Variables | Total (n = 124) | Cardiac death (n = 49) | Non-cardiac death (n = 75) | p value |
---|---|---|---|---|
Demographic and Clinical Characteristics | ||||
Age at diagnosis (years), Mean ± SD | 59.87 ± 9.24 | 59.82 ± 9.92 | 59.91 ± 8.84 | 0.958 |
Age at symptom onset (years), Mean ± SD | 59.08 ± 9.30 | 59.12 ± 9.65 | 59.05 ± 9.14 | 0.968 |
Male sex, n (%) | 80 (64.52) | 32 (65.31) | 48 (64.00) | 0.882 |
Height (cm), Median (IQR) | 167.00 (160.00, 170.25) | 165.00 (160.00, 171.00) | 167.00 (160.00, 170.00) | 0.632 |
Weight (kg), Median (IQR) | 64.00 (58.00, 71.25) | 64.00 (57.50, 71.00) | 64.00 (58.00, 72.00) | 0.814 |
Body mass index (kg/m²), Median (IQR) | 23.19 (21.68, 25.38) | 23.02 (21.88, 24.97) | 23.34 (21.47, 26.08) | 1.000 |
Body surface area (m²), Median (IQR) | 1.69 (1.56, 1.81) | 1.67 (1.56, 1.82) | 1.69 (1.59, 1.80) | 0.671 |
Mayo Clinic 2004 Staging System | < 0.001 | |||
Stage 1, n (%) | 15 (12.1) | 0 (0.0) | 15 (20.0) | |
Stage 2, n (%) | 60 (48.4) | 20 (40.8) | 40 (53.3) | |
Stage 3, n (%) | 49 (39.5) | 29 (59.2) | 20 (26.7) | |
Mayo Clinic 2012 Staging System | < 0.001 | |||
Stage 1, n (%) | 33 (26.6) | 3 (6.1) | 30 (40.0) | |
Stage 2, n (%) | 34 (27.4) | 14 (28.6) | 20 (26.7) | |
Stage 3, n (%) | 49 (39.5) | 27 (55.1) | 22 (29.3) | |
Stage 4, n (%) | 8 (6.5) | 5 (10.2) | 3 (4.0) | |
European Modification 2015 of Mayo Staging System | < 0.001 | |||
Stage 1, n (%) | 15 (12.1) | 0 (0.0) | 15 (20.0) | |
Stage 2, n (%) | 60 (48.4) | 20 (40.8) | 40 (53.3) | |
Stage 3a, n (%) | 32 (25.8) | 19 (38.8) | 13 (17.3) | |
Stage 3b, n (%) | 17 (13.7) | 10 (20.4) | 7 (9.3) | |
NYHA functional class, Median (IQR) | 2.00 (1.00, 2.25) | 2.00 (2.00, 3.00) | 1.00 (1.00, 2.00) | < 0.001 |
Time from symptom onset to diagnosis (months), Median (IQR) | 6.00 (2.00, 12.00) | 6.00 (2.00, 12.00) | 6.00 (2.50, 12.00) | 0.841 |
Cardiac manifestations as initial presentation, n (%) | 34 (27.42) | 23 (46.94) | 11 (14.67) | < 0.001 |
History of hypotension, n (%) | 21 (16.94) | 13 (26.53) | 8 (10.67) | 0.021 |
Syncope/presyncope episodes, n (%) | 10 (8.06) | 9 (18.37) | 1 (1.33) | 0.002 |
Initial admission MAP (mmHg), Mean ± SD | 90.22 ± 12.12 | 87.91 ± 14.05 | 91.72 ± 10.51 | 0.108 |
Initial admission SBP (mmHg), Median (IQR) | 123.00 (110.00, 132.50) | 120.00 (99.00, 135.00) | 125.00 (114.00, 131.50) | 0.181 |
Initial admission DBP (mmHg), Median (IQR) | 75.00 (68.00, 80.00) | 70.00 (66.00, 80.00) | 77.00 (70.00, 80.00) | 0.069 |
Lowest recorded MAP (mmHg), Median (IQR) | 85.67 (72.92, 92.33) | 76.67 (68.33, 86.67) | 87.00 (82.00, 93.33) | 0.001 |
Lowest recorded SBP (mmHg), Median (IQR) | 118.00 (97.00, 125.25) | 100.00 (90.00, 120.00) | 120.00 (110.00, 126.00) | 0.005 |
Lowest recorded MAP (mmHg), Median (IQR) | 70.00 (60.00, 76.00) | 62.00 (56.00, 70.00) | 71.00 (67.00, 78.00) | 0.001 |
Hypertension, n (%) | 52 (41.94) | 23 (46.94) | 29 (38.67) | 0.361 |
Coronary artery disease, n (%) | 23 (18.55) | 11 (22.45) | 12 (16.00) | 0.366 |
Myocardial infarction history, n (%) | 6 (4.84) | 3 (6.12) | 3 (4.00) | 0.912 |
Chronic kidney disease, n (%) | 54 (43.55) | 27 (55.10) | 27 (36.00) | 0.036 |
Hepatic dysfunction, n (%) | 21 (16.94) | 13 (26.53) | 8 (10.67) | 0.021 |
Hyperlipidemia, n (%) | 45 (36.29) | 16 (32.65) | 29 (38.67) | 0.496 |
Multiple myeloma, n (%) | 81 (65.32) | 33 (67.35) | 48 (64.00) | 0.702 |
Chemotherapy, n (%) | 98 (79.03) | 34 (69.39) | 64 (85.33) | 0.033 |
Autologous stem cell transplantation, n (%) | 14 (11.29) | 1 (2.04) | 13 (17.33) | 0.009 |
Organs with amyloid deposition | ||||
Renal involvement, n (%) | 102 (82.26) | 43 (87.76) | 59 (78.67) | 0.195 |
Soft tissue involvement, n (%) | 30 (24.19) | 10 (20.41) | 20 (26.67) | 0.426 |
Neurological involvement, n (%) | 38 (30.65) | 10 (20.41) | 28 (37.33) | 0.046 |
Gastrointestinal involvement, n (%) | 6 (4.84) | 4 (8.16) | 2 (2.67) | 0.334 |
Pulmonary involvement, n (%) | 3 (2.42) | 1 (2.04) | 2 (2.67) | 1.000 |
Hepatic involvement, n (%) | 25 (20.16) | 16 (32.65) | 9 (12.00) | 0.005 |
Total number of involved organs, Median (IQR) | 2.00 (2.00, 3.00) | 3.00 (2.00, 3.00) | 2.00 (2.00, 3.00) | 0.131 |
ECG features | ||||
Heart rate (bpm), Mean ± SD | 85.38 ± 14.05 | 84.82 ± 11.92 | 85.75 ± 15.35 | 0.720 |
PR interval (ms), Mean ± SD | 159.79 ± 26.32 | 165.59 ± 25.89 | 156.00 ± 26.08 | 0.047 |
P-wave duration (ms), Median (IQR) | 90.00 (84.00, 96.00) | 90.00 (86.00, 96.00) | 90.00 (82.00, 98.00) | 0.840 |
QRS duration (ms), Median (IQR) | 88.50 (82.00, 94.00) | 92.00 (84.00, 98.00) | 86.00 (82.00, 92.00) | 0.003 |
Corrected QT interval (ms), Median (IQR) | 428.00 (404.00, 450.50) | 435.00 (414.00, 471.00) | 425.00 (403.00, 443.50) | 0.079 |
QRS axis (degrees), Median (IQR) | 30.50 (-39.00, 58.00) | 4.00 (-54.00, 50.00) | 35.00 (-5.00, 59.00) | 0.094 |
R-wave amplitude in lead V5 (mV), Median (IQR) | 0.96 (0.58, 1.38) | 0.80 (0.34, 1.08) | 1.12 (0.68, 1.52) | 0.006 |
S-wave amplitude in lead V1 (mV), Median (IQR) | 0.65 (0.45, 0.93) | 0.65 (0.49, 0.92) | 0.63 (0.41, 0.94) | 0.838 |
Sokolow-Lyon index (mV), Median (IQR) | 1.46 (0.79, 2.52) | 1.23 (0.55, 2.48) | 1.56 (1.07, 2.54) | 0.073 |
R-wave in V5/S-wave in V1 ratio, Median (IQR) | 1.63 (1.13, 2.21) | 1.45 (0.98, 1.92) | 1.74 (1.21, 2.35) | 0.024 |
Minimum limb lead voltage (mV), Median (IQR) | 0.20 (0.15, 0.30) | 0.20 (0.10, 0.30) | 0.25 (0.20, 0.30) | 0.127 |
Maximum limb lead voltage (mV), Median (IQR) | 0.65 (0.45, 0.85) | 0.55 (0.45, 0.80) | 0.70 (0.50, 0.88) | 0.092 |
Mean limb lead voltage (mV), Median (IQR) | 0.45 (0.33, 0.59) | 0.42 (0.29, 0.56) | 0.47 (0.36, 0.60) | 0.149 |
QRS axis deviation (degrees), Median (IQR) | 35.00 (14.00, 84.00) | 68.00 (26.00, 99.00) | 32.00 (11.50, 61.50) | 0.009 |
Low Sokolow-Lyon index (< 1.5), n (%) | 56 (45.16) | 28 (57.14) | 28 (37.33) | 0.030 |
Low limb lead voltage, n (%) | 56 (45.16) | 30 (61.22) | 26 (34.67) | 0.004 |
Low precordial lead voltage, n (%) | 29 (23.39) | 17 (34.69) | 12 (16.00) | 0.016 |
Pseudo-infarct pattern, n (%) | 39 (31.45) | 21 (42.86) | 18 (24.00) | 0.027 |
Impaired R-wave progression, n (%) | 39 (31.45) | 21 (42.86) | 18 (24.00) | 0.027 |
Arrhythmias, n (%) | 31 (25.00) | 22 (44.90) | 9 (12.00) | < 0.001 |
Bradycardia and conduction abnormalities, n (%) | 33 (26.61) | 23 (46.94) | 10 (13.33) | < 0.001 |
T-wave abnormality, n (%) | 51 (41.13) | 28 (57.14) | 23 (30.67) | 0.003 |
ST-segment abnormalities, n (%) | 19 (15.32) | 8 (16.33) | 11 (14.67) | 0.802 |
Fragmented QRS complexes, n (%) | 12 (9.68) | 7 (14.29) | 5 (6.67) | 0.275 |
Atrial arrhythmias, n (%) | 20 (16.13) | 11 (22.45) | 9 (12.00) | 0.122 |
Ventricular arrhythmias, n (%) | 16 (12.90) | 14 (28.57) | 2 (2.67) | < 0.001 |
Left axis deviation, n (%) | 44 (35.48) | 23 (46.94) | 21 (28.00) | 0.031 |
Echocardiographic features | ||||
LV end-diastolic diameter (mm), Mean ± SD | 45.79 ± 5.36 | 45.71 ± 6.22 | 45.85 ± 4.75 | 0.901 |
LV end-systolic diameter (mm), Median (IQR) | 29.00 (26.75, 32.25) | 31.00 (29.00, 36.00) | 28.00 (26.00, 30.50) | < 0.001 |
LVEF (%), Median (IQR) | 64.00 (56.75, 69.00) | 56.00 (47.00, 65.00) | 66.00 (62.00, 71.00) | < 0.001 |
Left atrial anteroposterior diameter (mm), Mean ± SD | 38.86 ± 5.51 | 41.22 ± 4.57 | 37.32 ± 5.56 | < 0.001 |
Left atrial transverse diameter (mm), Mean ± SD | 40.74 ± 5.80 | 42.60 ± 4.51 | 39.53 ± 6.24 | 0.002 |
Left atrial longitudinal diameter (mm), Median (IQR) | 53.00 (49.00, 56.00) | 54.00 (53.00, 57.00) | 50.00 (46.00, 53.00) | < 0.001 |
Left atrial diameter index, Mean ± SD | 23.00 ± 3.58 | 24.59 ± 3.60 | 21.96 ± 3.18 | < 0.001 |
Right atrial transverse diameter (mm), Median (IQR) | 36.00 (33.00, 39.00) | 38.00 (36.00, 42.00) | 35.00 (32.50, 37.00) | < 0.001 |
Right atrial anteroposterior diameter (mm), Median (IQR) | 47.00 (44.00, 51.00) | 50.00 (47.00, 54.00) | 46.00 (42.85, 50.00) | < 0.001 |
Right ventricular diameter (mm), Median (IQR) | 33.00 (30.00, 37.00) | 35.00 (32.00, 38.00) | 31.00 (29.00, 36.00) | 0.002 |
LV mass (g), Median (IQR) | 201.33 (164.45, 257.14) | 216.55 (188.02, 262.62) | 189.19 (147.83, 243.88) | 0.007 |
LV mass index (g/m²), Median (IQR) | 118.96 (95.40, 150.37) | 130.87 (108.14, 158.08) | 106.72 (87.73, 143.43) | 0.005 |
Interventricular septal thickness (mm), Median (IQR) | 12.30 (10.00, 14.00) | 13.00 (11.30, 15.00) | 12.00 (10.00, 13.65) | 0.008 |
LV posterior wall thickness (mm), Median (IQR) | 11.40 (10.00, 13.00) | 12.00 (11.00, 15.00) | 11.00 (10.00, 12.40) | 0.002 |
LV relative wall thickness, Median (IQR) | 0.49 (0.42, 0.58) | 0.54 (0.43, 0.64) | 0.48 (0.40, 0.56) | 0.011 |
LV hypertrophy, n (%) | 65 (52.42) | 34 (69.39) | 31 (41.33) | 0.002 |
Aortic diameter (mm), Median (IQR) | 31.00 (29.00, 33.00) | 31.00 (29.00, 33.00) | 31.00 (29.50, 33.00) | 0.937 |
Ascending aorta diameter (mm), Mean ± SD | 33.45 ± 3.81 | 33.53 ± 3.86 | 33.40 ± 3.79 | 0.856 |
Pulmonary artery diameter (mm), Median (IQR) | 25.00 (24.00, 27.00) | 26.00 (24.00, 28.00) | 25.00 (23.00, 26.50) | 0.006 |
E-wave (m/s), Median (IQR) | 89.50 (73.00, 105.25) | 97.00 (84.00, 115.00) | 85.00 (66.50, 95.50) | < 0.001 |
A-wave (m/s), Mean ± SD | 81.94 ± 26.86 | 74.80 ± 29.10 | 86.60 ± 24.37 | 0.016 |
E/A ratio, Median (IQR) | 1.15 (0.76, 1.46) | 1.29 (1.14, 1.92) | 0.86 (0.73, 1.21) | < 0.001 |
Pericardial effusion (grade), Median (IQR) | 0.00 (0.00, 2.00) | 1.00 (0.00, 2.00) | 0.00 (0.00, 1.00) | < 0.001 |
Valvular regurgitation (grade), Median (IQR) | 2.00 (1.00, 2.00) | 2.00 (2.00, 3.00) | 2.00 (1.00, 2.00) | < 0.001 |
Valvular thickening, n (%) | 17 (13.71) | 6 (12.24) | 11 (14.67) | 0.701 |
Wall motion abnormality, n (%) | 25 (20.16) | 19 (38.78) | 6 (8.00) | < 0.001 |
LV systolic dysfunction, n (%) | 25 (20.16) | 21 (42.86) | 4 (5.33) | < 0.001 |
LV diastolic dysfunction, n (%) | 94 (75.81) | 39 (79.59) | 55 (73.33) | 0.426 |
Right ventricular systolic dysfunction, n (%) | 12 (9.68) | 8 (16.33) | 4 (5.33) | 0.087 |
Pulmonary arterial hypertension, n (%) | 34 (27.42) | 21 (42.86) | 13 (17.33) | 0.002 |
Combined ECG and echocardiographic parameters | ||||
Limb leads QRS score to LV wall thickness ratio, Median (IQR) | 0.23 (0.16, 0.33) | 0.20 (0.12, 0.30) | 0.24 (0.17, 0.34) | 0.011 |
Sokolow-Lyon index / LV posterior wall thickness, Median (IQR) | 1.50 (0.92, 2.05) | 1.23 (0.72, 1.68) | 1.73 (1.00, 2.22) | 0.004 |
Sokolow-Lyon index / LV relative wall thickness, Median (IQR) | 32.13 (20.68, 49.79) | 28.75 (15.05, 40.00) | 39.75 (23.04, 53.41) | 0.010 |
Sokolow-Lyon index / LV mass, Median (IQR) | 0.08 (0.05, 0.12) | 0.07 (0.04, 0.10) | 0.11 (0.06, 0.13) | 0.001 |
Voltage-to-mass ratio, Median (IQR) | 0.14 (0.09, 0.21) | 0.11 (0.07, 0.15) | 0.17 (0.10, 0.23) | 0.001 |
Laboratory examination | ||||
Cardiac troponin I (ng/mL), Median (IQR) | 0.06 (0.02, 0.21) | 0.12 (0.05, 0.25) | 0.03 (0.01, 0.11) | 0.002 |
BNP (pg/mL), Median (IQR) | 403.92 (133.52, 1339.66) | 1202.24 (407.20, 2986.00) | 202.00 (72.98, 724.00) | < 0.001 |
NT-proBNP (pg/mL), Median (IQR) | 3149.00 (849.83, 7746.75) | 6204.00 (3846.00, 13306.25) | 1537.50 (456.10, 4525.00) | < 0.001 |
Serum albumin (g/L), Median (IQR) | 32.90 (26.65, 36.52) | 33.60 (29.40, 36.50) | 32.30 (24.65, 36.60) | 0.297 |
Serum globulin (g/L), Median (IQR) | 25.50 (20.17, 32.85) | 27.50 (21.20, 32.40) | 24.00 (19.70, 32.90) | 0.252 |
Serum total protein (g/L), Median (IQR) | 59.80 (50.43, 69.60) | 62.30 (55.30, 68.80) | 59.00 (48.55, 70.10) | 0.342 |
Albumin-to-globulin ratio, Mean ± SD | 1.24 ± 0.54 | 1.22 ± 0.50 | 1.26 ± 0.57 | 0.690 |
Serum creatinine (µmol/L), Median (IQR) | 85.00 (65.40, 150.53) | 96.50 (71.90, 216.80) | 81.60 (63.90, 123.65) | 0.070 |
Creatinine clearance (mL/min), Median (IQR) | 71.10 (39.38, 93.36) | 68.92 (26.50, 79.18) | 74.42 (50.85, 96.12) | 0.068 |
β2-Microglobulin (mg/L), Median (IQR) | 4.83 (2.94, 7.35) | 5.30 (3.10, 9.86) | 4.14 (2.90, 6.29) | 0.037 |
ALT (U/L), Median (IQR) | 18.00 (13.00, 26.00) | 18.00 (12.00, 29.00) | 18.00 (13.50, 23.50) | 0.446 |
AST (U/L), Median (IQR) | 22.50 (17.00, 29.00) | 24.00 (19.00, 36.00) | 22.00 (16.00, 25.00) | 0.024 |
AST-to-ALT ratio, Median (IQR) | 1.27 (0.95, 1.55) | 1.33 (1.00, 1.57) | 1.21 (0.95, 1.52) | 0.421 |
Serum calcium (mmol/L), Median (IQR) | 2.16 (2.01, 2.29) | 2.16 (2.01, 2.28) | 2.17 (2.01, 2.31) | 0.988 |
Hemoglobin (g/L), Median (IQR) | 107.50 (83.50, 126.50) | 106.00 (82.00, 118.00) | 113.00 (84.50, 132.00) | 0.147 |
White blood cell count (×10⁹/L), Median (IQR) | 6.01 (5.00, 7.62) | 5.71 (4.98, 7.70) | 6.09 (5.08, 7.50) | 0.609 |
Platelet count (×10⁹/L), Median (IQR) | 198.50 (138.00, 272.00) | 168.00 (117.00, 236.00) | 213.00 (164.00, 283.50) | 0.014 |
Immunoglobulin G (mg/dL), Median (IQR) | 666.50 (414.25, 1072.50) | 769.00 (451.00, 1320.00) | 643.00 (378.50, 889.00) | 0.149 |
Immunoglobulin A (mg/dL), Median (IQR) | 62.90 (31.15, 132.25) | 53.30 (31.00, 93.40) | 68.40 (32.55, 202.50) | 0.138 |
Immunoglobulin M (mg/dL), Median (IQR) | 31.65 (20.12, 48.82) | 31.80 (19.20, 45.00) | 31.50 (22.25, 50.85) | 0.590 |
Immunoglobulin E (IU/mL), Median (IQR) | 18.50 (17.70, 40.10) | 18.50 (17.70, 42.80) | 19.40 (17.70, 39.00) | 0.739 |
Serum free light chain-λ (mg/dL), Median (IQR) | 318.50 (195.50, 588.25) | 413.00 (240.00, 715.00) | 269.00 (182.00, 440.00) | 0.002 |
Serum free light chain-κ (mg/dL), Median (IQR) | 450.00 (295.25, 617.50) | 402.00 (266.00, 526.00) | 492.00 (322.00, 653.00) | 0.022 |
Urine free light chain-λ (mg/dL), Median (IQR) | 5.44 (5.00, 27.63) | 16.50 (5.00, 48.40) | 5.00 (5.00, 14.50) | 0.005 |
Urine free light chain-κ (mg/dL), Median (IQR) | 3.98 (1.85, 16.83) | 4.20 (1.85, 16.90) | 3.51 (1.85, 15.75) | 0.985 |
Difference of free light chains (mg/dL), Median (IQR) | 248.50 (102.50, 536.75) | 244.00 (64.00, 542.00) | 249.00 (116.00, 504.00) | 0.354 |
Free light chain ratio, Median (IQR) | 1.92 (1.34, 3.13) | 1.72 (1.17, 3.79) | 2.01 (1.39, 3.07) | 0.200 |