Table 1

Clinical Characteristics at Inclusion of 117 Patients With AC, and Comparison of Patients Without and With Life-Threatening Ventricular Arrhythmia During Follow-Up

All (N = 117)No VA (n = 99)VA (n = 18)p ValueAdjusted HR (95% CI)p Value
Age, yrs40 ± 1740 ± 1 839 ± 160.89
BSA, m22.0 ± 0.22.0 ± 0.22.0 ± 0.20.52
Definite AC66 (56)52 (53)14 (78)0.05
Female58 (50)53 (54)5 (28)0.04
High-intensity exercise42 (37)28 (29)14 (82)<0.0014.2 (1.1–15.6)0.04
AA medication7 (6)2 (2)5 (28)<0.001
 Amiodarone2 (2)0 (0)2 (11)0.02
 Flecainide2 (2)1 (1)1 (6)0.29
 Sotalol3 (3)1 (1)2 (11)0.06
Beta-blocker36 (31)27 (27)9 (50)0.06
Mutation97 (83)86 (89)11 (65)0.02
 DSC2 (2)2 (2)0 (0)1.00
 DSG5 (5)5 (5)0 (0)1.00
 DSP3 (3)3 (3)0 (0)1.00
 PKP287 (90)76 (78)11 (65)0.26
Probands34 (29)20 (20)14 (78)<0.0015.0 (1.4–17.5)0.01
Syncope37 (32)25 (25)12 (67)0.0012.0 (0.7–6.0)0.20

Values are mean ± SD or n (%), unless otherwise stated. The p values are calculated by Student's t-test, chi-square test, or Fischer’s exact test as appropriate. Adjusted HR by multivariable Cox regression of possible confounders (p < 0.10).

AA = anti-arrhythmic; AC = arrhythmogenic cardiomyopathy; BSA = body surface area; DSC = desmocollin-gene; DSG = desmoglein-gene; DSP = desmoplakin-gene; NSVT = nonsustained ventricular tachycardia; PKP2 = plakophillin 2-gene; VA = life-threatening ventricular arrhythmia.

  • We restricted the multivariable analysis to only include the 3 parameters with the highest Harrell’s C-statistic from univariable Cox regression.