PY - 2021/10

Y1 - 2021/10

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85117798568&partnerID=8YFLogxK

U2 - 10.1136/openhrt-2021-001755

DO - 10.1136/openhrt-2021-001755

M3 - Journal article

C2 - 34645699

VL - 8

SP - e001755

JO - Open Heart

JF - Open Heart

SN - 2053-3624

IS - 2

M1 - e001755

ER -

ID: 68253841