Structural and functional cardiac analyses using modern and sensitive myocardial techniques in adult Pompe disease

The International Journal of Cardiovascular Imaging
Daniel A MorrisWilhelm Haverkamp

Abstract

The purpose of this study was to analyze comprehensively the heart using modern and sensitive myocardial techniques in order to determine if structural or functional cardiac alterations are present in adult Pompe disease. Twelve patients with adult Pompe disease and a control group of 187 healthy subjects of similar age and gender were included. Structural and functional cardiac characteristics were analyzed by conventional and 2D speckle-tracking echocardiography. In addition, in a subgroup of adult Pompe patients, we analyzed the myocardial and musculoskeletal features by means of cardiac and whole-body muscle magnetic resonance imaging. Patients with Pompe disease had significant structural and functional musculoskeletal alterations such as atrophy with fatty replacement and weakness in trunk and extremities. In contrast, Pompe patients had similar structural and functional myocardial features to healthy subjects (LV strain -20.7 ± 1.9 vs. -21.3 ± 2.1%; RV strain -24.2 ± 5.3 vs. -24.8 ± 3.8%; LA strain 41.5 ± 10.3 vs. 44.8 ± 11.0%; P > 0.05; and no evidence of LV and RV hypertrophy or LA enlargement). In addition, there was no evidence of valvular cardiac alterations, electrocardiographic abnormalities, or myocardial fibrosi...Continue Reading

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Citations

Jan 21, 2016·Circulation. Cardiovascular Genetics·Stephan C A WensW W M Pim Pijnappel
Jul 3, 2020·ESC Heart Failure·Athanasios FrydasElisabeth Pieske-Kraigher
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Jan 3, 2021·Neuromuscular Disorders : NMD·H A van KootenP A van Doorn

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