title: The value of cardiac MRI versus echocardiography in the pre-operative assessment of patients with Duchenne muscular dystrophy.
abstract: OBJECTIVE
  Duchenne Mmuscular Ddystrophy (DMD) related cardiomyopathy is associated with significant perioperative mortality. Cardiac MRI (CMR) has not previously been systematically evaluated as pre-operative assessment tool for heart function in DMD. Our aim was to establish whether CMR versus echocardiography contributes to pre-operative DMD assessment.
  
  
  METHODS
  Case records were retrospectively reviewed of 35 consecutive DMD boys who underwent evaluation for surgical procedures between 2010 and 2013.
  
  
  RESULTS
  Echocardiography revealed a median left ventricular (LV) shortening fraction (SF) of 29/% (range: 7-44). 37% of boys (13/35) had abnormal SF <25%, 66% (23/35) showed hypokinesia and 26% (9/35) had LV dilatation. CMR revealed a median left ventricular ejection fraction (LVEF) of 52% (range: 27-67%). 57% of boys (20/35) had abnormal LVEF <55%, 71% (25/35) had hypokinesia, and 82% late gadolinium enhancement. Extensive versus minimal late gadolinium enhancement was associated with reduced left ventricular ejection fraction (48% vs 58%; p = 0.003) suggesting more severe cardiomyopathy. Although echocardiography shortening fraction correlated with CMR ejection fraction (rs = 0.67; p < 0.001), three-quarter of echocardiography studies had suboptimal scanning windows and in 26% measurements significantly over- or underestimated left-ventricular function compared to CMR.
  
  
  CONCLUSIONS
  Our findings clearly demonstrate the added value of CMR versus echocardiography in assessing DMD-cardiomyopathy. Particularly when echocardiographic scanning windows are suboptimal, CMR should be considered to allow accurate pre-operative cardiac assessment.
authors.affiliation: Dubowitz Neuromuscular Centre, UCL Institute of Child Health & Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: A
authors.initials: A
authors.lastname: Brunklaus
authors.affiliation: Dubowitz Neuromuscular Centre, UCL Institute of Child Health & Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: E
authors.initials: E
authors.lastname: Parish
authors.affiliation: Dubowitz Neuromuscular Centre, UCL Institute of Child Health & Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: F
authors.initials: F
authors.lastname: Muntoni
authors.affiliation: Department of Paediatric Anaesthesia, Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: S
authors.initials: S
authors.lastname: Scuplak
authors.affiliation: Department of Orthopaedics, Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: S K
authors.initials: SK
authors.lastname: Tucker
authors.affiliation: Department of Paediatric Cardiology, Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: M
authors.initials: M
authors.lastname: Fenton
authors.affiliation: Department of Paediatric Cardiology, Great Ormond Street Hospital, London WC1N 3JH, UK.
authors.forename: M L
authors.initials: ML
authors.lastname: Hughes
authors.affiliation: Dubowitz Neuromuscular Centre, UCL Institute of Child Health & Great Ormond Street Hospital, London WC1N 3JH, UK. Electronic address: Adnan.Manzur@gosh.nhs.uk.
authors.forename: A Y
authors.initials: AY
authors.lastname: Manzur
mesh_terms: D009202:Cardiomyopathies / Q000209:etiology / Q000601:surgery*; D002648:Child; D004452:Echocardiography / Q000379:methods*; D006801:Humans; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications* / Q000503:physiopathology; D011300:Preoperative Care / Q000379:methods*; D012189:Retrospective Studies
keywords: CMR; Cardiac MRI; Duchenne muscular dystrophy; Echocardiography