Impact of enzyme replacement therapy on cardiac morphology and function and late enhancement in Fabry's cardiomyopathy.
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.
Beer
Meinrad
M
Institut für Röntgendiagnostik, Würzburg, Germany. beer@roentgen.uni-wuerzburg.de
Weidemann
Frank
F
Breunig
Frank
F
Knoll
Anita
A
Koeppe
Sabrina
S
Machann
Wolfram
W
Hahn
Dietbert
D
Wanner
Christoph
C
Strotmann
Jörg
J
Sandstede
Jörn
J