Characteristics of patients (n=63)
Characteristic | Value |
Sociodemographic | |
Male sex, n (%) | 36 (57) |
Age (years) | 48.0 (17.6) |
Genetics | |
Desmosomal mutations, n (%) | 23 (36.5) |
DSP | 10 (15.9) |
DSG2 | 5 (7.9) |
PKP2 | 8 (12.7) |
Non-desmosomomal mutations, n (%) | 29 (46) |
FLNC | 14 (22.2) |
DES | 8 (12.7) |
TMEM43 | 5 (7.9) |
LMNA | 2 (3.2) |
Gene elusive, n (%) | 11 (17.5) |
Phenotypic form | |
ARVC, n (%) | 24 (38.1) |
ALVC, n (%) | 32 (50.8) |
Biventricular AC, n (%) | 7 (11.1) |
Clinical features | |
Left ventricular ejection fraction, n (%) | |
>52% | 34 (54) |
≤52% | 29 (46) |
VT/VF history | 21 (33.3) |
ICD, n (%) | 36 (57) |
Appropriate ICD shocks history | 11 (17.5) |
Medications at baseline | |
Beta-blocker, n (%) | 38 (60) |
ACE inhibitor, n (%) | 21 (33) |
Sacubitril/valsartan, n (%) | 16 (25) |
Sotalol, n (%) | 9 (14) |
Amiodarone, n (%) | 0 (0) |
Physical activity | |
Total physical activity (min/day) | 290.2 (123.2) |
Moderate-to-vigorous intensity (min/day) | 20.9 (16.3) |
Ventricular tachycardias, n | |
Sustained VT | 0 |
RR-NSVT | 35 |
Mean (SD) unless otherwise stated.
AC, arrhythmogenic cardiomyopathy; ALVC, arrhythmogenic left ventricular cardiomyopathy; ARVC, arrhythmogenic right ventricular cardiomyopathy; DES, desmin; DSG2, desmoglein-2; DSP, desmoplakin; FLNC, filamin C; ICD, implantable cardioverter defibrillator; LMNA, lamin A/C; LVEF, left ventricular ejection fraction; PKP2, plakophilin-2; RR-NSVT, rapid-rate non-sustained ventricular tachycardia; TMEM43, transmembrane protein 43; VT/VF, ventricular tachycardia/ventricular fibrillation.