MedStar Authors catalog › Details for: The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population.
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The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population.

by Horton, Kenneth D.
Citation: Journal of Cardiac Failure. 24(10):672-679, 2018 10..Journal: Journal of cardiac failure.Published: ; 2018ISSN: 1071-9164.Full author list: Plehn JF; Hasbani K; Ernst I; Horton KD; Drinkard BE; Di Prospero NA.UI/PMID: 28986271.Subject(s): Adolescent | Cardiomyopathy, Hypertrophic/di [Diagnosis] | *Cardiomyopathy, Hypertrophic/et [Etiology] | Cardiomyopathy, Hypertrophic/pp [Physiopathology] | Child | Cross-Sectional Studies | Disease Progression | Double-Blind Method | *Echocardiography, Doppler/mt [Methods] | Female | Follow-Up Studies | *Friedreich Ataxia/co [Complications] | *Heart Ventricles/dg [Diagnostic Imaging] | Heart Ventricles/pp [Physiopathology] | Humans | *Magnetic Resonance Imaging, Cine/mt [Methods] | Male | *Ventricular Function, Left/ph [Physiology] | Ventricular RemodelingInstitution(s): MedStar Health Research InstituteActivity type: Journal Article.Medline article type(s): Journal ArticleDigital Object Identifier: https://dx.doi.org/10.1016/j.cardfail.2017.09.012 (Click here) Abbreviated citation: J Card Fail. 24(10):672-679, 2018 10.Local Holdings: Available online from MWHC library: 1995 - present.Abstract: BACKGROUND: Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described.Abstract: METHODS: We performed echocardiography (echo), cardiac magnetic resonance imaging (cMRI) and neurologic assessment in a cross-sectional analysis of 48 genetically-confirmed FA subjects aged 9-17 years with moderate neurologic impairment but without a cardiovascular history. Echo and cMRI-determined left ventricular mass were indexed to height in g/m2.7 (LVMI). LV remodeling was categorized as concentric remodeling (CR), concentric hypertrophy (CH) or eccentric hypertrophy based upon echo-determined relative LV wall thickness.Abstract: RESULTS: Echo LVMI exceeded age-based normal values in 85% of subjects and cMRI-determined LVMI correlated with depression of both diastolic and systolic tissue Doppler velocity (E': r= -0.65, p<0.001, S': r=-0.46, p<0.001) as well as increased early diastolic Doppler flow velocity/tissue velocity ratio (r=0.55, p<0.001), a marker of elevated LV filling pressure. Similar associations were found with echo LV mass. Depressed LV relaxation and increased LV stiffness were observed in 88% and 71% of subjects despite a normal LV ejection fraction in almost all cases (mean = 60 + 7%). CR and CH were present in 40% and 44% of the study group though significant depressions of E' and S' were observed only in subjects with CH (p<0.005).Abstract: CONCLUSIONS: A subclinical hypertrophic cardiomyopathy is common in pediatric FA patients and CH is associated with both diastolic and systolic dysfunction. Copyright (c) 2017. Published by Elsevier Inc.

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