Left Ventricular Geometry, Global Function, and Dyssynchrony in Infants and Children With Pompe Cardiomyopathy Undergoing Enzyme Replacement Therapy

Publication year: 2011 Source: Journal of Cardiac Failure, Available online 9 September 2011 Chun-An Chen, Yin-Hsiu Chien, Wuh-Liang Hwu, Ni-Chung Lee, Jou-Kou Wang, … BackgroundEnzyme replacement therapy (ERT) for infantile-onset Pompe disease effectively reduces the left ventricular (LV) mass. This study sought to explore detailed process of LV reverse remodeling after ERT with the use of tissue Doppler and stain rate imaging.Methods and ResultsNine infants and children with Pompe cardiomyopathy undergoing ERT for ≥1 year, as well as 36 healthy control subjects, were studied.

Publication year: 2011 Source: Journal of Cardiac Failure, Available online 9 September 2011 Chun-An Chen, Yin-Hsiu Chien, Wuh-Liang Hwu, Ni-Chung Lee, Jou-Kou Wang, … BackgroundEnzyme replacement therapy (ERT) for infantile-onset Pompe disease effectively reduces the left ventricular (LV) mass. This study sought to explore detailed process of LV reverse remodeling after ERT with the use of tissue Doppler and stain rate imaging.Methods and ResultsNine infants and children with Pompe cardiomyopathy undergoing ERT for ≥1 year, as well as 36 healthy control subjects, were studied.

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Left Ventricular Geometry, Global Function, and Dyssynchrony in Infants and Children With Pompe Cardiomyopathy Undergoing Enzyme Replacement Therapy