Biatrial performance in children with hypertrophic cardiomyopathy: CMR study

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Łukasz Mazurkiewicz1, Lidia Ziółkowska2, Joanna Petryka3

  • 1Departament of Cardiomyopathies, CMR Unit, Institute of Cardiology, 42nd Alpejska Str, 04-682, Warsaw, Poland. lmazurkiewicz@ikard.pl.

European Radiology
|June 9, 2018
PubMed

Insights

Biatrial mechanics are impaired in children with hypertrophic cardiomyopathy (HCM), with left atrial issues linked to outflow tract obstruction and right atrial problems associated with fibrosis.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Cardiac Imaging

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart disease characterized by ventricular hypertrophy.
  • Biatrial abnormalities are common in HCM but their specific relationship with left ventricular outflow tract obstruction (LVOTO) and myocardial characteristics remains unclear in pediatric populations.
  • Understanding these relationships is crucial for assessing disease severity and guiding treatment in children with HCM.

Purpose of the Study:

  • To investigate biatrial mechanics in children with HCM.
  • To determine the association between biatrial function and left ventricular outflow tract obstruction (LVOTO).
  • To explore the relationship between biatrial function, left ventricular (LV) hypertrophy, diastolic dysfunction, and fibrosis in pediatric HCM.

Main Methods:

  • Cardiac magnetic resonance (CMR) and echocardiography were performed on 55 children with HCM and 20 healthy controls.
  • Phasic components of biatrial function, biventricular diastolic function, and myocardial fibrosis were quantified.
  • LVOTO severity, LV mass, and hypertrophy were assessed.

Main Results:

  • Children with HCM exhibited significantly increased left atrial (LA) and right atrial (RA) volumes and reduced atrial contractile function compared to controls.
  • LA volumetric and mechanical dysfunction were strongly associated with the degree of LVOTO.
  • RA volumetric and contractile dysfunction were linked to LV fibrosis and mass.

Conclusions:

  • Biatrial volumetric and functional indices are severely compromised in pediatric HCM.
  • LVOTO appears to be a significant driver of LA volumetric and mechanical abnormalities.
  • RA dysfunction is associated with myocardial fibrosis and increased LV mass in this population.
Abstract

Keywords:
ChildrenFibrosisHeart atriaHypertrophic cardiomyopathyStrain

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