Table 1:
Baseline characteristics of ARVC patients at the time of ARVC diagnosis. Continuous variables are presented as mean ± standard deviation or median [IQR], as appropriate.
| Variable (Patients with available data at diagnosis) | All Patients (n=408) | Absence of VA event (276) | Occurrence of VA event (132) | p value |
|---|---|---|---|---|
| Age at Diagnosis (n=408) | 37 (±15.1) | 38 (±15.8) | 33 (±12.6) | <0.001 |
| Male Sex (n=408) | 164 (40.2%) | 96 (34.8%) | 68 (51.5%) | 0.001 |
| Caucasian Race (n=407) | 397 (97.5%) | 269 (97.5%) | 128 (97.7%) | 0.881 |
| Proband (n=408) | 240 (58.8%) | 135 (48.9%) | 105 (79.5%) | <0.001 |
| Pathogenic / likely pathogenic variant (n=405) | 298 (73.6%) | 211 (77.0%) | 87 (66.4%) | 0.024 |
| PKP2 | 197 (48.6%) | 133 (48.5%) | 64 (48.9%) | 0.953 |
| DSP | 13 (3.2%) | 10 (3.6%) | 3 (2.3%) | 0.468 |
| DSG2 | 11 (2.7%) | 5 (1.8%) | 6 (4.6%) | 0.111 |
| PLN | 27 (6.7%) | 20 (7.3%) | 7 (5.3%) | 0.46 |
| Other | 13 (3.2%) | 7 (2.6%) | 6 (4.6%) | 0.279 |
| Symptoms* (n=361) | 232 (64.3%) | 131 (55.3%) | 101 (81.5%) | <0.001 |
| History of Cardiac Syncope (n=408) | 77 (18.9%) | 39 (14.1%) | 38 (28.8%) | <0.001 |
| Anterior T-wave inversions (n=398) | 3 [2.0; 4.0] | 3 [1.0; 4.0] | 3 [3.0; 4.0] | <0.001 |
| Inferior T-wave inversions (n=387) | 0 [0.0; 1.0] | 0 [0.0; 1.0] | 0 [0.0; 1.0] | 0.028 |
| Total T-wave inversions (ant.+inf.) (n=387) | 3 [2.0; 5.0] | 3 [2.0; 4.0] | 4 [3.0; 5.0] | <0.001 |
| 24 hr. PVC count (n=343) | 1186 [361; 4095] | 860 [183; 2751] | 2879 [1151; 60785] | <0.001 |
| Presence of NSVT (n=370) | 195 (52.7%) | 114 (44.4%) | 81 (71.7%) | <0.001 |
| RVEF (%) (n=348) | 44 (±10.1) | 45 (±8.8) | 40 (±11.6) | <0.001 |
| LVEF (%) (n=355) | 58 (±8.0) | 58 (±7.9) | 57 (±8.3) | 0.304 |
| ICD at any point (n=407) | 277 (68.1%) | 150 (54.5%) | 127 (96.2%) | <0.001 |
| ICD prior to dx. | 18 (4.4%) | 16 (5.8%) | 2 (1.5%) | 0.049 |
| ICD within 6 mo. of dx. | 129 (31.6%) | 59 (21.4%) | 70 (53.0%) | <0.001 |
| ICD arrhythmia monitoring zone cycle length (ms) | 350 [323, 400] | 350 [328, 375] | 351 [322, 400] | 0.520 |
| ICD arrhythmia treatment zone cycle length (ms) | 300 [286, 320] | 300 [285, 316] | 300 [289, 333] | 0.017 |
| Baseline anti-arrhythmic prescription (n=391) | 59 (15.1%) | 36 (13.5%) | 23 (18.4%) | 0.21 |
| Amiodarone prescription | 8 (2%) | 5 (2%) | 3 (2%) | 0.753 |
| Sotalol prescription | 45 (11%) | 27 (10%) | 18 (14%) | 0.245 |
| Baseline beta-blocker prescription (n=392) | 153 (39.0%) | 96 (36.0%) | 57 (45.6%) | 0.068 |
| ARVC calculator predicted 5-year VA risk (%) | 29 (±23%) | 21 (±19%) | 45 (±23%) | <0.001 |
| Observed 5-year VA risk (%) | 29% [95%CI: 24, 34] | --- | --- |