Table 1. Baseline clinical and demographic characteristics of patients with transthyretin amyloid cardiomyopathy, stratified by subtype.
| Variable | Patients with ATTR-CA, No. (%) | P value | ||
|---|---|---|---|---|
| Overall (n=183) | ATTRwt (n=112) | ATTRm (n=71) | ||
| Age, years | 73.0 (66.0–79.0) | 74.5 (69.0–81.0) | 69.0 (59.0–77.0) | <0.001 |
| Sex | 0.017 | |||
| Male | 143 (78.1%) | 94 (83.9%) | 49 (69.0%) | |
| Female | 40 (21.9%) | 18 (16.1%) | 22 (31.0%) | |
| Follow-up duration (years) | 1.36 (0.48–2.97) | 1.05 (0.43–2.86) | 1.51 (0.66–3.10) | 0.196 |
| Death | 46 (25.3%) | 30 (27.0%) | 16 (22.5%) | 0.496 |
| Method of diagnosis | 0.043 | |||
| PYP scan | 88 (48.1%) | 55 (49.1%) | 33 (46.5%) | |
| Biopsy | 68 (37.2%) | 46 (41.1%) | 22 (31.0%) | |
| Imaging* | 16 (8.7%) | 8 (7.1%) | 8 (11.3%) | |
| Genetics only | 10 (5.5%) | 2 (1.8%) | 8 (11.3%) | |
| Family history | 23 (12.6%) | 4 (3.6%) | 19 (26.8%) | <0.001 |
| Symptoms at diagnosis | <0.001 | |||
| No symptoms | 19 (10.4%) | 6 (5.4%) | 13 (18.3%) | |
| Cardiac | 140 (76.5%) | 99 (88.4%) | 41 (57.8%) | |
| Neuropathic | 12 (6.6%) | 2 (1.8%) | 10 (14.1%) | |
| Mixed | 11 (6.0%) | 5 (4.5%) | 6 (8.5%) | |
| Other† | 1 (0.6%) | 0 (0%) | 1 (1.4%) | |
| Comorbidities | ||||
| Dyslipidaemia | 81 (51.9%) | 54 (56.8%) | 34 (55.7%) | 0.125 |
| Diabetes | 29 (17.5%) | 15 (14.9%) | 14 (21.5%) | 0.269 |
| Hypertension | 95 (56.9%) | 56 (54.9%) | 39 (60.0%) | 0.517 |
| Carpal tunnel syndrome | 55 (30.2%) | 35 (31.2%) | 20 (28.6%) | 0.702 |
| NYHA class | 175 | 108 | 67 | 0.141 |
| Asymptomatic‡ | 9 (5.1%) | 2 (1.9%) | 7 (10.5%) | |
| Class 1 | 26 (14.9%) | 15 (13.9%) | 11 (16.4%) | |
| Class 2 | 68 (38.9%) | 45 (41.7%) | 23 (34.3%) | |
| Class 3 | 62 (35.4%) | 40 (37.0%) | 22 (32.8%) | |
| Class 4 | 10 (5.7%) | 6 (5.6%) | 4 (6.0%) | |
| Transplantation | 0.064 | |||
| Liver | 4 (2.2%) | 1 (0.9%) | 3 (4.3%) | |
| Heart | 2 (1.1%) | 0 (0%) | 2 (2.9%) | |
| None | 174 (96.7%) | 109 (99.1%) | 65 (92.9%) | |
| Systems dysfunction | ||||
| Multiorgan involvement* | 21 (11.6%) | 7 (6.3%)* | 14 (20.0%)* | 0.005 |
| Autonomic dysfunction | 31 (17.0%) | 12 (10.7%) | 19 (27.1%) | 0.004 |
| Sensory peripheral neuropathy | 43 (23.6%) | 14 (12.5%) | 29 (41.4%) | <0.001 |
| Motor neuropathy | 21 (11.5%) | 7 (6.3%) | 14 (20.0%) | 0.005 |
| Neuropathy symptoms | 87 (47.8%) | 46 (41.2%) | 41 (58.6%) | 0.022 |
| Laboratory measurements | ||||
| Creatinine, µmol/L | 122.79±66.75 | 126.37±54.17 | 117.41±82.26 | 0.019 |
| eGFR, mL/min | 58.35±24.15 | 53.8±21.1 | 65.03±26.83 | 0.009 |
| BNP, pg/mL | 469.82±463.7 | 524.29±513.46 | 397.51±380.31 | 0.093 |
| cTn, pg/mL | 309.77±1243.07 | 173.52±486.55 | 475.75±1766.92 | 0.975 |
| Echocardiogram parameters | ||||
| IVSd, mm | 1.52±0.39 | 1.55±0.36 | 1.48±0.43 | 0.321 |
| LVIDd, mm | 4.37±0.57 | 4.43±0.57 | 4.3±0.58 | 0.384 |
| LVIDs, mm | 3.33±0.69 | 3.34±0.73 | 3.32±0.64 | 0.869 |
| LVPWd, mm | 1.45±0.4 | 1.52±0.38 | 1.36±0.4 | 0.029 |
| LV mass, g | 269.68±109.71 | 287.29±110.82 | 246.32±104.73 | 0.024 |
| LVEF, % | 51.26±12.69 | 52.18±11.24 | 49.99±14.45 | 0.391 |
| 99mTc‐PYP scintigraphy parameters | ||||
| H/CL ratio | 1.90±0.47 | 1.92±0.47 | 1.86±0.47 | 0.660 |
| Medications | ||||
| Diuretic | 125 (68.3%) | 82 (73.2%) | 43 (60.6%) | 0.073 |
| Beta blocker | 83 (45.4%) | 55 (49.1%) | 28 (39.4%) | 0.200 |
| ACEi | 50 (27.3%) | 35 (31.2%) | 15 (21.1%) | 0.134 |
| ARB | 31 (16.9%) | 17 (15.2%) | 14 (19.7%) | 0.425 |
| Digoxin | 7 (3.8%) | 5 (4.5%) | 2 (2.8%) | 0.571 |
| MCRA | 57 (31.2%) | 41 (36.6%) | 16 (22.5%) | 0.045 |
| Diflunisal | 10 (5.5%) | 6 (5.4%) | 4 (5.6%) | 0.936 |
| Other§ | 67 (36.6%) | 37 (33.0%) | 30 (42.3%) | 0.207 |
Data are presented as median (IQR) for continuous variables and n (%) for categorical variables. Due to missing observations, the number of patients included in the analysis is specified (ie, overall total) for variables where it differs from the total. P values were calculated using the Wilcoxon rank-sum test for continuous variables and the χ2 or Fisher’s exact test for categorical variables.
Imaging includes CMR and/or transthoracic echocardiography as the primary imaging modality for diagnosis.
'Other’ symptoms include those related to specific comorbid conditions that led to hospitalisation preceding the formal diagnosis of amyloidosis.
'Asymptomatic’ under NYHA class refers to patients with amyloid deposits identified in non-cardiac tissue without clearly defined clinical cardiac disease at the time of assessment.
'Other’ medications include disease-modifying therapies for transthyretin amyloidosis (eg, patisiran, inotersen, tafamidis) and other classes of cardiac medications not otherwise specified in the table.
ACEi, ACE inhibitor; ARB, angiotensin-receptor blocker; ATTR-CA, transthyretin amyloid cardiomyopathy; ATTRm, hereditary transthyretin amyloidosis; ATTRwt, wild type transthyretin amyloidosis; BNP, B type natriuretic peptide; CMR, cardiovascular magnetic resonance; cTn, cardiac troponin; eGFR, estimated glomerular filtration rate; H/CL, heart-to-contralateral lung (ratio); IVSd, interventricular septal thickness at end-diastole; LV, left ventricular; LVEF, left ventricular ejection fraction; LVIDd, left ventricular internal diameter at end-diastole; LVIDs, left ventricular internal diameter at end-systole; LVPWd, left ventricular posterior wall thickness at end-diastole; MCRA, mineralocorticoid receptor antagonist; 99mTc-PYP, technetium-99m pyrophosphate; NYHA, New York Heart Association; PYP, pyrophosphate.