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Subclinical Left Ventricular Dysfunction in Children and Adolescence With Thalassemia Intermedia

Roya Isa Tafreshi1, Mohammad Radgoodarzi2, Kadijeh Arjmandi Rafsanjani3

  • 1Department of Pediatric Cardiology, Ali Asghar Children's Hospital, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

Frontiers in Pediatrics
|July 5, 2022
PubMed

Insights

Early cardiac dysfunction is detectable in children with thalassemia intermedia (TI) using advanced echocardiography. Tissue Doppler imaging (TDI) reveals subtle left ventricular (LV) systolic and diastolic impairments, even without high iron levels.

Area of Science:

  • Cardiology
  • Pediatrics
  • Medical Imaging

Background:

  • Thalassemia intermedia (TI) is associated with significant cardiac morbidity.
  • Early detection of cardiac complications is crucial for managing TI patients.
  • Left ventricular (LV) dysfunction can occur even in asymptomatic individuals.

Purpose of the Study:

  • To assess left ventricular (LV) function in asymptomatic children and adolescents with TI.
  • To utilize novel tissue Doppler imaging (TDI) indices for early diagnosis of ventricular impairment.
  • To identify early systolic and diastolic dysfunction in TI patients.

Main Methods:

  • Compared echocardiographic parameters in 28 asymptomatic TI patients and 35 healthy controls.
  • Utilized 2-D, pulsed Doppler, and TDI for LV mass, mitral inflow, and annular velocities.
  • Assessed myocardial performance index (MPI) and myocardial acceleration during isovolumic contraction (IVA).
  • Estimated cardiac iron load using magnetic resonance imaging T2*.

Main Results:

  • Left ventricular hypertrophy (LVH) present in 46.4% of TI patients.
  • Reduced TDI-derived peak systolic myocardial velocity (s') observed in TI patients.
  • Elevated E/e' ratio, an index of diastolic function, in TI patients (9 ± 1 vs. 6 ± 1, p < 0.05).
  • TDI-derived MPI > 0.33 detected global LV dysfunction with 78% sensitivity and 80% specificity.
  • Patients with LVH showed higher TDI-MPI and lower s' and IVA values.

Conclusions:

  • Subtle LV systolic and diastolic dysfunction occurs early in young TI patients, independent of iron concentration.
  • LV remodeling is a key adaptive response contributing to myocardial impairment in TI.
  • TDI indices are valuable for early detection of cardiac dysfunction in pediatric TI.
Abstract

Keywords:
cardiac complicationschildrenechocardiographyleft ventricular dysfunctionthalassemia intermedia

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