Fabry disease: development and progression of left ventricular hypertrophy despite long-term enzyme replacement therapy.
Niccolò MauriziAlbina NowakChristiane GrunerMehdi NamdarChristian SchmiedAlessandra Pia PorrettaGuillaume BarbeyVeronique MonzambaniPierre MonneyFrédéric BarbeyPublished in: Heart (British Cardiac Society) (2024)
Over a median follow-up of >10 years under ERT, 36% of the patients still had LVMI cardiac progression, and 32%, predominantly older men, experienced major AFD-related events. LVH at treatment initiation was a strong predictor of LVMI progression and adverse events on ERT.
Keyphrases
- replacement therapy
- left ventricular
- end stage renal disease
- smoking cessation
- ejection fraction
- newly diagnosed
- chronic kidney disease
- hypertrophic cardiomyopathy
- heart failure
- middle aged
- prognostic factors
- aortic stenosis
- physical activity
- mitral valve
- coronary artery disease
- percutaneous coronary intervention
- acute coronary syndrome
- cardiac resynchronization therapy
- african american
- combination therapy
- community dwelling
- transcatheter aortic valve replacement