Introduction

The prognostic value of late gadolinium enhancement (LGE) in adult dilated cardiomyopathy (DCM) has been confirmed. However, the prognostic value of LGE in children with DCM remains to be investigated. This study aims to evaluate the prognostic value of late LGE in children with DCM.

Methods

This retrospective single-center study analyzed 76 consecutive children with DCM who underwent cardiac magnetic resonance (CMR) to assess left ventricular dysfunction and myocardial fibrosis between July 2018 and December 2022. The composite endpoint was all-cause mortality, heart transplant, and rehospitalization for heart failure. The risk factors for the composite endpoint were identified with multivariable Cox analysis.

Results

Among the 76 children (mean age, 6.1±4.9 years; 48 male [63.2%]), 42 patients (55.3%) were primary DCM, and 34 (44.7%) were secondary DCM. Forty-two patients (55.3%) had LGE, and 34 did not. During a mean follow-up period of 16.4±16.6 months (range, 1-69 months), a total of 40 patients reached the composite endpoint, in which 20 (50.0%) died of heart failure, 3 (7.5%) underwent a heart transplant, 17 (42.5%) rehospitalized for heart failure. Patients who reached the composite endpoint had a higher Lg-BNP (3.4 pg/mL [3.2, 3.7] vs. 2.6 pg/mL [1.5, 3.5], p=0.004). There is no significant difference among sex, age of diagnosis, body surface area, family history of DCM, and NYHA/Ross class between patients who reached the composite endpoint and those who did not. While, patients who reached the composite endpoint had a higher indexed left ventricular (LV) end-diastolic volume (LVEDVi) (130.8±47.5mL/m2 vs 103.1±33.4 mL/m2, p=0.004), indexed LV end-systolic volume (LVESVi) (110.8mL/m2 [59.4, 131.9] vs. 62.1mL/m2 [44.2, 92.7], p=0.003), indexed LV mass (LVMi) (59.7g/m2 [45.2, 70.1] vs. 49.6g/m2 [39.1, 59.3], p=0.03) and lower LVEF (19.8% [14.5, 29.7] vs. 33.1% [22.9, 47.1]), p=0.002). In univariable Cox regression analyses, Lg-BNP, systolic blood pressure, LVEDVi, LVESVi, LVMi, LVEF, and LGE present were all associated with the primary endpoint (all p<0.05). Multivariable stepwise analysis showed that only LGE present was associated with the composite endpoints (hazard ratio [HR], 2.59 [95% CI: 1.05, 6.37], p=0.039).

Conclusions

Myocardial fibrosis assessment by LGE based on CMR can be used to identify children with DCM who are at high risk of all-cause death, heart transplant, and clinical deterioration.

Acknowledgements

Not Applicable.

References

none