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. 2022 Sep;11(3):393-405.
doi: 10.1007/s40119-022-00265-7. Epub 2022 May 18.

Sex Differences in Wild-Type Transthyretin Amyloidosis: An Analysis from the Transthyretin Amyloidosis Outcomes Survey (THAOS)

Courtney M Campbell  1   2 , Samantha LoRusso  3 , Angela Dispenzieri  4 , Arnt V Kristen  5 , Mathew S Maurer  6 , Claudio Rapezzi  7   8 , Olivier Lairez  9 , Brian Drachman  10 , Pablo Garcia-Pavia  11   12 , Martha Grogan  13 , Doug Chapman  14 , Leslie Amass  14 ; THAOS investigators
Collaborators, Affiliations

Sex Differences in Wild-Type Transthyretin Amyloidosis: An Analysis from the Transthyretin Amyloidosis Outcomes Survey (THAOS)

Courtney M Campbell et al. Cardiol Ther. 2022 Sep.

Abstract

Introduction: Wild-type transthyretin amyloidosis (ATTRwt amyloidosis) is a progressive disease resulting from the accumulation of wild-type transthyretin (TTR) amyloid fibrils, and is diagnosed primarily in males. This analysis examined sex differences in patients with ATTRwt amyloidosis from the Transthyretin Amyloidosis Outcomes Survey (THAOS).

Methods: THAOS is an ongoing, global, longitudinal, observational survey of patients with transthyretin amyloidosis, including both inherited and wild-type disease, and asymptomatic carriers of TTR mutations. THAOS data were analyzed to identify potential differences in demographic and clinical characteristics between males and females with ATTRwt amyloidosis (data cutoff: August 1, 2021).

Results: Of 1386 patients with ATTRwt amyloidosis, 84 (6%) were female and 1302 (94%) were male. Females had a higher median age at enrollment (80 vs. 78 years; p = 0.002) and symptom onset (75 vs. 73 years; p = 0.045) than males. Mean left ventricular (LV) ejection fraction was higher (53% vs. 48%; p = 0.001) and mean LV diastolic diameter lower (42 vs. 46 mm; p < 0.001) in females versus males, but sex was not identified as a predictor of LV mean wall thickness adjusted for height (beta coefficient - 0.22; p = 0.460) or a predominantly cardiac phenotype (odds ratio 1.60; p = 0.191). Modified polyneuropathy disability scores differed between groups (p < 0.001), with a larger proportion of scores ≥ IIIa among females (23% vs. 7%).

Conclusions: Females with ATTRwt amyloidosis in THAOS tended to present at a later age and showed signs of less severe cardiac impairment and more severe walking impairment.

Trial registration: ClinicalTrials.gov: NCT00628745.

Keywords: ATTRwt amyloidosis; Registry; Sex; Transthyretin Amyloidosis Outcomes Survey.

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Figures

Fig. 1
Fig. 1
Cumulative incidence of ATTR-CM according to sex. The cumulative incidence curve showed that female patients tended to develop ATTR-CM at a later age. ATTR amyloidosis transthyretin amyloidosis; ATTR-CM transthyretin amyloid cardiomyopathy
See this image and copyright information in PMC

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