Key result
Children with mucopolysaccharidoses had lower LV global circumferential strain (P=0.014), radial strain (P=0.023), twisting (P=0.012), and RV global longitudinal strain (P<0.001) than controls.
Why the study?
Do asymptomatic children with mucopolysaccharidoses have detectable subclinical myocardial damage on 2D speckle tracking echocardiography compared to healthy controls?
Case-Control (n=30)
Do asymptomatic children with mucopolysaccharidoses have detectable subclinical myocardial damage on 2D speckle tracking echocardiography compared to healthy controls?
2D speckle tracking echocardiography can detect subclinical biventricular myocardial deformation abnormalities in asymptomatic pediatric patients with mucopolysaccharidoses despite normal ejection fraction.
May support strain imaging for subclinical assessment in pediatric MPS; leaves open prognostic value and management implications.
BACKGROUND: Mucopolysaccharidoses (MPS) are inherited lysosomal storage disorders caused by deficiency of required glycosaminoglycans breakdown enzymes, inducing cardiac involvement. Little is known about myocardial deformation involvement in MPS. Our aim was to assess biventricular structure and function in asymptomatic children with MPS using standard echo Doppler and 2D speckle tracking (STE). METHODS: Fifteen MPS children (one type I, six type II, three type III A, one III B, three IV A, one VI), asymptomatic for cardiac symptoms, and 15 age and sex-matched healthy controls underwent echo Doppler and STE. Left ventricular (LV) wall thicknesses, diameters, and mass were normalized by z-score. LV global longitudinal strain (GLS), global circumferential strain (GCS), global radial strain (GRS) at papillary muscles, LV twisting, and right ventricular (RV) GLS were measured. RESULTS: The two groups were comparable for body mass index, heart rate, and blood pressure. LV mass index and relative wall thickness were higher in MPS. Ejection fraction (EF), and s' velocity did not differ between the two groups. E/A ratio was lower and E/e' higher in MPS. Tricuspid annular plane systolic excursion, RV s' and e' were lower in MPS. LV GLS did not differ between the two groups, but GCS (P=.014), GRS (P=.023), twisting (P=.012), and RV GLS (P<.001) were lower in the MPS group. CONCLUSIONS: LV strain abnormalities are detectable in MPS pediatric patients, independently of MPS type, when EF is still normal. RV GLS is also involved consensually with TAPSE reduction. STE can be useful for detection of subclinical myocardial damage in MPS.
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Borgia et al. (2017) conducted a case-control in Mucopolysaccharidoses (n=30). Mucopolysaccharidoses vs. Healthy controls was evaluated on Biventricular structure and function (LV and RV strain). Children with mucopolysaccharidoses had lower LV global circumferential strain (P=0.014), radial strain (P=0.023), twisting (P=0.012), and RV global longitudinal strain (P<0.001) than controls.
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