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Diffuse myocardial fibrosis by T1 mapping is associated with heart failure in pediatric primary dilated

Nadya Al-Wakeel-Marquard1, Franziska Seidel2, Christopher Herbst3

  • 1German Heart Center Berlin, Department of Congenital Heart Disease and Pediatric Cardiology, Augustenburger Platz 1, 13353 Berlin, Germany; Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Institute for Imaging Science and Computational Modelling in Cardiovascular Medicine, Augustenburger Platz 1, 13353 Berlin, Germany; DZHK (German Centre for Cardiovascular Research), partner site Berlin.

International Journal of Cardiology
|March 19, 2021
PubMed

Insights

In pediatric dilated cardiomyopathy (DCM), increased myocardial fibrosis (ECV) is linked to younger age and heart failure markers. This finding highlights ECV

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Cardiovascular Imaging

Background:

  • Diffuse myocardial fibrosis is a known predictor of adverse outcomes in adult cardiomyopathy (CM).
  • The clinical significance of myocardial fibrosis in pediatric CM remains largely unexplored.
  • Cardiovascular magnetic resonance (CMR) T1 mapping allows for non-invasive assessment of extracellular volume (ECV), a marker of diffuse fibrosis.

Purpose of the Study:

  • To evaluate myocardial extracellular volume (ECV) using CMR T1 mapping in pediatric patients with different CM phenotypes.
  • To analyze the correlation between ECV and clinical/functional parameters in pediatric CM.
  • To investigate the relevance of ECV in dilated (DCM), hypertrophic (HCM), and left ventricular non-compaction (LVNC) CM.

Main Methods:

  • Prospective enrollment of pediatric patients with DCM, HCM, LVNC, and healthy controls.
  • Standardized CMR with modified Look-Locker Inversion recovery (MOLLI) T1 mapping was performed.
  • ECV measurements were compared between patient groups and controls and correlated with clinical and functional data.

Main Results:

  • Pediatric DCM patients showed significantly higher ECV compared to controls (38.1 ± 7.5% vs. 27.2 ± 3.6%, p = 0.014).
  • Elevated ECV in DCM correlated with younger age (p = 0.044), elevated N-terminal pro brain natriuretic peptide (r = 0.66, p = 0.038), and reduced biventricular ejection fraction and stroke volume.
  • In contrast, ECV was within normal limits in HCM and LVNC and did not correlate with clinical or functional parameters.

Conclusions:

  • Increased diffuse myocardial fibrosis, indicated by elevated ECV, is present in pediatric DCM and is associated with younger age.
  • ECV serves as a valuable marker reflecting heart failure severity in pediatric DCM, correlating with clinical and functional indicators.
  • ECV appears to be a relevant biomarker in pediatric DCM, whereas its role in HCM and LVNC requires further investigation.
Abstract

Keywords:
CardiomyopathyCardiovascular magnetic resonanceExtracellular volumeFibrosisHeart failurePediatric

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