Heart failure & cardiomyopathies
Prevalence, Characteristics and Outcomes of Older Patients with Hereditary Versus Wild-Type Transthyretin Amyloid Cardiomyopathy
Authors' conclusion
Up to 20.7% of elderly patients with ATTR-CM have a pathogenic TTR variant.
Study context
- Population
- Patients over 70 years diagnosed with ATTR-CM at the UK National Amyloidosis Centre, January 2010–August 2022
- Participants
- 2,029 participants
- Exposure
- ATTRv-CM vs ATTRwt-CM
- ATTRv-CMn = 421
- Outcome
- All-cause mortality
- Follow-up
- Median (range) 29 (12-48) months
Methodological detailsconsecutive patients, single-centre
- Consecutive patients
- Single-centre
Main finding
As reported in the abstract
Prevalence of pathogenic TTR variants (ATTRv-CM)
| Group | Value | V122I | T60A | V30M | Other pathogenic TTR variants |
|---|---|---|---|---|---|
| ATTRv-CM | 20.7% | 327 (77.7%) | 47 (11.2%) | 16 (3.8%) | 31 (7.3%) |
ATTRv-CM
Value20.7%
V122I327 (77.7%)
T60A47 (11.2%)
V30M16 (3.8%)
Other pathogenic TTR variants31 (7.3%)
Observational data: associations, not effects.
Further findings
All-cause mortality · median (range) follow-up 29 (12-48) months
- ATTRv-CM associated with increased all-cause mortality vs ATTRwt-CM; poorest survival in V122I-associated ATTRv-CM
- P < 0.001
Factors independently associated with ATTRv-CM (multivariable logistic regression)
- Younger age at diagnosis, OR per year
- 0.85, p < 0.001
- Female sex, OR
- 2.73, p < 0.001
- Afro-Caribbean ethnicity, OR
- 65.5, p < 0.001
- AF, OR
- 0.65, p = 0.015
- Ischaemic heart disease, OR
- 0.54, p = 0.007
- Peripheral polyneuropathy, OR
- 5.70, p < 0.001
- Orthostatic hypotension, OR
- 6.29, p < 0.001
Original publication
Prevalence, Characteristics and Outcomes of Older Patients with Hereditary Versus Wild-Type Transthyretin Amyloid Cardiomyopathy. Eur J Heart Fail. Published 30 January 2023. doi:10.
Summarised from the published abstract; every number is checked against it