Estimating the Gender Distribution of Patients with Wild-Type Transthyretin Amyloid Cardiomyopathy: A Systematic Review
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REVIEW
Estimating the Gender Distribution of Patients with Wild-Type Transthyretin Amyloid Cardiomyopathy: A Systematic Review and MetaAnalysis Florint Kroi . Nils Fischer . Ana Gezin . Mahmoud Hashim . Mark Hermannes Rozenbaum
Received: October 8, 2020 Ó The Author(s) 2020
ABSTRACT Introduction: This study investigates the gender distribution in patients diagnosed with wild-type transthyretin amyloidosis cardiomyopathy (ATTRwt). Methods: A systematic review and meta-analysis of the male proportion in diagnosed ATTRwt patients were conducted. To avoid overlapping population, pooled estimates in the primary analysis were based on all unique studies. In secondary analyses, we considered predefined subsets of studies based on study sample size, recruitment years, geography, study design, age at diagnosis, and method of diagnosis. Additional meta-regression analyses were tested for potential determinants of gender distribution.
Electronic supplementary material The online version of this article (https://doi.org/10.1007/s40119020-00205-3) contains supplementary material, which is available to authorized users. F. Kroi A. Gezin M. Hashim Ingress-Health, Rotterdam, The Netherlands N. Fischer Pfizer, NewYork, USA e-mail: [email protected] M. H. Rozenbaum (&) Pfizer, Rotterdam, The Netherlands e-mail: [email protected]
Results: Twenty-eight unique studies (2542 patients) were included in the meta-analysis. Male proportion in patients with ATTRwt was 86.9% (95% confidence interval 81.5–91.6%). Studies, including patients older than 80 years at diagnosis, had a 29.1% (p value \ 0.001) lower male proportion compared to studies, including younger patients. After adjusting for age, studies using autopsy as a method of diagnosis had a 21.1% (p value 0.002) lower male proportion compared to other studies. Conclusions: Studies conducted to date suggest ATTRwt disproportionally affects males. The proportion of males was significantly impacted by the age at diagnosis and method diagnosis, which may suggest important gender-based differences in the clinical manifestation and diagnostic challenges of ATTRwt in females that warrant future research. Keywords: Meta-analysis; Systematic review; Wild-type transthyretin amyloidosis cardiomyopathy
Cardiol Ther
Key Summary Points This is the first meta-analysis of male proportion in patients with ATTRwt. Studies conducted to date suggest that ATTRwt disproportionately affects male patients. In the primary analysis, the male proportion was 86.9% (95% CI: 81.5–91.6%). The mean age at diagnosis is a determinant of the gender distribution.
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INTRODUCTION Transthyretin amyloidosis cardiomyopathy (ATTR-CM) is a rare, progressively debilitating, fatal disease [1]. ATTR-CM is caused by instability
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