Left-ventricular mechanics in children with hypertrophic cardiomyopathy. CMR study

Łukasz Mazurkiewicz1, Lidia Ziółkowska2, Joanna Petryka3

  • 1Department of Cardiomyopathies, CMR Unit, Institute of Cardiology, Warsaw, Poland.

Insights

Juvenile hypertrophic cardiomyopathy (HCM) significantly impairs left-ventricular (LV) myocardial mechanics, with reduced strain globally and especially in hypertrophied segments. Left-ventricular outflow tract obstruction (LVOTO) further compromises these mechanics, more so than fibrosis or mass.

Area of Science:

  • Cardiology
  • Biomedical Engineering
  • Pediatric Medicine

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
  • Assessing myocardial mechanics is crucial for understanding HCM progression in children.
  • Left-ventricular outflow tract obstruction (LVOTO) is a common complication in pediatric HCM.

Purpose of the Study:

  • To evaluate myocardial displacement abnormalities in juvenile HCM.
  • To investigate how fibrosis, LVOTO, and hypertrophy affect these abnormalities.
  • To quantify alterations in myocardial mechanics using cardiovascular magnetic resonance (CMR).

Main Methods:

  • Cardiovascular magnetic resonance (CMR) imaging was performed on 55 children with HCM and 20 controls.
  • Left-ventricular (LV) feature tracking (FT) was used to quantify strain and strain rates.
  • Comparisons were made between HCM patients and controls, and between HCM patients with and without LVOTO.

Main Results:

  • Children with HCM showed reduced LV strain in both hypertrophied and non-hypertrophied segments compared to controls.
  • LV global longitudinal strain rate, LV circumferential strain rate, LV radial strain, and LV radial strain rate were significantly lower in patients with LVOTO.
  • Myocardial mechanics indices were significantly associated with the degree of LVOTO, and to a lesser extent with LV mass and fibrosis.

Conclusions:

  • Myocardial mechanics are globally reduced in juvenile HCM, with pronounced deficits in hypertrophied segments.
  • LVOTO significantly exacerbates the impairment of LV strains and strain rates.
  • The degree of LVOTO appears to be a more significant factor in altering myocardial mechanics than LV mass or fibrosis.
Abstract

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