Key result
Pediatric HCM features enlarged biatrial volumes and reduced function, with LA impairment tracking LVOT obstruction.
Cross-Sectional (n=75)
No
p-value: p=<0.05
In children with hypertrophic cardiomyopathy, biatrial function is severely compromised, with left atrial malfunction linked to LVOTO severity and right atrial dysfunction linked to LV fibrosis and mass.
May support atrial assessment in pediatric HCM; leaves open prognostic value in longitudinal cohorts.
OBJECTIVES: To investigate biatrial mechanics and their relation with left ventricular outflow tract (LVOT) obstruction (LVOTO), the degree of hypertrophy, indices of ventricular diastolic function and fibrosis in children with hypertrophic cardiomyopathy (HCM). METHODS: Fifty-five consecutive, prospectively recruited children with HCM (mean age 12.5 ± 4.6 years, 69.1% male), 19 (34.5%) of whom had LVOTO, underwent cardiac magnetic resonance and echocardiography with quantification of phasic components of biatrial function, biventricular diastolic function and fibrosis. Twenty healthy, sex-matched subjects served as controls. RESULTS: We found a significant increase of left atrial (LA) and right atrial (RA) volumes and reduction in the majority of indices of contractile function, strains and strain rates (p < 0.05) in children with HCM compared with controls. Nearly all of the LA dynamics markers attained a significant association with the LVOT gradient (p < 0.05), the RA volumes and contractile functions were affected by LV fibrosis and mass (p < 0.05), and the RA mechanical components were related to the degree of LVOTO (p < 0.05). The minority of biatrial dynamics markers were associated with indices of ventricular diastolic function. CONCLUSIONS: The majority of biatrial volumetric and functional indices were severely compromised in children with HCM compared with controls. The degree of LVOTO appears to trigger LA volumetric and LA and RA mechanical malfunction. On the other hand, the deterioration of RA volumetric components was linked to LV fibrosis and mass. KEY POINTS: • Biatrial function was severely compromised in children with HCM. • Left atrial malfunction was associated with the degree of LVOTO. • Fibrosis and LV mass were related to RA volumetric and contractile dysfunction. • The degree of LVOTO was linked to right atrial mechanical abnormalities.
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Mazurkiewicz et al. (2018) conducted a cross-sectional in Hypertrophic cardiomyopathy (n=75). Hypertrophic cardiomyopathy vs. Healthy controls was evaluated on Biatrial volumetric and functional indices (LA and RA volumes, strains, and strain rates) (p=<0.05). Children with hypertrophic cardiomyopathy exhibited significantly increased biatrial volumes and reduced contractile function compared to healthy controls, with left atrial malfunction associated with the degree of left ventricular outflow tract obstruction.