Impact of Enzyme Replacement Therapy on Cardiac Morphology and Function and Late Enhancement in Fabry’s Cardiomyopathy

• Published in: The American journal of cardiology Vol. 97; no. 10; pp. 1515 - 1518
• Main Authors: Beer, Meinrad, Weidemann, Frank, Breunig, Frank, Knoll, Anita, Koeppe, Sabrina, Machann, Wolfram, Hahn, Dietbert, Wanner, Christoph, Strotmann, Jörg, Sandstede, Jörn
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 15.05.2006; Elsevier; Elsevier Limited
• Subjects: Adult; alpha-Galactosidase - therapeutic use; Biological and medical sciences; Cardiology; Cardiology. Vascular system; Cardiovascular disease; Contrast Media; Drug therapy; Echocardiography, Doppler; Enzymes; Fabry Disease - diagnostic imaging; Fabry Disease - drug therapy; Fabry Disease - physiopathology; Female; Gadolinium DTPA; Genetic disorders; Heart; Humans; Magnetic Resonance Imaging; Male; Medical sciences; Myocarditis. Cardiomyopathies; Statistics, Nonparametric; Treatment Outcome; Cardiac performance; Late; Replacement therapy; Cardiomyopathy; Enzyme; Heart disease; Morphology; Cardiovascular disease; Circulatory system; Cardiology; Myocardial disease; Phlebology
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2005.11.087

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.