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Left Ventricular Noncompaction Cardiomyopathy in Children: Characterization of Clinical Status Using Tissue Doppler-Derived Indices of Left Ventricular Diastolic Relaxation
  1. Colin J McMahon
  1. Baylor College of Medicine, United States
    1. Ricardo H Pignatelli
    1. Baylor College of Medicine, United States
      1. Sherif F Nagueh
      1. Baylor College of Medicine, United States
        1. Vei-Vei Lee
        1. Baylor College of Medicine, United States
          1. William Vaughn
          1. Baylor College of Medicine, United States
            1. Santiago O Valdes
            1. Baylor College of Medicine, United States
              1. John P Kovalchin
              1. Baylor College of Medicine, United States
                1. John Lynn Jefferies
                1. Baylor College of Medicine, United States
                  1. William J Dreyer
                  1. Baylor College of Medicine, United States
                    1. Susan W Denfield
                    1. Baylor College of Medicine, United States
                      1. Sarah Clunie
                      1. Baylor College of Medicine, United States
                        1. Jeffrey A Towbin
                        1. Baylor College of Medicine, United States
                          1. Benjamin W Eidem (eidem.benjamin{at}mayo.edu)
                          1. Mayo Clinic College of Medicine, United States

                            Abstract

                            Background Left ventricular noncompaction (LVNC) may manifest an undulating phenotype ranging from dilated to hypertrophic appearance. It is unknown whether tissue Doppler (TD) velocities can predict adverse clinical outcomes including death and need for transplantation in children with LVNC.

                            Methods and Results We studied 56 children (median age 4.5 years, median follow-up 26 months) with LVNC evaluated at one hospital from January 1999 to May 2004 compared to 56 age/sex-matched control group. Children with LVNC had significantly decreased early diastolic TD velocities at the lateral mitral (11.0 versus 17.0 cm/s) and septal (8.9 versus 11.0 cm/s) annuli compared to normal controls (p<0.001 for each comparison). Using receiver operator characteristic (ROC) curves the lateral mitral Ea velocity proved the most sensitive and specific predictor for meeting the primary endpoint at one year after diagnosis (area under the curve (AUC) = 0.888, standard error (SE) = 0.048, 95% confidence intervals 0.775-0.956). Freedom from death or transplantation was 85% at one year and 77% at two years.

                            Conclusions TD velocities are significantly reduced in patients with LVNC compared to a normal control group. Reduced lateral mitral Ea velocity helps predict children with LVNC who are at risk of adverse clinical outcomes including death and need for cardiac transplantation.

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                              Dr. John L. Jefferies has recently co-authored a paper published by Heart. On the paper, his name reads J. Lynn Jefferies. This should read John L. Jefferies

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                                BMJ Publishing Group Ltd and British Cardiovascular Society