170 | Chapter 8 Table 1. Baseline characteristics of ARVC patients without and with sustained VA during follow-up (continued) All (n=150) No VA (n=113) VA (n=37) p-value NSVT 67 (45) 46 (41) 21 (57) 0.141 CMR (n = 141) RVEF, % 46 ± 10 48 ± 9 39 ± 10 <0.001 RVEDVi, ml 109 ± 29 105 ± 29 125 ± 24 0.002 LVEF, % 54 ± 9 54 ± 8 52± 10 0.387 LGE 23 (20) 16 (18) 7 (25) 0.419 Traditional echocardiography LVEF, % 55 ± 8 55 ± 7 53 ± 12 0.216 RVFAC, % 39 ± 9 40 ± 9 35 ± 8 0.017 RVOT, mm 35 ± 7 34 ± 6 37 ± 7 0.058 Echocardiographic deformation imaging LV GLS, % -18.2 ± 3.2 -18.7 ± 2.5 -16.7 ± 4.3 0.001 LVMD, ms 38(31 – 47) 38 (30 – 46) 42 (36 – 49) 0.107 RVFWLS, % -19.5 ± 7.0 -21.0 ± 6.3 -15.0 ± 6.9 <0.001 RVMD, ms 33 (23 – 44) 30 (21 – 43) 43 (29 – 67) 0.003 RV deformation pattern abnormal Type I Type II Type III 97 (66) 51 (35) 65 (44) 32 (22) 63 (56) 50 (45) 50 (45) 13 (12) 34 (94) 2 (6) 15 (42) 19 (53) <0.001 Values are n (%), mean ± standard deviation or median (25th, 75th percentiles) according to the distribution of normality. Abbreviations: ARVC = arrhythmogenic right ventricular cardiomyopathy, CMR = cardiac magnetic resonance, DSG2 = desmoglein-2, DSP = desmoplakin, EDVi = end diastolic volume indexed, EF = ejection fraction, FAC = fractional area change, GLS = global longitudinal strain, LGE = late gadolinium enhancement, LV = left ventricular, MD = mechanical dispersion, NSVT = non-sustained ventricular tachycardia, PKP2 = plakophillin-2, P/LP = pathogenic or likely pathogenic, PVC = premature ventricular complex, , RV = right ventricular, RVFWLS = right ventricular free wall longitudinal strain, RVOT = right ventricular outflow tract, TFC = task force criteria, TWI = T-wave inversion, VA = ventricular arrhythmia. Outcomes Thirty-seven patients (25%) experienced the primary outcome of first sustained VA after a median follow-up of 2.9 years (IQR 0.9 – 6.0) (Figure 2, see Supplemental Figure 2 for data per centre). The corresponding annual event rate of the primary endpoint was 3.6% (95% CI 2.6 – 5.0%). The most common primary endpoint was appropriate ICD therapy (n = 26, 70%), followed by sustained VA (n = 10, 27%) and sudden cardiac arrest (n = 1, 3%). At last follow-up, half of the patients (n = 75, 50%) had received an ICD. Eight (5%) patients had died of mostly non-cardiac cause (n = 6, 4%) and three (2%) had undergone heart transplantation.
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