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- Open Access
Diffuse myocardial fibrosis in pediatric hypertrophic cardiomyopathy
© Hussain et al; licensee BioMed Central Ltd. 2013
- Published: 30 January 2013
- Hypertrophic Cardiomyopathy
- Gadopentetate Dimeglumine
- Diffuse Fibrosis
- Diffuse Myocardial Fibrosis
- Coefficient Technique
Fibrosis is a common end-point in pathological processes. It is unclear, however, if diffuse fibrosis occurs early in the pathogenesis of hypertrophic cardiomyopathy (HCM). The purpose of this study was to evaluate the presence of diffuse myocardial fibrosis in children and adolescents with HCM using quantification of T1 changes late after gadolinium administration and to assess for associations between markers of fibrosis and standard clinical parameters of disease.
12 controls (mean age 12.8yrs; 7 male) and 28 patients (mean age 12.8yrs; 21 male) participated. All patients had a clinical diagnosis of HCM. Among these, 20 children had HCM-specific mutations. Clinical parameters are given in table 1. PCf for both septal (0.27±0.17 vs. 0.13±0.09 ml/g; p=0.03) and lateral walls (0.22±0.09 vs. 0.07±0.10; p<0.001) were increased in patients compared to controls. PCf did not correlate with age, either in patients or normal individuals.
Parameters in Controls vs. HCM Patients
Controls (mean ± s.d.)
12.8 ± 2.2
12.8 ± 2.6
Septum partition coefficient (ml/g)
0.13 ± 0.09
0.27 ± 0.17
Lateral wall partition coefficient (ml/g)
0.07 ± 0.10
0.22 ± 0.09
10.1 ± 6.6
283 ± 429
Number with LGE
Ejection fraction (%)
57 ± 3
68 ± 10
Indexed myocardial mass (g/m2)
54 ± 7
103 ± 40
Eight patients had overt areas of LGE. These patients did not show increased coefficients compared to those without LGE (0.27±0.15 vs. 0.27±0.19 and 0.22±0.09 vs. 0.22±0.09; p=0.95 and 0.98 respectively). However, patients that were symptomatic (dyspnoea, arrhythmia or chest pain) had higher lateral wall coefficients than asymptomatic HCM patients (0.27±0.08 vs. 0.17±0.08; p=0.006). Similarly, patients with raised BNP (>100 pg/ml) had raised lateral wall coefficients (0.27±0.07 vs. 0.20±0.07; p=0.03). Other clinical parameters did not show any discrimination with respect to the lateral coefficient.
Diffuse fibrosis, demonstrated by the partition coefficient technique, is demonstrable in children and adolescents with HCM. Markers of fibrosis show an association with symptoms and raised serum BNP. Further study of the prognostic implication of this technique is warranted.
No specific funding was given for this research.
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