Key result
LV apical aneurysm in Fabry disease with LVH links to ~259% higher major CV risk.
Why the study?
Left ventricular apical aneurysm is an increasingly identified phenotype in Fabry disease, but its clinical prevalence and prognostic implications over long-term follow-up required exploration.
Does the presence of left ventricular apical aneurysm increase the risk of adverse cardiovascular events in patients with Fabry disease?
Cohort (n=266)
No
Does the presence of left ventricular apical aneurysm increase the risk of adverse cardiovascular events in patients with Fabry disease?
Effect estimate: HR 3.59 (95% CI 1.30-9.91)
Absolute Event Rate: 72.7% vs 18.1%
p-value: p=0.01
In patients with Fabry disease and left ventricular hypertrophy, the presence of a left ventricular apical aneurysm is associated with a more than 3-fold increased risk of adverse cardiovascular events.
Identifies higher-risk Fabry patients with LVH for closer monitoring; leaves open whether interventions targeting apical aneurysm improve outcomes.
Background A previously underrecognized phenotype of left ventricular apical aneurysm (LVAA) has been increasingly identified in Fabry disease. This study explored LVAA's clinical prevalence and its prognostic implications over a long‐term follow‐up. Methods and Results We retrospectively analyzed 268 consecutive patients with Fabry disease at a tertiary medical center. Patients with increased left ventricular mass index were recognized as having left ventricular hypertrophy (LVH). LVAA was identified using either echocardiography or cardiovascular magnetic resonance imaging. Two patients with ischemic LVAA were excluded. The primary end point was a composite of cardiovascular events, including heart failure hospitalization, sustained ventricular tachycardia, ischemic stroke, and all‐cause mortality. Of 266 enrolled patients, 105 (39.5%) had LVH (age 58.5±11.9 years, 48.6% men), and 11 (10.5%) had LVAA. Over 49.3±34.8 months of follow‐up, 25 patients with LVH experienced composite events, including 9 heart failure hospitalizations, 4 sustained ventricular tachycardia, 6 ischemic strokes, and 15 mortalities. In patients with LVH, those with LVAA had a significantly higher risk of composite events and lower event‐free survival than those without LVAA (8 [72.7%] versus 17 [18.1%], log‐rank P <0.001). LVAA was independently associated with an increased risk of composite events (hazard ratio, 3.59 [95% CI, 1.30–9.91]; P =0.01) after adjusting for age, sex, advanced heart failure, renal function, dyslipidemia, atrial fibrillation, left ventricular ejection fraction, left ventricular diastolic function, and left ventricular mass index. Conclusions LVAA is present in approximately 10% of patients with Fabry disease and LVH. It is associated with an increased risk of adverse cardiovascular events and may necessitate aggressive treatment.
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Chang et al. (2022) conducted a cohort in Fabry disease (n=266). Left ventricular apical aneurysm (LVAA) vs. Without LVAA was evaluated on Composite of cardiovascular events, including heart failure hospitalization, sustained ventricular tachycardia, ischemic stroke, and all-cause mortality (HR 3.59, 95% CI 1.30-9.91, p=0.01). Left ventricular apical aneurysm in Fabry disease with left ventricular hypertrophy was associated with increased risk of composite cardiovascular events (HR 3.59; 95% CI 1.30–9.91; P=0.01).
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