Table 1.
All patients N = 208 | Patients who were NYHA class I/II n = 156 | Patients who were NYHA class III n = 43 | |
---|---|---|---|
Age | |||
Mean (SD) | 79.6 (7.0) | 79.4 (6.8) | 79.8 (8.0) |
Median (min, max) | 81.0 (46, 90) | 81.0 (46, 90) | 81.0 (47, 90) |
Sex, n (%) | |||
Male | 179 (86.1) | 136 (87.2) | 34 (79.1) |
Female | 29 (13.9) | 20 (12.8) | 9 (20.9) |
Disease duration, years (IQR) | |||
Age at first symptoms, median | 78.5 (74.0, 82.0) | 78.0 (74.0, 82.0) | 78.0 (73.0, 83.0) |
Age at diagnosis, median | 80.0 (75.0, 84.0)a | 80.0 (75.0, 83.0)b | 79.0 (75.0, 85.0) |
Years since diagnosis, median | 0.5 (0.1, 1.3)a | 0.5 (0.1, 1.3)b | 0.3 (0.1, 1.2) |
Diagnostic method, n* (%) | |||
Scintigraphy | 191 (91.8) | 144 (92.3) | 38 (88.4) |
Other imaging | 172 (82.7) | 133 (85.3) | 30 (69.8) |
Symptoms | 131 (63.0) | 100 (64.1) | 26 (60.5) |
Genetic testing | 107 (51.4) | 81 (51.9) | 23 (53.5) |
Physical examination | 80 (38.5) | 58 (37.2) | 19 (44.2) |
Biopsy | 61 (29.3) | 44 (28.2) | 17 (39.5) |
Heart | 24 (39.3) | 15 (34.1) | 9 (52.9) |
Salivary gland | 28 (45.9) | 23 (52.3) | 5 (29.4) |
Fat pad | 6 (9.8) | 5 (11.4) | 1 (5.9) |
Carpal ligament | 3 (4.9) | 1 (2.3) | 2 (11.8) |
Family history | 3 (1.4) | 3 (1.9) | 0 |
Type of ATTR-CM, n (%) | |||
n with genetic testing | 155 | 119 | 29 |
Wild-type | 141 (91.0) | 109 (91.6) | 25 (86.2) |
Variantc | 14 (9.0) | 10 (8.4) | 4 (13.8) |
NYHA class, n (%) | |||
n with classification | 199 | 156 | 43 |
I | 36 (18.1) | 36 (23.1) | 0 |
II | 120 (60.3) | 120 (76.9) | 0 |
III | 43 (21.6) | 0 | 43 (100.0) |
IV | 0 | 0 | 0 |
Left ventricular ejection fraction, n (%) | |||
n with measure | 197 | 149 | 42 |
≥55% | 103 (52.3) | 80 (53.7) | 20 (47.6) |
40%–54% | 77 (39.1) | 56 (37.6) | 18 (42.9) |
≤39% | 17 (8.6) | 13 (8.7) | 4 (9.5) |
ATTR-CM, transthyretin amyloid cardiomyopathy; IQR, interquartile range; NYHA, New York Heart Association; SD, standard deviation.
n = 207.
n = 155.
Including n = 7 with I68l (p.I88l; 1 NYHA class III), 1 with V30M (p.V50M; NYHA class III), 1 with V122I (p.V142I; NYHA class III), and 4 with other transthyretin gene variations (1 NYHA class III).
More than one can apply.