Cardiomyocyte Globotriaosylceramide Accumulation in Adult Male Patients with Fabry Disease and IVS4 + 919G>A GLA Mutation is Progressive with Age and Correlates with Left Ventricular Hypertrophy and Reduced Left Ventricular Ejection Fraction.
Fu-Pang ChangTing-Rong HsuSheng-Che HungShih Hsien SungWen-Chung YuDau-Ming NiuBehzad NajafianPublished in: medRxiv : the preprint server for health sciences (2023)
The unbiased stereological methods introduced in this study unraveled novel relationships between cardiomyocyte structure and important imaging and clinical parameters. These novel tools can help better understand Fabry cardiomyopathy pathophysiology.
Keyphrases
- ejection fraction
- aortic stenosis
- left ventricular
- hypertrophic cardiomyopathy
- heart failure
- replacement therapy
- transcatheter aortic valve replacement
- angiotensin ii
- cardiac resynchronization therapy
- high resolution
- acute myocardial infarction
- multiple sclerosis
- aortic valve
- left atrial
- coronary artery disease
- high glucose
- young adults