Table I.
Participants’ demographic and baseline characteristics
| Characteristics | All (n = 10) | CL-FD group (n = 5) | CV-FD group (n = 5) | p-valuea |
|---|---|---|---|---|
| Age, years, mean (SD) | 59.00 (11.41) | 53.00 (14.05) | 65.00 (2.35) | 0.151 |
| Height, cm, mean (SD) | 159.72 (1.30) | 153.62 (1.47) | 167.35 (0.40) | 0.111 |
| Weight, kg, mean (SD) | 58.43 (9.79) | 52.06 (5.93) | 66.40 (7.50) | 0.032* |
| BMI, kg/m2, mean (SD) | 23.01 (3.67) | 22.51 (4.91) | 23.64 (1.71) | 0.413 |
| Resting SBP, mmHg, mean (SD) | 119.67 (16.58) | 112.40 (7.40) | 128.75 (21.50) | 0.286 |
| Resting DBP, mmHg, mean (SD) | 72.22 (15.62) | 64.40 (14.52) | 82.00 (11.83) | 0.111 |
| Resting HR, bpm, mean (SD) | 66.22 (11.04) | 69.00 (12.10) | 62.75 (10.05) | 0.556 |
| FVC, L, mean (SD) | 2.51 (0.59) | 2.22 (0.55) | 2.88 (0.44) | 0.111 |
| FVCP, %, mean (SD) | 83.33 (8.70) | 85.40 (10.29) | 80.75 (6.70) | 0.413 |
| FEV1, L, mean (SD) | 1.95 (0.43) | 1.87 (0.53) | 2.06 (0.32) | 0.730 |
| FEV1P, %, mean (SD) | 80.67 (11.90) | 86.60 (9.91) | 73.25 (10.69) | 0.190 |
| bDuration of ERT use, mean (SD) | 52.50 (38.65) | 75.30 (34.85) | 29.70 (29.09) | 0.222 |
| Number of patients with LVH, n, % | 6 (60) | 2 (40) | 4 (80) | 0.197 |
p < 0.05.
Refers to the p-value of a Mann-Whitney U test for continuous variables and χ2 test for non-continuous variables between patients with classic Fabry disease and cardiac variant Fabry disease.
Duration of ERT from first use to the time receiving baseline exercise testing.
CL-FD: classic Fabry disease; CV-FD: cardiac variant Fabry disease; BMI: body mass index; SBP: systolic blood pressure; DBP: diastolic blood pressure; ERT: enzyme replacement therapy; HR: heart rate; FVC: functional vital capacity; FVCP: percentage of predicted forced vital capacity; FEV1: force expiratory volume at 1 min; FEV1P: percentage of predicted forced expiratory volume at 1 min; LVH: left ventricular hypertrophy; SD: standard deviation.