Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden
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Nationwide prevalence and characteristics of transthyretin amyloid cardiomyopathy in Sweden. / Lauppe, Rosa Elisabeth; Liseth Hansen, Johan; Gerdesköld, Christian; Rozenbaum, Mark H.; Strand, Anne Mette; Vakevainen, Merja; Kuusisto, Johanna; Gude, Einar; Gustafsson, Finn; Gustav Smith, J.
In: Open Heart, Vol. 8, No. 2, e001755, 2021, p. 1-9.Research output: Contribution to journal › Journal article › Research › peer-review
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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 - Gustav Smith, J.
N1 - 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.
PY - 2021
Y1 - 2021
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.
KW - Cardiomyopathies
KW - Epidemiology
KW - Heart failure
KW - Risk factors
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
AN - SCOPUS:85117798568
VL - 8
SP - 1
EP - 9
JO - Open Heart
JF - Open Heart
SN - 2398-595X
IS - 2
M1 - e001755
ER -
ID: 302817826