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. 2022 Aug;9(4):2528-2537.
doi: 10.1002/ehf2.13961. Epub 2022 May 12.

Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

Rosa Lauppe  1 , Johan Liseth Hansen  1   2 , Anna Fornwall  1 , Katarina Johansson  3 , Mark H Rozenbaum  4 , Anne Mette Strand  5 , Merja Väkeväinen  6 , Johanna Kuusisto  7 , Einar Gude  8 , J Gustav Smith  9   10 , Finn Gustafsson  11
Affiliations

Prevalence, characteristics, and mortality of patients with transthyretin amyloid cardiomyopathy in the Nordic countries

Rosa Lauppe et al. ESC Heart Fail. 2022 Aug.

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 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.

Keywords: ATTR-CM; Amyloidosis; Heart failure; Mortality; Prevalence; Red flags.

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Conflict of interest statement

Rosa Lauppe, Johan Liseth Hansen, and Anna Fornwall are employed by Quantify Research and funded by Pfizer to conduct this study; Quantify Research is a consultancy and works with a range of different pharmaceutical companies. Katarina Johansson, Mark H. Rozenbaum, Anne Mette Strand, and Merja Vakevainen are Pfizer employees and hold Pfizer stock and/or stock options. Johanna Kuusisto received support from Pfizer for her collaboration in this manuscript as well as grants or contracts from Sanofi‐Genzyme, Pfizer, and The Finnish Foundation for Cardiovascular Research. J.K. also received consulting fees and honoraria for lectures, presentations, speaker's bureaus, manuscript writing, or educational events from Sanofi‐Genzyme, Pfizer, Bayer, Takeda, Amgen, and Chiesi as well as payment for expert testimony from Sanofi‐Genzyme, Pfizer, Bayer, Takeda, and Amgen. J.K. has received support for participation on a Data Safety Monitoring Board or Advisory Board from Sanofi‐Genzyme, Pfizer, Bayer, Takeda, Amgen, and Chiesi and is supported by Amgen for Leadership or fiduciary role in other board, society, committee, or advocacy group. Einar Gude has received grants and honoraria for lectures from Pfizer. Finn Gustafsson has received support from Pfizer for work on the present manuscript and consulting fees from Pfizer, Alnylam, and Ionis. J Gustav Smith has no conflict of interests.

Figures

Figure 1
Figure 1
ATTR‐CM patient identification.
Figure 2
Figure 2
Prevalence by country and year.
Figure 3
Figure 3
Mortality of ATTR‐CM patients in Denmark, Finland, Norway and Sweden.
Figure 4
Figure 4
Aggregated mortality comparing ATTR‐CM cohort and matched HF cohort.
Figure 5
Figure 5
Aggregated mortality by gender for ATTR‐CM cohort and matched HF cohort.
See this image and copyright information in PMC

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