TY  - BOOK
AU  - Horton, Kenneth D
TI  - The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population
SN  - 1071-9164
PY  - 2018///
KW  - *Cardiomyopathy, Hypertrophic/et [Etiology]
KW  - *Echocardiography, Doppler/mt [Methods]
KW  - *Friedreich Ataxia/co [Complications]
KW  - *Heart Ventricles/dg [Diagnostic Imaging]
KW  - *Magnetic Resonance Imaging, Cine/mt [Methods]
KW  - *Ventricular Function, Left/ph [Physiology]
KW  - Adolescent
KW  - Cardiomyopathy, Hypertrophic/di [Diagnosis]
KW  - Cardiomyopathy, Hypertrophic/pp [Physiopathology]
KW  - Child
KW  - Cross-Sectional Studies
KW  - Disease Progression
KW  - Double-Blind Method
KW  - Female
KW  - Follow-Up Studies
KW  - Heart Ventricles/pp [Physiopathology]
KW  - Humans
KW  - Male
KW  - Ventricular Remodeling
KW  - MedStar Health Research Institute
KW  - Journal Article
N1  - Available online from MWHC library: 1995 - present
N2  - 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)
UR  - https://dx.doi.org/10.1016/j.cardfail.2017.09.012
ER  -