Baseline demographic and investigation data: FD-CIED versus FD-control
| FD-CIED | FD-control | P value* | |
| Sample size (n) | 90 | 276 | N/A |
| Age (years) | 56±13 | 46±16 | <0.001 |
| Male gender (n, %) | 69 (76.7) | 100 (36.2) | <0.001 |
| On ERT (n, %) | 62 (68.9) | 131 (47.5) | <0.001 |
| Classical mutation (n, %) | 39 (43) | 163 (59) | 0.010 |
| BMI (kg/m2) | 27±6 | 25±5 | 0.002 |
| HR (bpm) | 71±18 | 65±13 | 0.064 |
| SBP (mm Hg) | 125±18 | 123±18 | 0.406 |
| DBP (mm Hg) | 74±11 | 74±10 | 0.914 |
| MSSI | 15.1±9.7 | 7.3±7.7 | <0.001 |
| Comorbidities | |||
| IHD (n, %) | 6 (6.7) | 7 (2.5) | 0.115 |
| CKD stage 3–5 (n, %) | 18 (20) | 26 (9.4) | 0.017 |
| eGFR (median, IQR) | 82 (46 to 74) | 84 (72–90) | 0.017 |
| HTN (n, %) | 18 (20) | 35 (12.7) | 0.120 |
| DM (n, %) | 8 (8.9) | 8 (2.9) | 0.046 |
| Stroke/TIA (n, %) | 22 (24.4) | 11 (4.0) | <0.001 |
| ECG | n=76 | n=239 | |
| Abnormal (n, %) | 74 (97.4) | 115 (48.1) | <0.001 |
| AF/PAF (n, %) | 8 (10.5) | 9 (3.7) | 0.037 |
| PR interval (ms) | 174±40 | 147±28 | <0.001 |
| QRS duration (ms) | 136±32 | 99±20 | <0.001 |
| Holter monitoring | n=52 | n=85 | |
| Abnormal | 37 (71.2) | 12 (14.1) | <0.001 |
| Echocardiography | n=82 | n=91 | |
| LVEF (%) | 57±13 | 62±7 | 0.002 |
| LVH (n, %) | 78 (95.1) | 36 (39.6) | <0.001 |
| LA dilatation (n, %) | 56 (68.3) | 13 (14.3) | <0.001 |
| CMR | n=46 | n=210 | |
| LVMi (g/m2) | 150±37.3 | 83±36 | <0.001 |
| MWT (mm) | 20±4.9 | 12±4.6 | <0.001 |
| LVEDVi (mL/m2) | 82±32 | 70±14 | 0.143 |
| LVESVi (mL/m2) | 31±31 | 19±7 | 0.150 |
| LGE (n, %) | 29 (63.0) | 85 (40.5) | < 0.05 |
*P-values are comparing FD-CIED versus FD-control.
AF, atrial fibrillation; BMI, body mass index; CKD, chronic kidney disease; CMR, cardiac magnetic resonance; DBP, diastolic blood pressure; DM, diabetes mellitus; ECG, electrocardiogram; ERT, enzyme replacement therapy; FD-CIED, Fabry disease with cardiovascular implantable electronic device; HR, heart rate; HTN, hypertension; IHD, ischemic heart disease; LA, left atrium; LGE, late gadolinium enhancement; LVEDVi, indexed left ventricular end-diastolic volume; LVEF, left ventricular ejection fraction; LVESVi, indexed left ventricular end-systolic volume; LVH, left ventricular hypertrophy; LVMi, indexed left ventricular mass; MSSI, Mainz Severity Score Index; MWT, maximum wall thickness; PAF, paroxysmal atrial fibrillation; SBP, systolic blood pressure; TIA, transient ischemic attack; eGFR, estimated glomerular filtration rate.