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B-Type Natriuretic Peptide Predicts Disease Severity in Children With Hypertrophic Cardiomyopathy
  1. Juan-Pablo Kaski (j.kaski{at}ucl.ac.uk)
  1. Great Ormond Street Hospital, United Kingdom
    1. Maria-Teresa Tome-Esteban (maite.tome{at}uclh.nhs.uk)
    1. Great Ormond Street Hospital, United Kingdom
      1. Sarah J Mead-Regan (meads{at}gosh.nhs.uk)
      1. Great Ormond Street Hospital, United Kingdom
        1. Antonios Pantazis (antonis.pantazis{at}uclh.nhs.uk)
        1. Great Ormond Street Hospital, United Kingdom
          1. Jan Marek (marekj{at}gosh.nhs.uk)
          1. Great Ormond Street Hospital, United Kingdom
            1. John E Deanfield (deanfj{at}gosh.nhs.uk)
            1. Great Ormond Street Hospital, United Kingdom
              1. William J McKenna (william.mckenna{at}uclh.nhs.uk)
              1. Great Ormond Street Hospital, United Kingdom
                1. Perry M Elliott (pelliott{at}doctors.org.uk)
                1. Great Ormond Street, United Kingdom

                  Abstract

                  Objective To assess the relation of B-type natriuretic peptide (BNP) levels to symptoms and markers of disease severity in children with hypertrophic cardiomyopathy (HCM).

                  Background In adults with HCM, plasma BNP levels correlate with dyspnoea class and other markers of disease severity. In children with HCM, symptoms are a poor guide to disease severity and there are no studies evaluating the clinical utility of BNP testing.

                  Methods Forty-four consecutive patients with HCM aged ≤17 years (27 males, median age 13.6 years) underwent assessment of plasma BNP. Patients underwent clinical evaluation, including ECG, echocardiography and tissue Doppler imaging.

                  Results BNP levels correlated with maximal left ventricular (LV) wall thickness (r=0.631, p<0.0001), resting LV outflow tract gradient (r=0.611, p<0.0001), transmitral E/septal Ea (E/Eas) ratio (r=0.770, p<0.0001), and percent predicted maximum VO2 (r=-0.390, p=0.025); there was no relation between BNP and heart failure symptoms. BNP levels were higher in patients who had undergone implantation of an internal cardioverter-defibrillator (309 [IQR 181-391] vs. 50 [IQR 18-188] pg/ml, p=0.001). BNP was independently associated with E/Eas (r=0.632, p<0.0001) and maximal LV wall thickness (r=0.412, p=0.008) on multivariate analysis. At a cut-off of 50pg/ml, BNP had a positive predictive value of 93% and a negative predictive value of 80% for predicting E/Eas>10 (area under the ROC curve = 0.875 [p<0.0001]).

                  Conclusions BNP levels correlate with non-invasive parameters of disease severity in children with HCM, including measures of elevated LV filling pressures. In a group of patients in whom the evaluation of symptoms is difficult, BNP may be a useful additional tool in the assessment of disease severity.

                  • cardiomyopathy
                  • echocardiography
                  • hypertrophy
                  • natriuretic peptides
                  • paediatric

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