Table 1.
Baseline clinical characteristics
| Overall | Patients without sustained VA | Patients with sustained VA | P-value | |
|---|---|---|---|---|
| Total | 528 (100.0) | 382 (72.3) | 146 (27.7) | |
| Demographics | ||||
| Male sex | 236 (44.7) | 155 (40.6) | 81 (55.5) | 0.003 |
| Age at diagnosis (years) | 38.16 ± 15.47 | 39.73 ± 15.84 | 34.05 ± 13.67 | <0.001 |
| Caucasian ethnicity (n = 498) | 485 (91.9) | 348 (91.1) | 137 (93.8) | 0.064 |
| Proband status | 263 (49.8) | 151 (39.5) | 112 (76.7) | <0.001 |
| Pathogenic mutation (n = 504) | 340 (64.4) | 248 (64.9) | 92 (63.0) | 0.599 |
| PKP2 | 258 (48.9) | 185 (48.4) | 73 (50.0) | 0.582 |
| DSP | 23 (4.4) | 18 (4.7) | 5 (3.4) | |
| DSG2 | 17 (3.2) | 15 (3.9) | 2 (1.4) | |
| PLN | 26 (4.9) | 19 (5.0) | 7 (4.8) | |
| Multiple mutations | 6 (1.1) | 4 (1.0) | 2 (1.4) | |
| Other | 10 (1.9) | 7 (1.8) | 3 (2.1) | |
| History | ||||
| Symptoms | 307 (58.1) | 190 (49.7) | 117 (80.1) | <0.001 |
| Cardiac syncope | 107 (20.3) | 59 (15.4) | 48 (32.9) | <0.001 |
| Recent cardiac syncope (n = 519) | 48 (9.1) | 23 (6.0) | 25 (17.1) | <0.001 |
| ECG/continuous ECG monitoring | ||||
| TWI in ≥3 precordial leads (n = 517) | 298 (56.4) | 193 (50.5) | 105 (71.9) | <0.001 |
| TWI in ≥2 inferior leads (n = 506) | 85 (16.1) | 53 (13.9) | 32 (21.9) | 0.021 |
| NSVT (n = 470) | 231 (43.8) | 145 (38.0) | 86 (58.9) | <0.001 |
| 24 h PVC count (n = 425) | 1007 (278–3731) | 833 (125–2768) | 2782 (992–5918) | <0.001 |
| Imaging | ||||
| RVEF (%) (n = 510)a | 43.80 ± 10.40 | 45.40 ± 9.55 | 39.33 ± 11.37 | <0.001 |
| LVEF (%) (n = 515) | 57.66 ± 8.42 | 58.16 ± 8.00 | 56.34 ± 9.34 | 0.029 |
| Treatment at baseline | ||||
| ICD | 218 (41.3) | 136 (35.6) | 82 (56.2) | <0.001 |
| Beta-blockers (n = 511) | 200 (37.9) | 142 (37.2) | 58 (39.7) | 0.343 |
| Anti-arrhythmic drugs (n = 510) | 82 (15.5) | 50 (13.1) | 32 (21.9) | 0.019 |
Variables are expressed as frequency (%), mean ± standard deviation, or median (IQR). Total number of patients for a given variable mentioned if missing data.
DSG2, desmoglein-2; DSP, desmoplakin; ICD, implantable cardioverter-defibrillator; IQR, interquartile range; LVEF, left ventricular ejection fraction; NSVT, non-sustained ventricular tachycardia; PKP2, plakophilin-2; PLN, phospholamban; PVC, premature ventricular complex; RVEF, right ventricular ejection fraction; TWI, T-wave inversion; VA, ventricular arrhythmia.
a
RVEF estimation was based on quantitative measurement by CMR in 327 patients, by echocardiography in 160, by qualitative CMR assessment in 20, and by angiography in 3.