Circulation. Genomic and precision medicine · 2026
Cardiovascular Morbidity and Mortality in Fabry Disease.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Monda Emanuele, Bakalakos Athanasios, Del Franco Annamaria, Lillo Rosa, Meucci Maria Chiara, Spinelli Letizia, Parisi Vanda, Schiavo Maria Alessandra, Rubino Marta, Graziani Francesca, Cappelli Francesco, Pieroni Maurizio, Pisani Antonio, Iaccarino Guido, Lachmann Robin, Murphy Elaine, Ramaswami Uma, Hughes Derralynn, Biagini Elena, Limongelli Giuseppe, Elliott Perry Mark
Аннотация
BACKGROUND: Cardiac involvement is the main determinant of adverse outcomes in Fabry disease. The study aimed to investigate cardiovascular outcomes in patients with Fabry disease.
METHODS: This was a multicenter, retrospective, longitudinal study of consecutively referred adult patients with Fabry disease. The primary end point was the occurrence of major adverse cardiovascular events defined as a composite of cardiovascular death, major arrhythmic events, bradyarrhythmias requiring pacemaker implantation, and stroke.
RESULTS: A total of 680 patients (age, 42.3±15.9 years; 41.0% male; 68.7% on disease-specific therapy) were included. During a median follow-up of 7.1 (interquartile range, 3.9-11.6) years, 92 patients (13.5%) experienced a major adverse cardiovascular event. At 10 years, freedom from major adverse cardiovascular event was 85.1% (95% CI, 81.3-88.2) and was lower in men compared with women (76.1% [95% CI, 68.9-81.9] versus 91.3% [95% CI, 87.0-94.2]; log-rank χ2=26.9; P<0.001). On multivariable analysis, age (hazard ratio, 1.04 [95% CI, 1.01-1.06] per 1 year; P<0.001), estimated glomerular filtration rate (hazard ratio, 0.99 [95% CI, 0.98-0.99] per 1 mL/min per 1.73 m2; P<0.001), QRS interval (hazard ratio, 1.02 [95% CI, 1.01-1.03] per 1 ms; P=0.002), and left ventricular mass index (hazard ratio, 1.01 [95% CI, 1.00-1.01] per 1 g/m2; P=0.032) were independent predictors of major adverse cardiovascular events during follow-up.
CONCLUSIONS: This study shows that the prevention and treatment of cardiovascular disease remain an unmet need for patients with Fabry disease.
METHODS: This was a multicenter, retrospective, longitudinal study of consecutively referred adult patients with Fabry disease. The primary end point was the occurrence of major adverse cardiovascular events defined as a composite of cardiovascular death, major arrhythmic events, bradyarrhythmias requiring pacemaker implantation, and stroke.
RESULTS: A total of 680 patients (age, 42.3±15.9 years; 41.0% male; 68.7% on disease-specific therapy) were included. During a median follow-up of 7.1 (interquartile range, 3.9-11.6) years, 92 patients (13.5%) experienced a major adverse cardiovascular event. At 10 years, freedom from major adverse cardiovascular event was 85.1% (95% CI, 81.3-88.2) and was lower in men compared with women (76.1% [95% CI, 68.9-81.9] versus 91.3% [95% CI, 87.0-94.2]; log-rank χ2=26.9; P<0.001). On multivariable analysis, age (hazard ratio, 1.04 [95% CI, 1.01-1.06] per 1 year; P<0.001), estimated glomerular filtration rate (hazard ratio, 0.99 [95% CI, 0.98-0.99] per 1 mL/min per 1.73 m2; P<0.001), QRS interval (hazard ratio, 1.02 [95% CI, 1.01-1.03] per 1 ms; P=0.002), and left ventricular mass index (hazard ratio, 1.01 [95% CI, 1.00-1.01] per 1 g/m2; P=0.032) were independent predictors of major adverse cardiovascular events during follow-up.
CONCLUSIONS: This study shows that the prevention and treatment of cardiovascular disease remain an unmet need for patients with Fabry disease.