The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population.

MedStar author(s):
Citation: Journal of Cardiac Failure. 24(10):672-679, 2018 10.PMID: 28986271Institution: MedStar Health Research InstituteForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *Cardiomyopathy, Hypertrophic/et [Etiology] | *Echocardiography, Doppler/mt [Methods] | *Friedreich Ataxia/co [Complications] | *Heart Ventricles/dg [Diagnostic Imaging] | *Magnetic Resonance Imaging, Cine/mt [Methods] | *Ventricular Function, Left/ph [Physiology] | Adolescent | Cardiomyopathy, Hypertrophic/di [Diagnosis] | Cardiomyopathy, Hypertrophic/pp [Physiopathology] | Child | Cross-Sectional Studies | Disease Progression | Double-Blind Method | Female | Follow-Up Studies | Heart Ventricles/pp [Physiopathology] | Humans | Male | Ventricular RemodelingYear: 2018Local holdings: Available online from MWHC library: 1995 - presentISSN:
  • 1071-9164
Name of journal: Journal of cardiac failureAbstract: BACKGROUND: Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described.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.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.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).All authors: Di Prospero NA, Drinkard BE, Ernst I, Hasbani K, Horton KD, Plehn JFFiscal year: FY2019Fiscal year of original publication: FY2018Digital Object Identifier: Date added to catalog: 2017-10-17
Holdings
Item type Current library Collection Call number Status Date due Barcode
Journal Article MedStar Authors Catalog Article 28986271 Available 28986271

Available online from MWHC library: 1995 - present

BACKGROUND: Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described.

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.

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.

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).

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