Highlights
- •The phenotypic presentation of ATTR-CM is similar in women and men in this single center study
- •Increased rate of diagnosis of ATTR-CM in women is driven by an increase in diagnosis of the wild type form.
- •This increased diagnosis of wtATTR-CM in women is temporally related to the non-invasive diagnosis and tafamidis.
Abstract
Background
Transthyretin amyloid cardiomyopathy (ATTR-CM), particularly wild type (wtATTR-CM),
is thought to mainly affect men. Non-invasive diagnosis and approved therapeutics
have been associated with increased disease recognition. We investigated the trajectory
of ATTR-CM diagnosis in women.
Methods
This observational study utilized data collected on 140 consecutive ATTR-CM patients
diagnosed between 2005 and 2022 who are followed at the Oregon Health and Science
University Amyloidosis Clinic. Subgroup analysis was performed on patients with wtATTR-CM
which included 113 subjects (80.1%). The proportion of women among patients diagnosed
with ATTR-CM prior to 2019 was compared with that of those diagnosed 2019–2022 (2019
was the year of tafamidis approval by the FDA). The clinical characteristics of male
and female ATTR-CM patients were compared as well.
Results
Of the 140 ATTR-CM patients, 16 (11.4%) were women (age 77 ± 9 years) and 124 (88.6%)
were men (age 76 ± 9 years). There was an increase in the rate of women diagnosed
with ATTR-CM from pre 2019 to 2019–2022 in the overall cohort (4/68 [5.9%] vs 12/72
[16.7%]) and wild type subgroup (0/51 [0%] vs 7/62 [11.3%]). There were several differences
in baseline clinical characteristics between women and men in this cohort, yet all
women had a clear clinical phenotype of ATTR-CM.
Conclusions
There has been a significant increase in the rate of wtATTR-CM diagnoses in women,
who presented with clear phenotypes of ATTR-CM. Further studies are needed to understand
the effect of increased recognition of ATTR-CM in women on disease epidemiology, natural
history, and outcomes.
Keywords
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Article info
Publication history
Published online: December 29, 2022
Accepted:
December 28,
2022
Received in revised form:
December 3,
2022
Received:
October 9,
2022
Identification
Copyright
© 2022 Elsevier B.V. All rights reserved.