title: Cross-sectional analysis of electrocardiograms in a large heterogeneous cohort of Friedreich ataxia subjects.
abstract: Electrocardiographic (ECG) findings in Friedreich ataxia and their relation to disease characteristics have not been well described. In this retrospective cross-sectional study, the authors reviewed baseline ECGs from 239 children and adults with Friedreich ataxia. ECG abnormalities--assessed in relation to participant age, sex, shorter guanine-adenine-adenine triplet repeat length, age of disease onset, and functional disability score--were found in 90% of subjects, including nonspecific ST-T wave changes (53%), right axis deviation (32%), left ventricular hypertrophy (19%), and right ventricular hypertrophy (13%). Female sex and shorter guanine-adenine-adenine repeat lengths were associated with a normal ECG (P = .004 and P = .003). Males and those of younger age were more likely to show ventricular hypertrophy (P = .006 and P = .026 for left ventricular hypertrophy and P < .001 and P = .001 for right). Neurologic status as measured by the functional disability score did not predict ECG abnormalities.
authors: Department of Pediatrics, Division of Neurology, Children's Hospital of Philadelphia, PA 19104, USA.
authors: Kimberly A
authors: KA
authors: Schadt
authors: Lisa S
authors: LS
authors: Friedman
authors: Sean R
authors: SR
authors: Regner
authors: George E
authors: GE
authors: Mark
authors: David R
authors: DR
authors: Lynch
authors: Kimberly Y
authors: KY
authors: Lin
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D006332:Cardiomegaly / Q000175:diagnosis / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D004562:Electrocardiography; D005260:Female; D005621:Friedreich Ataxia / Q000150:complications*; D006331:Heart Diseases / Q000175:diagnosis* / Q000209:etiology*; D006801:Humans; D017379:Hypertrophy, Left Ventricular; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D055815:Young Adult