Table 3

Echocardiography cardiac structure measurements of Fabry cohort

Post-treatmentBaselineYear 1Year 2Year 5Year 7Year 10
LVMI (g/m2)
 Non-CKD548.5±12.746.1±12.750.2±21.048.8±13.048.6±13.548.3±13.5*
 CKD573.3±23.171.3±19.378.1±24.690.4±30.184.1±30.3100.2±39.7
IVSd (mm)
 Non-CKD510.7±2.210.3±2.210.4±2.710.8±2.211.4±2.111.2±2.1*
 CKD514.5±2.615.7±4.115.6±3.916.3±5.018.2±6.021.2±7.3
PWd (mm)
 Non-CKD510.6±1.910.2±1.910.5±2.610.8±2.310.9±2.410.4±2.6*
 CKD513.8±3.213.9±3.613.0±3.515.0±2.514.7±2.115.4±3.9
LVEDD (cm)
 Non-CKD55.22±0.325.25±0.405.35±0.355.24±0.335.09±0.485.22±0.34
 CKD55.41±0.775.29±1.025.60±0.925.57±0.795.00±0.405.06±0.64
LA (cm)
 Non-CKD53.87±0.543.74±0.623.77±0.563.71±0.493.79±0.583.59±0.43
 CKD54.08±0.754.05±0.954.26±0.704.29±0.703.98±0.474.18±0.64
  • Non-CKD5 cohort size: baseline (N=15), year 1 (N=15), year 2 (N=15), year 5 (N=12), year 7 (N=12) and year 10 (N=12).

  • CKD5 cohort size: baseline (N=10), year 1 (N=9), year 2 (N=10), year 5 (N=7), year 7 (N=6) and year 10 (N=5).

  • Two-way ANOVA comparing non-CKD5 with CKD5 for parameters LVMI, IVSd, PWd and LA over the duration of treatment were statistically different (p<0.0001, p<0.0001, p<0.0001 and p=0.023, respectively) while LVEDD remained stable (p=0.37).

  • *LVMI, IVSd and PWd in non-CKD5 cohort showed no significant increase over 10 years (p=0.96, p=0.61, p=0.83, respectively).

  • ANOVA, analysis of variance; CKD5, chronic kidney disease stage 5; IVSd, inter-ventricular septal wall thickness; LA, left atrial diameter; LVEDD, LV end diastolic diameter; LVMI, LV mass index; PWd, posterior wall thickness.