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Myocardial deformation in pediatric patients with mucopolysaccharidoses: A two‐dimensional speckle tracking echocardiography study
Author(s) -
Borgia Francesco,
Pezzullo Enrica,
Schiano Lomoriello Vincenzo,
Sorrentino Regina,
Lo Iudice Francesco,
Cocozza Sara,
Della Casa Roberto,
Parenti Giancarlo,
Strisciuglio Pietro,
Trimarco Bruno,
Galderisi Maurizio
Publication year - 2017
Publication title -
echocardiography
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.404
H-Index - 62
eISSN - 1540-8175
pISSN - 0742-2822
DOI - 10.1111/echo.13444
Subject(s) - asymptomatic , medicine , cardiology , ejection fraction , speckle tracking echocardiography , doppler echocardiography , mass index , ventricular function , heart failure , body mass index , blood pressure , diastole
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 .