Table 2.
Total (n = 246) | Hereditary ATTR‐CM (n = 13) | Wild‐type ATTR‐CM (n = 233) | p‐value | |
---|---|---|---|---|
Baseline characteristics | ||||
Female sex, n (%) | 39 (15.9) | 5 (38.5) | 34 (14.6) | 0.022 |
Race, n (%) | <0.001 | |||
Caucasian | 244 (99.2) | 11 (84.6) | 233 (100) | |
Black | 2 (0.8) | 2 (15.4) | 0 (0.0) | |
Age at diagnosis, years, median (IQR) | 80 (76–85) | 79 (73–82) | 80 (76–85) | 0.26 |
ATTRv genotype (n = 35), n (%) | ||||
Val50Met | 3 (23.1) | |||
Val142Ile | 10 (76.9) | |||
Type of diagnosis, n (%) | 1.00 | |||
Invasive | 65 (26.4) | 3 (23.1) | 62 (26.6) | |
Non‐invasive | 181 (73.6) | 10 (76.9) | 171 (73.4) | |
Diagnosis era, n (%) | 1.00 | |||
<2015 | 25 (10.2) | 1 (7.7) | 24 (10.3) | |
2016–2021 | 221 (89.8) | 12 (92.3) | 209 (89.7) | |
Onset symptoms, n (%) | 0.802 | |||
Dyspnoea | 191 (63.7) | 23 (65.7) | 168 (63.4) | |
Peripheral oedema | 12 (4.0) | 0 | 12 (4.5) | |
Chest pain | 8 (2.7) | 1 (2.9) | 7 (2.6) | |
Palpitations | 14 (4.7) | 3 (8.6) | 11 (4.2) | |
Syncope | 19 (6.3) | 3 (8.6) | 16 (6.0) | |
Incidental finding | 43 (14.3) | 4 (11.4) | 39 (14.7) | |
Other | 13 (4.3) | 1 (2.9) | 12 (4.5) | |
Polyneuropathy, n (%) | 40 (16.3) | 8 (61.5) | 32 (13.7) | <0.001 |
Carpal tunnel syndrome, n (%) | 87 (35.4) | 7 (53.9) | 80 (34.3) | 0.152 |
History of embolism, n (%) | 22 (8.9) | 3 (23.1) | 19 (8.2) | 0.07 |
Atrial fibrillation, n (%) | 166 (67.5) | 7 (53.9) | 159 (68.2) | 0.281 |
Hypertension, n (%) | 172 (69.9) | 8 (61.5) | 164 (70.4) | 0.498 |
Coronary artery disease, n (%) | 40 (16.3) | 0 | 40 (17.8) | 0.135 |
Pacemaker, n (%) | 40 (22.5) | 2 (22.2) | 38 (22.5) | 1 |
Chronic kidney disease (stage ≥3A), n (%) | 126 (51.2) | 5 (38.5) | 121 (51.9) | 0.402 |
Baseline blood tests | ||||
eGFR, ml/min/1.73 m2, mean ± SD | 58.6 ± 18.1 | 63.1 ± 20.3 | 58.3 ± 17.9 | 0.357 |
NT‐proBNP, pg/ml, median (IQR) | 2592 (1020–4498) | 4794 (1650–9524) | 2544 (1020–4413) | 0.104 |
Conventional troponin I (μg/L), median (IQR) | 0.06 (0.03–0.11) | 0.08 (0.04–0.19) | 0.06 (0.03–0.10) | 0.45 |
Baseline ECG, n (%) | ||||
1st degree AV block | 42 (17.1%) | 1 (7.7%) | 41 (17.6%) | 0.70 |
LBBB | 41 (16.7%) | 3 (23.1%) | 38 (16.3) | 0.27 |
RBBB | 44 (17.9%) | 1 (7.7%) | 43 (18.5%) | 0.29 |
Baseline echocardiogram | ||||
Interventricular wall thickness, mm, mean ± SD | 17.9 ± 3.4 | 18.7 ± 3.0 | 17.9 ± 3.4 | 0.40 |
End‐diastolic left ventricular diameter, mm, mean ± SD | 42.7 ± 6.4 | 40.5 ± 5.8 | 42.8 ± 6.5 | 0.21 |
Left ventricular ejection fraction, %, mean ± SD | 54.2 ± 11.5 | 57.1 ± 6.8 | 54.0 ± 11.7 | 0.35 |
Global longitudinal strain, −% (n = 173), mean ± SD | 12.3 ± 4.4 | 11.2 ± 2.5 | 12.3 ± 4.5 | 0.44 |
Left atrial diameter, mm, mean ± SD | 44.7 ± 6.4 | 41.1 ± 6.9 | 44.9 ± 6.4 | 0.06 |
Aortic stenosis (at least moderate), n (%) | 10 (3.3) | 0 (0.0) | 10 (3.8) | 0.61 |
Pericardial effusion, n (%) | 52 (17.5) | 9 (25.7) | 43 (16.4) | 0.17 |
ATTR‐CM, transthyretin amyloid cardiomyopathy; ATTRv, hereditary transthyretin amyloidosis; AV, atrioventricular; ECG, electrocardiogram; eGFR, estimated glomerular filtration rate; IQR, interquartile range; LBBB, left bundle branch block; NT‐proBNP, N‐terminal pro‐brain natiuretic peptide; RBBB, right bundle branch block; SD, standard deviation.