Abstract

Background: In adults with hypertrophic cardiomyopathy (HCM), plasma B-type natriuretic peptide (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 no studies have evaluated the clinical utility of BNP testing.

Objective: To assess the relation of BNP levels to symptoms and markers of disease severity in children with HCM.

Methods: Forty-four consecutive patients with HCM (27 male, age ⩽17 years (median 13.6) underwent assessment of plasma BNP. Clinical evaluation of patients was carried out, including ECG, echocardiography and tissue Doppler imaging.

Results: BNP levels correlated with maximal left ventricular (LV) wall thickness (r_s = 0.631, p<0.001), resting LV outflow tract gradient (r_s = 0.611, p<0.001), transmitral E/septal Ea (E/Ea^s) ratio (r_s = 0.770, p<0.001) and percentage predicted maximum VO₂ (r_s = −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 than in those who had not (309 (interquartile range (IQR) 181–391) vs 50 (IQR 18–188) pg/ml, p = 0.001). BNP was independently associated with E/Ea^s (r_s = 0.632, p<0.001) and maximal LV wall thickness (r_s = 0.412, p = 0.008) on multivariate analysis. At a cut-off point of 50 pg/ml, BNP had a positive predictive value of 93% and a negative predictive value of 80% for predicting E/Ea^s >10 (area under the receiver operator characteristic curve  =  0.875 (p<0.001)).

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