Author/Authors :
Beer، نويسنده , , Meinrad and Weidemann، نويسنده , , Frank and Breunig، نويسنده , , Frank and Knoll، نويسنده , , Anita and Koeppe، نويسنده , , Sabrina and Machann، نويسنده , , Wolfram and Hahn، نويسنده , , Dietbert and Wanner، نويسنده , , Christoph and Strotmann، نويسنده , , Jِrg and Sandstede، نويسنده , , Jِrn، نويسنده ,
Abstract :
The present study evaluated the evolution of cardiac morphology, function, and late enhancement as a noninvasive marker of myocardial fibrosis, and their inter-relation during enzyme replacement therapy in patients with Fabry’s disease using magnetic resonance imaging and color Doppler myocardial imaging. Late enhancement, which was present in up to 50% of patients, was associated with increased left ventricular mass, the failure of a significant regression of hypertrophy during enzyme replacement therapy, and worse segmental myocardial function. Late enhancement may predict the effect of enzyme replacement therapy on left ventricular mass and cardiac function.
