Table 2.

Clinical characteristics of PKP2 mutation-positive and PKP2 mutation-negative arrhythmogenic right ventricular cardiomyopathy patients

	PKP2 mutation-positive n = 28	PKP2 mutation-negative n = 28	p
Male, n (%)	25 (89)	23 (82)	0.45
Age at diagnosis, years, mean (SD)	32 (11)	41 (12)	0.003
SCD in the young in the family, n (%)	4 (14)	4 (14)	1.0
ARVC in the family, n (%)	8 (29)	6 (21)	0.54
History of sports activity, n (%)	10 (36)	10 (36)	0.92
Cardiac arrest, n (%)	4 (14)	5 (18)	0.72
Syncope, n (%)	14 (50)	14 (50)	1.0
Sustained VT, n (%)	22 (79)	20 (71)	0.54
Nonsustained VT, n (%)	25 (89)	24 (86)	0.69
PVB > 500/day, n (%)	25 (89)	27 (96)	0.30
AF/AFL, n (%)	8 (29)	7 (25)	0.76
ICD, n (%)	20 (71)	15 (54)	0.17
Appropriate ICD interventions, n (%)	16 (57)	10 (36)	0.37
VT ablation, n (%)	14 (50)	15 (54)	0.79
Total ITFC punctation, mean (SD)	6.5 (1.5)	6.5 (1.7)	0.90
Negative T waves in leads V1–V3 (in the absence of RBBB), n (%)	21 (75)	12 (43)	0.01
QRS > 110 ms, n (%)	14 (50)	17 (61)	0.42
QRS dispersion, ms, mean (SD)	22 (22)	31 (19)	0.09
Epsilon wave, n (%)	6 (21)	21 (75)	< 0.001
Notched S in leads V1–V3, n (%)	15 (54)	13 (46)	0.31
RVOT PLAX, mm, mean (SD)	42 (8)	45 (12)	0.33
Regional RV akinesia or dyskinesia, n (%)	25 (89)	27 (96)	0.30
LVEF, %, mean (SD)	60 (7)	48 (20)	0.04
Heart failure symptoms, n (%)	6 (21)	15 (54)	0.01
RV damage progression, n (%)	19 (68)	19 (68)	0.83
LV damage progression, n (%)	5 (18)	12 (43)	0.04
Death or HTx, n (%)	3 (11)	11 (39)	0.03
Sudden death, n (%)	0 (0)	3 (11)	0.24
Death of heart failure or HTx, n (%)	3 (11)	8 (29)	0.18
Follow-up duration, years, mean (SD)	17.4 (9.1)	14.8 (8.0)	0.24

AF atrial fibrillation, AFL atrial flutter, HTx heart transplant, ICD implantable cardioverter-defibrillator, ITFC International Task Force Criteria, LV left ventricle, LVEF left ventricular ejection fraction, PLAX parasternal long-axis view, PVB premature ventricular beats, RBBB right bundle branch block, RV right ventricle, RVOT right ventricular outflow tract, VT ventricular tachycardia