Table 1. Baseline Patient Characteristics.
Characteristic | Data (N = 169)a |
---|---|
Age, mean (SD), y | 36.3 (15.0) |
Sex | |
Men | 95 (56.2) |
Women | 74 (43.8) |
Proband status | 128/169 (75.7) |
2010 TFC fulfillment | |
Class I: morphology | |
Major | 78 (46.2) |
Minor | 35 (20.7) |
Class II: tissue characterization | |
Major | 27 (16.0) |
Minor | 5 (3.0) |
Class III: repolarization abnormalities | |
Major | 89 (52.7) |
Minor | 50 (29.6) |
Class IV: depolarization abnormalities | |
Major | 9 (5.3) |
Minor | 46 (27.2) |
Class V: arrhythmias | |
Major | 31 (18.3) |
Minor | 101 (59.8) |
Class VI: family history | |
Major | 96 (56.8) |
Minor | 13 (7.7) |
Pathogenic/likely pathogenic variation | 85 (50.3) |
PKP2 | 54 (32.0) |
DSP | 19 (11.2) |
DSG2 | 8 (4.7) |
DES | 2 (1.2) |
FLNC | 2 (1.2) |
Recent cardiac syncope | 24 (14.2) |
TWI, median (IQR) | 3 (2-4) |
NSVT at diagnosis | 61 (36.1) |
24-h PVC count, median (IQR) | 5852 (4409-7295) |
History of sustained ventricular tachycardia at diagnosis | 47 (27.8) |
RVEF at CMR, mean (SD), % | 46.0 (12.2) |
Treatment at baseline | |
β-Blockers | 86 (50.9) |
AADs | 39 (23.1) |
Sotalolo | 36 (21.3) |
Class Ic | 6 (3.5) |
Amiodarone hydrochloride | 3 (1.8) |
ICD | 73 (43.2) |
Abbreviations: AADs, antiarrhythmic drugs; CMR, cardiac magnetic resonance; DES, desmin; DSG2, desmoglein 2; DSP, desmoplakin; FLNC, filamin C; ICD, implantable cardioverter-defibrillator; NSVT, nonsustained ventricular tachycardia; PKP2, plakophylin 2; PVC, premature ventricular complex; RVEF, right ventricular ejection fraction; TFC, Task Force Criteria; TWI, T-wave inversion.
Unless otherwise indicated, data are expressed as number (%) of patients.