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Beer, M; Weidemann, F; Breunig, F; Knoll, A; Koeppe, S; Machann, W; Hahn, D; Wanner, C; Strotmann, J; Sandstede, J.
Impact of enzyme replacement therapy on cardiac morphology and function and late enhancement in Fabry's cardiomyopathy
Am J Cardiol. 2006; 97(10):1515-1518
Doi: 10.1016/j.amjcard.2005.11.087
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PubMed
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- Leading authors Med Uni Graz
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Beer Meinrad
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- 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.
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