Male (n = 167) | Female (n = 107) | All (n = 274) | |
---|---|---|---|
Age (full range) | 61 (24–88) years | 60 (21–86) years | 60 (21–88) years |
Genotype | |||
TTR mutation | 105 (63%) | 71 (66%) | 176 (64%) |
V30M | 101 (96%) | 70 (99%) | 171 (97%) |
Other | 4 (4%) | 1 (1%) | 5 (3%) |
No TTR mutation | 26 (16%) | 17 (16%) | 43 (16%) |
Not tested | 36 (21%) | 19 (18%) | 55 (20%) |
Symptom/findinga | |||
Polyneuropathy | 146 (87%) | 100 (93%) | 246 (90%) |
Cardiomyopathy | 62 (35%) | 17 (16%) | 79 (29%) |
GI symptoms | 43 (26%) | 41 (38%) | 84 (31%) |
Eye complications | 21 (13%) | 26 (24%) | 47 (17%) |
Other symptoms | 1 (0.5%) | 2 (2%) | 3 (1%) |
Echocardiogram | |||
IVSd ≥ 12 mm | 68 (72%) | 25 (45%) | 93 (62%) |
IVSd < 12 mm | 26 (28%) | 31 (55%) | 57 (38%) |
99mTc-DPD scint | |||
Positive | 18 (78%) | 6 (46%) | 24 (67%) |
Negative | 5 (22%) | 7 (54%) | 12 (33%) |
Amyloid in first Bx | 70 (42%) | 39 (36%) | 109 (40%) |