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Longitudinal strain in Friedreich Ataxia: a potential marker for early left ventricular dysfunction.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	St John Sutton, Martin Ky, Bonnie Regner, Sean R. Schadt, Kim Plappert, Ted He, Jiwei D'Souza, Benjamin Lynch, David R.
Description	Author Affiliation: St John Sutton M ( Department of Cardiology, University of Pennsylvania Medical School, Philadelphia, Pennsylvania)
Abstract	BACKGROUND: Friedreich's ataxia (FRDA) is a neurodegenerative disorder resulting from deficiency of frataxin, characterized by cardiac hypertrophy associated with heart failure and sudden cardiac death. However, the relationship between remodeling and novel measures of cardiac function such as strain, and the time-dependent changes in these measures are poorly defined. METHODS AND RESULTS: We compared echocardiographic parameters of cardiac size, hypertrophy, and function in 50 FRDA patients with 50 normal controls and quantified the following measures of cardiac remodeling and function: left ventricular (LV) volumes, mass, relative wall thickness (RWT), ejection fraction (EF), and myocardial strain. Linear regression analysis was used to identify significant differences in echocardiographic parameters in FRDA compared with normal subjects. In analyses adjusted for age, sex, and body surface area, significant differences were observed between parameters of remodeling (LV mass, RWT, and volumes) and function in FRDA patients compared with controls. In particular, longitudinal strain was significantly decreased in FRDA patients compared with controls (-12.4% vs. -16.0%, P < 0.001), despite similar and normal left ventricular ejection fraction (LVEF). Over 3 years of follow-up, there was no change in strain, LV size, LV mass, or LVEF among FRDA patients. CONCLUSION: Longitudinal strain is reduced in FRDA despite normal LVEF, indicative of subclinical cardiac dysfunction. Given late declines in LVEF in FRDA, longitudinal strain may provide an earlier index of myocardial dysfunction in FRDA.
File Format	HTM / HTML
ISSN	07422822
Issue Number	1
Volume Number	31
e-ISSN	15408175
Journal	Echocardiography
Language	English
Publisher	Wiley
Publisher Date	2014-01-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Research Support, Non-u.s. Gov't Ventricular Dysfunction, Left Humans Middle Aged Stress, Mechanical Male Elastic Modulus Journal Article Feasibility Studies Young Adult Etiology Discipline Cardiology Adult Female Echocardiography Sensitivity And Specificity Physiopathology Reproducibility Of Results Anisotropy Adolescent Aged Friedreich Ataxia Early Diagnosis Methods Elasticity Imaging Techniques
Content Type	Text
Resource Type	Article
Subject	Radiology, Nuclear Medicine and Imaging Cardiology and Cardiovascular Medicine

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