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Table 2 Clinical demographics and investigation data: Fabry versus HCM

From: Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy

  Fabry HCM: primary prevention p valuec
Primary prevention Secondary prevention p valueb
Sample size (n, %) 36 (72) 14 (28) N/A 64 N/A
Follow-up duration (years) 3.8 ± 2.6 5.8 ± 3.9 0.036 6.4 ± 2.9  < 0.001
Age (years) 58 ± 12 57 ± 12 0.922 56 ± 19 0.516
Male gender (n, %) 8 (16) 2 (4) 0.704 21 (33) 0.373
On ERT (n, %) 23 (46) 11 (22) 0.501 N/A
Classical mutation (n, %) 16 (32) 4 (8) 0.353 N/A
BMI (kg/m2) 27.0 ± 6.0 31.6 ± 7.2 0.087 28.4 ± 6.2 0.411
HR (bpm) 64 ± 16 55 ± 8 0.265 66 ± 12 0.633
SBP (mmHg) 122 ± 22 124 ± 19 0.824 128 ± 22 0.436
DBP (mmHg) 71 ± 16 73 ± 7 0.785 76 ± 12 0.292
MSSI 16.7 ± 9.4 11.9 ± 7.1 0.104 N/A
Comorbidities
IHD (n, %) 2 (4) 0 (0) 1.000 3 (5) 1.000
CKD stage 3–5 (n, %) 8 (16) 0 (0) 0.087 0 (0) < 0.005
HTN (n, %) 8 (16) 2 (4) 0.704 16 (25) 0.946
DM (n, %) 3 (6) 2 (4) 0.611 4 (6) 0.700
Stroke/TIA (n, %) 7 (14) 0 (0) 0.169 2 (3) 0.010
ECG n = 48    n = 53  
Abnormal (n, %) 33 (69) 13 (27) 1.000 43 (81) 0.114
AF/PAF (n, %) 3 (6) 0 (0) 0.550 3 (6) 0.664
PR interval (ms) 172 ± 36 145 ± 30 0.051 178 ± 39 0.557
QRS duration (ms) 135 ± 32 134 ± 32 0.939 116 ± 31 0.006
Echocardiography n = 48    n = 62  
LVH (n, %) 32 (67) 12 (25) 1.000 55 (89) 0.485
LA dilated (n, %) 24 (50) 10 (21) 1.000 30 (48) 0.060
CMR n = 27    n = 28  
LVEDV (ml) 158.2 ± 75.0 143.0 ± 18.3 0.791 145.0 ± 53.2 0.564
LVESV (ml) 73.0 ± 80.3 26.0 0.594 54.5 ± 44.9 0.386
LVEF (%)a 58 (53–65) 55 (43–65) 0.747 57 (55–64) 0.904
LVMi (g/m2) 143.6 ± 38.4 164.0 ± 45.0 0.379 102.4 ± 35.7 0.009
MWT (mm) 21.8 ± 5.2 21.3 ± 4.0 0.867 21.6 ± 5.5 0.965
LGE (n, %)d 14/16 (88) 5/9 (56) 0.072 25 (89) 1.000
 Extensive (> 3 AHA segments) 11/16 (69) 3/9 (33) 0.115 14 (50) 0.373
 Mild (1–2 AHA segment e.g. BIFL) 3/16 (19) 2/9 (22) 1.000 9 (32) 0.487
 RV insertion point 0 (0) 0 (0) N/A 2 (7) 0.526
Biomarkers
High sensitive troponin T (ng/L)a 121 (51–154) 90 (44–272) 0.927 19 (13–38)  < 0.001
NT-pro BNP (ng/l)a 1708 (626–4068) 1319 (719–1894) 0.667 888 (353–2070) 0.081
  1. Bold italics indicate statistically significant results (p < 0.05)
  2. Italics indicate the level of significance
  3. aNon-parametric data so presented as median (IQR)
  4. bp value comparing primary and secondary prevention device implantation in FD
  5. cp value comparing primary prevention device implantation in FD and HCM
  6. dProportions taken from patients who had a CMR and were given Gadolinium contrast