Table 1.
Baseline characteristics according to genotypes
| Overall (n = 577) | Gene negative (n = 358) | TTN (n = 81) | RBM20 (n = 22) | BAG3 (n = 20) | DSP (n = 17) | FLNC (n = 17) | MYH7 (n = 15) | TNNT2 (n = 14) | LMNA (n = 14) | MYBPC3 (n = 11) | DMD (n = 8) | P value* | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age (years) | 53.5 (±14.1) | 55.2 (±13.5) | 53.6 (±15.3) | 50.2 (±14.4) | 49.7 (±11.8) | 47.5 (±12.5) | 46.8 (±13.4) | 56.4 (±13.5) | 43.9 (±17.4) | 44.5 (±11.9) | 57.0 (±11.5) | 45.7 (±15.1) | <0.001 |
| Female sex | 196 (34.0%) | 113 (31.6%) | 26 (32.1%) | 13 (59.1%) | 5 (25%) | 12 (70.6%) | 8 (47.1%) | 8 (53.3%) | 5 (35.7%) | 5 (35.7%) | 0 (0%) | 2 (25.0%) | 0.66 |
| Clinical status | |||||||||||||
| NYHA | 0.03 | ||||||||||||
| NYHA I | 219 (38.0%) | 114 (31.8%) | 36 (44.4%) | 11 (50%) | 7 (35%) | 10 (58.8%) | 13 (76.5%) | 7 (46.7%) | 7 (50%) | 9 (64.3%) | 2 (18.2%) | 3 (37.5%) | |
| NYHA II | 178 (30.8%) | 129 (36.0%) | 14 (17.3%) | 5 (22.7%) | 7 (35%) | 4 (23.5%) | 3 (17.7%) | 5 (33.3%) | 2 (14.3%) | 2 (14.3%) | 5 (45.5) | 2 (25.0%) | |
| NYHA III | 154 (26.7%) | 97 (27.1%) | 27 (33.3%) | 6 (27.3%) | 5 (25%) | 3 (17.7%) | 1 (5.9%) | 3 (20%) | 2 (21.4%) | 3 (21.4%) | 4 (36.4%) | 2 (25.0%) | |
| NYHA IV | 26 (4.5%) | 18 (5.0%) | 4 (4.9%) | 0 (0%) | 1 (5%) | 0 (0%) | 0 (0%) | 0 (0%) | 2 (14.3%) | 0 (0%) | 0 (0%) | 1 (12.5%) | |
| NTProBNP (pg/mL) | 2637.6 (±4715.2) | 2962.9 (±5044.8) | 3282.7 (±5794.3) | 267.7 (±155.3) | 2315.1 (±3114.7) | 1166.0 (±1379.7) | 235.7 (±346.5) | 716.7 (±771.3) | 2540.6 (±3063.9) | 1326.9 (±1758.7) | 1042.1 (±684.0) | 2116.0 (±1474.4) | 0.04 |
| Skeletal muscle disease | 16 (2.8%) | 8 (2.2%) | 0 (0%) | 0 (0%) | 1 (5%) | 0 (0%) | 0 (0%) | 3 (20%) | 0 (0%) | 2 (14.3%) | 2 (18.2%) | 0 (0%) | 0.04 |
| Devices | |||||||||||||
| ICD | 161 (27.9%) | 89 (24.9%) | 19 (23.5%) | 6 (27.3%) | 8 (40%) | 9 (52.9%) | 8 (47.1%) | 4 (26.7%) | 2 (14.3%) | 9 (64.3%) | 4 (36.4%) | 3 (37.5%) | 0.01 |
| CRT | 63 (10.9%) | 51 (14.2%) | 4 (4.94%) | 2 (9.1%) | 2 (10%) | 0 (0%) | 0 (0%) | 0 (0%) | 0 (0%) | 3 (21.4%) | 1 (9.1%) | 0 (0%) | 0.05 |
| ECG | |||||||||||||
| AF | 46 (8.0%) | 34 (9.5%) | 6 (7.41%) | 1 (4.5%) | 0 (0%) | 2 (11.8%) | 0 (0%) | 0 (0%) | 0 (0%) | 1 (7.1%) | 2 (18.2%) | 0 (0%) | 0.06 |
| LBBB | 179 (31.0%) | 157 (43.9%) | 6 (7.41%) | 4 (18.2%) | 1 (5%) | 1 (5.9%) | 0 (0%) | 2 (13.3%) | 2 (14.3%) | 4 (28.6%) | 2 (18.2%) | 0 (0%) | <0.001 |
| Complete AVB | 55 (9.5%) | 38 (10.6%) | 8 (9.9%) | 1 (4.5%) | 1 (5%) | 0 (0%) | 0 (0%) | 1 (6.7%) | 1 (7.1%) | 5 (35.7%) | 0 (0%) | 0 (0%) | 0.56 |
| Abnormal TWI | 221 (38.3%) | 141 (39.4%) | 33 (40.8%) | 3 (13.6%) | 7 (35%) | 8 (47.1%) | 6 (35.3%) | 7 (46.7%) | 6 (42.9%) | 3 (21.4%) | 5 (45.5%) | 2 (25.0%) | 0.21 |
| Low QRS V limb leads | 60 (10.4%) | 18 (5.0%) | 14 (17.3%) | 0 (0%) | 4 (20%) | 7 (41.2%) | 6 (35.3%) | 1 (6.7%) | 3 (21.4%) | 3 (21.4%) | 3 (27.3%) | 1 (12.5%) | <0.001 |
| Low QRS V precordial leads | 15 (2.6%) | 6 (1.7%) | 3 (3.7%) | 0 (0%) | 0 (0%) | 1 (5.9%) | 3 (17.6%) | 0 (0%) | 0 (0%) | 0 (0%) | 1 (9.1%) | 1 (12.5%) | 0.13 |
Abbreviations: AF, atrial fibrillation; AVB, atrioventricular block; CRT, cardiac resynchronization therapy; ECG, electrocardiogram; ICD, implantable cardioverter defibrillator; LBBB, left bundle branch block; NYHA, New York Heart Association; TWI, T wave inversion; V, voltage.
* P value refers to overall analysis between groups.