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Cardiac evaluation in children with Prader-Willi syndrome.
MedLine Citation:
PMID:  22181352     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Aim:  To assess cardiac anatomy and myocardial systolic function in children with PWS. Methods:  Physical examination, electrocardiographic (ECG) recordings and transthoracic echocardiograms including two-dimensional speckle tracking echocardiography (2DSTE), were performed and evaluated in the Radboud University Hospital Nijmegen, the Netherlands. In total 19 children diagnosed with PWS and 38 age-matched control subjects underwent cardiac evaluation. Results:  Abnormal ECG findings were detected in nine PWS patients. Echocardiography revealed mild structural cardiac abnormalities in two patients. Conventional echocardiographic findings did not indicate systolic left ventricular dysfunction, in contrast to 2DSTE examination. Global peak systolic strain (-rate) measurements, in all three directions of contraction, were significantly lower in children with PWS (P<0.001) compared with healthy age-matched children. In two-thirds of the patients, 2DSTE revealed abnormal systolic deformation (peak systolic strain as well as strain rate). T2P values in PWS patients were similar to control subject. Systolic myocardial function appears more affected in case of maternal uniparental disomy. Conclusion:  Cardiac evaluation, including 2DSTE, detects frequent alterations in myocardial systolic function in children diagnosed with PWS, whose conventional echocardiographic findings did not indicate ventricular systolic dysfunction. Since cardiovascular morbidity and mortality is substantial in PWS, especially adults, we emphasize the need for cardiac assessment in PWS.
Authors:
Karen A Marcus; Janiëlle A A E M van Alfen-van der Velden; Barto J Otten; Gert Weijers; Helger G Yntema; Chris L de Korte; Livia Kapusta
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-12-19
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992)     Volume:  -     ISSN:  1651-2227     ISO Abbreviation:  -     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2011-12-20     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9205968     Medline TA:  Acta Paediatr     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Acta Paediatrica © 2011 Foundation Acta Paediatrica.
Affiliation:
Children's Heart Centre, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands Department of Pediatric Endocrinology and Metabolism, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands Clinical Physics Laboratory, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands Pediatric Cardiology Unit, Edith Wolfson Medical Center, Holon, Israel.
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