Table 3.
IVS4 mutation | Classical Fabry mutation | |||
---|---|---|---|---|
Age at first symptom | Male (N = 30) | Female (N = 11) | Male (N = 6) | Female (N = 12) |
LVHa | n = 30 (100%) | n = 10 (90.9%) | n = 4 (66.7%) | n = 11 (91.7%) |
Mean (SD) | 55.9 (11.3)b | 57.4 (13.7) | 30.0 (15.1)b,c | 49.6 (8.9)c |
Median (range) | 58.9 (19.9–68.3) | 57.8 (34.7–86.0) | 28.1 (14.3–49.4) | 51.7 (32.6–59.7) |
Chest pain | n = 9 (30%) | n = 5 (45.5%) | n = 1 (16.7%) | n = 8 (66.7%) |
Mean (SD) | 48.6 (12.7) | 40.9 (10.8) | 43.5 | 47.8 (13.6) |
Median (range) | 47.4 (27.1–66.6) | 43.2 (24.4–52.1) | 43.5 (43.5–43.5) | 48.8 (29.2–68.2) |
Arrhythmia or conductive abnormality | n = 25 (83.3%)d | n = 6 (54.5%) | n = 0 (0%)d | n = 7 (58.3%) |
Mean (SD) | 58.0 (7.3) | 63.3 (19.6) | – | 49.2 (11.5) |
Median (range) | 60.3 (39.8–67.5) | 60.5 (37.1–86.0) | – | 51.2 (32.7–65.7) |
Palpitations or syncope | n = 10 (33.3%) | n = 5 (45.5%) | n = 1 (16.7%) | n = 6 (50%) |
Mean (SD) | 54.7 (10.2) | 42.4 (11.9) | 43.5 | 51.6 (13.2) |
Median (range) | 58.8 (39.8–66.6) | 47.0 (24.4–54.8) | 43.5 (43.5–43.5) | 52.5 (29.2–68.2) |
aAge at first diagnosis of LVH
bAge, IVS4 male vs. classical Fabry male; p < 0.05
cAge, classical Fabry male vs. classical Fabry female; p < 0.05
dPrevalence, IVS4 male vs. classical Fabry male; p < 0.001