title: Early detection of myocardial dysfunction in children with mitochondrial disease: An ultrasound and two-dimensional strain echocardiography study.
abstract: BACKGROUND
  Myocardial dysfunction in children diagnosed with mitochondrial disease is an ominous sign and has been associated with substantial increased mortality rates. Early detection of cardiac involvement would therefore be desirable. Two dimensional strain echocardiography (2DSTE) has proven to be more sensitive than conventional echocardiography for the detection of early myocardial dysfunction in various (cardiac) conditions.
  
  
  OBJECTIVE
  To determine left ventricular systolic function in children with mitochondrial disorders by means of physical examination, electrocardiography (ECG), conventional echocardiography and 2DSTE.
  
  
  METHODS
  A total of 27 children with established mitochondrial disease and 54 age-matched control subjects underwent cardiac evaluation. Longitudinal, circumferential and radial peak systolic strain (S) values were determined as well as peak systolic strain rate (Sr) and the time to peak global systolic strain (T2P). One Way analysis of Variance was performed to assess the influence of the presence of mitochondrial disease on conventional echocardiographic and 2DSTE outcomes.
  
  
  RESULTS
  Conventional echocardiographic findings did not indicate systolic left ventricular dysfunction. Global peak S, Sr and T2P measurements in all three directions were significantly lower in children with mitochondrial disease (P<0.001) when compared to controls.
  
  
  CONCLUSIONS
  2DSTE detects alterations in myocardial systolic function in children diagnosed with mitochondrial disease, whose conventional echocardiographic findings did not indicate ventricular systolic dysfunction.
authors.affiliation: Children's Heart Centre, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. k.marcus@cukz.umcn.nl
authors.forename: Karen A
authors.initials: KA
authors.lastname: Marcus
authors.forename: Marlieke
authors.initials: M
authors.lastname: Barends
authors.forename: Eva
authors.initials: E
authors.lastname: Morava-Kozicz
authors.forename: Ton
authors.initials: T
authors.lastname: Feuth
authors.forename: Chris L
authors.initials: CL
authors.lastname: de Korte
authors.forename: Livia
authors.initials: L
authors.lastname: Kapusta
mesh_terms: D000293:Adolescent; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D042241:Early Diagnosis; D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D028361:Mitochondrial Diseases / Q000150:complications* / Q000175:diagnosis*; D012680:Sensitivity and Specificity