Evaluation of left ventricular systolic function in Hutchinson-Gilford progeria syndrome children by three-dimensional echocardiography

Abstract

Objective To investigate the left ventricular(LV) function in Hutchinson-Gilford progeria syndrome(HGPS) children by three-dimensional echocardiography. Methods Six HGPS children, 21 age-matched children, and 14 senescence-matched healthy volunteers were examined by three-dimensional echocardiography.Image data were collected and analyzed by TomTec 4D LV-Analysis software. LV end-diastolic and end-systolic volume (LVEDV, LVESV), ejection fraction (LVEF), mass, global longitudinal strain (GLS), global circumferential strain (GCS), twist, torsion, mean time to peak, systolic dyssynchrony index (SDI) were calculated and analyzed. Results ①Cardiac function: compared with age-matched children and senescence-matched healthy volunteers, LVEDD showed no significant difference, LVESD, LVEDV, LVESV and mass of HGPS children were significant impaired than those of age-matched children and senescence-matched healthy volunteers (P<0.05); LVEF in HGPS children and age-matched children were significant higher than that in senescence-matched healthy volunteers(P<0.05). ②Strain analysis: GLS of HGPS children and age-matched children were significant higher than that of senescence-matched healthy volunteers(P<0.05), there was no significant difference between HGPS children and age-matched children. GCS of HGPS children showed no significant differences with age-matched children and senescence-matched healthy volunteers.Torsion and twist among three groups showed no significant difference. ③LV synchrony: compared with age-matched children and senescence-matched healthy volunteers, the mean time to peak in HGPS children was significantly reduced (P<0.05). The SDI values in the HGPS children and age-matched children were significantly lower than that of senescence-matched healthy volunteers(P<0.05), while HGPS children and age-matched children showed no significant difference(P<0.05). Conclusions LV dyssynchrony is not showed in HGPS but does in senescence-matched healthy volunteers, indicating HGPS may not have the same aging process as the natural senescence and need further investigation. Key words: Echocardiography, real-time three-dimensional; Progeria; Ventricular function, left; Strain