Publication at First Faculty of Medicine, Third Faculty of Medicine |
2010
Abstract
Left ventricular hypertrophy is the hallmark of cardiac involvement in Fabry disease. However the pathophysiology of Fabry disease-related cardiomyopathy is still poorly understood.In our study we have demonstrated, based on magnetic resosonance spectroscopy and repeated echocardiographic examinations, a positive correlation between cardiac energy metabolism status at the time of diagnosis of Fabry disease and gain of left ventricular mass during several years of follow-up.
Impaired cardiac energy metabolism, ether primary or secondary, probably plays important role in the pathogenesis of left ventricular hypertrophy in Fabry disease.