Document Detail


Echocardiographic and electrocardiographic analyses of patients with severe motor and intellectual disabilities.
MedLine Citation:
PMID:  19165569     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Severe motor and intellectual disabilities (SMID) syndrome is a heterogeneous group of disorders with severe physical disabilities and mental retardation. Higher incidence of sudden death is also known in these patients. However, little is known about the cardiovascular features of patients with SMID. We examine the patients with severe motor and intellectual disabilities using echocardiogram and clarify their characterization of ventricular function. We performed electrocardiographic and echocardiographic analyses in SMID patients. In all patients, two-dimensional echocardiography with tissue Doppler analysis in the pulsed Doppler mode was performed. Of 121 patients, 104 patients had abnormal findings: 81 had poor R-wave progression, and 15 patients had low-voltage QRS on ECG. These findings strongly correlated with the degree of physical disability. However, on echocardiography, most patients had LVEF in the normal range, while LV Tei indices were significantly higher (0.43 vs 0.31 cm/s) and left ventricular end-diastolic dimension significantly smaller than healthy controls (P<0.05 for each comparison). Patients had significantly decreased early diastolic tissue Doppler velocities at the lateral mitral (5.3 vs 6.7 cm/s), tricuspid (6.7 vs 9.2 cm/s), and septal (5.9 vs 8.8 cm/s) annuli compared with controls (P<0.05 for each comparison). We show for the first time that SMID patients have low E/Ea ratios on tissue Doppler imaging while LV contractions are normal, suggesting the existence of latent diastolic dysfunction. This may be one of the reasons why the incidence of sudden death is higher in SMID patients.
Authors:
Keiichi Hirono; Tohru Konishi; Hideki Origasa; Fukiko Ichida; Toshio Miyawaki
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Publication Detail:
Type:  Comparative Study; Journal Article     Date:  2009-01-23
Journal Detail:
Title:  Heart and vessels     Volume:  24     ISSN:  1615-2573     ISO Abbreviation:  Heart Vessels     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2009-01-23     Completed Date:  2009-06-09     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8511258     Medline TA:  Heart Vessels     Country:  Japan    
Other Details:
Languages:  eng     Pagination:  46-53     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Faculty of Medicine, University of Toyama, 2630 Sugitani, 930-0194 Toyama, Japan. khirono-circ@umin.net
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Child
Child, Preschool
Disability Evaluation*
Echocardiography, Doppler / methods*
Electrocardiography / methods*
Female
Follow-Up Studies
Humans
Infant
Male
Mental Retardation / complications,  rehabilitation*
Middle Aged
Myotonia / complications,  rehabilitation*
Prognosis
Retrospective Studies
Stroke Volume / physiology
Ventricular Dysfunction / complications,  diagnosis*,  rehabilitation
Young Adult

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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