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Risk factors, stroke rates and aspirin prescribing trends in the Canadian Fabry disease initiative cohort
Emilie T Théberge1, Caroline Selvage2, Anita Thomas1
1University of British Columbia, 2329 West Mall, Vancouver, BC, Canada.
Insights
Fabry disease patients experience high stroke and TIA rates, even with low traditional risk factors. Aspirin (acetylsalicylic acid, ASA) use for primary prevention has decreased, despite its potential role in managing cardiovascular disease risks in this population.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Neurology
Background:
- Fabry disease (FD) is an X-linked genetic disorder caused by GLA variants, leading to premature cardiovascular disease (CVD) mortality.
- Recent clinical trials on aspirin (acetylsalicylic acid, ASA) have influenced primary prevention guidelines, creating uncertainty for FD patients.
- FD patients exhibit a higher incidence of early-onset, small vessel stroke compared to the general population.
Purpose of the Study:
- To evaluate the incidence of stroke and transient ischemic attack (TIA) in the Canadian Fabry Disease Initiative (CFDI) cohort.
- To assess the impact of updated aspirin (ASA) guidelines on primary prevention strategies for cardiovascular disease (CVD) in FD patients.
- To compare stroke/TIA rates in FD patients with the general Canadian population and analyze risk factors.
Main Methods:
- Retrospective analysis of longitudinal data from 641 patients in the CFDI (2007-2023).
- Comparison of incident stroke/TIA rates, other CVD events, and ASA/antiplatelet (ASA/AP) prescription patterns before and after 2018.
- Stratification of stroke/TIA rates by sex and GLA variant severity, and calculation of 10-year atherosclerotic CVD (ASCVD) risk.
Main Results:
- A high incidence of stroke/TIA was observed in the CFDI cohort, particularly in male patients and those with severe GLA variants.
- No patients under 60 with incident stroke/TIA had a high calculated 10-year ASCVD risk, indicating low traditional vascular risk.
- Prescription rates for ASA/AP for primary prevention decreased after 2018.
Conclusions:
- FD patients face a significant risk of stroke/TIA, often occurring at a younger age and independent of traditional vascular risk factors.
- The decline in primary prevention aspirin (ASA) use warrants further investigation regarding its impact on cardiovascular outcomes in Fabry disease.
- Current cardiovascular disease (CVD) risk assessment models may not fully capture the cerebrovascular risk in FD patients.
Background:
Fabry disease (FD) is an X-linked disorder caused by deleterious variants in GLA. Cardiovascular disease (CVD) causes premature mortality in FD. Hope for aspirin (acetylsalicylic acid, ASA) to reduce CVD risks in FD as primary prevention may have been tempered by the 2018 ARRIVE, ASCEND, and ASPREE clinical trials. It is unclear how new ASA guidance applies to FD patients, who have a high rate of young-onset, small vessel stroke compared with the general population.
Methods:
Longitudinal data spanning 2007-2023 from patients in the Canadian Fabry Disease Initiative (CFDI) were analyzed retrospectively. Incident stroke and transient ischemic attack (TIA), other CVD events, FD-specific risk factors, and ASA/antiplatelet ("ASA/AP") prescription before and after 2018 were compared between groups who never had an event ("primary prevention group") to those who had incident stroke/TIA during the study. Stroke/TIA rates were compared within CFDI by sex and GLA variant severity, and in the CFDI compared to Canadian statistics by sex. Ten-year atherosclerotic CVD (ASCVD) risk was calculated using the 2013 ACC/AHA risk calculator. ASA/AP prescription rate was compared before and after 2018.
Results:
Out of 641 patients, 57 had an incident stroke/TIA during the study, and 193 with complete data remained in the primary prevention group. Stroke/TIA rates were significantly higher among male patients (0.026 events per patient-year) than females (0.0098 events per patient-year), and higher among patients with severe GLA variants (males: 0.031 events per patient-year, females: 0.0096 events per patient-year) compared to those with attenuated variants (males: 0.011 events per patient-year, females: 0.0088 events per patient-year). No patients under 60 years at their incident stroke/TIA had high (≥ 10%) calculated 10-year ASCVD risk. Fewer patients were prescribed ASA/AP for primary prevention after 2018.
Conclusions:
There was a high incidence of stroke/TIA in the younger CFDI cohort compared to the general Canadian population, despite low levels of traditional vascular risk factors as represented in 10-year estimated ASCVD risk. Primary prevention use of ASA has declined.
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