Right Ventricular Functional Abnormalities in ARVC | 37 Table 1. Clinical characteristics and comparison per center All (n=160) Oslo (n=80) Utrecht (n=80) p-value Age, yrs 40.6 ± 16.8 40.9 ± 16.1 40.4 ± 17.6 0.87 Female, n(%) 88 (55) 44 (55) 44 (55) 1.00 Probands, n(%) 68 (43) 40 (50) 28 (35) 0.06 BSA, m2 1.9 ± 0.2 1.9 ± 0.2 1.9 ± 0.2 0.31 Definite AC, n(%) 97 (61) 47 (59) 50 (63) 0.63 AA medication, n(%) 44 (28) 12 (15) 32 (40) <0.001 Beta blockers, n(%) 60 (38) 27 (34) 33 (41) 0.33 Mutation, n(%) 139 (87) 59 (75) 80 (100) <0.001 PKP2, n(%) 124 (78) 53 (67) 71 (89) 0.001 ICD, n(%) 20 (14) 7 (9) 13 (19) 0.07 Syncope, n(%) 40 (25) 30 (38) 10 (13) <0.001 VA, n(%) 47 (29) 25 (31) 22 (28) 0.60 Disease stage, n(%) • Subclinical 36 (23) 15 (19) 21 (26) 0.26 • Electrical 38 (24) 25 (31) 13 (16) 0.03 • Structural 86 (54) 40 (50) 46 (58) 0.34 Values are, n (%), mean ± standard deviation. Abbreviations: AA = anti arrhythmic, AC = arrhythmogenic cardiomyopathy, BSA = body surface area, ICD = implantable cardioverter-defibrillator, PKP2 = plakophillin-2, VA = life-threatening ventricular arrhythmia. Ventricular arrhythmias Patients with VA were predominantly males (n = 28, 60%) and were significantly older (46.4 ± 13.9 vs. 38.2 ± 16.8 years, p = 0.002). Only two (2%) of the mutation-positive family members had experienced the arrhythmic outcome at evaluation (Table 2, analyses separated by center are enclosed in the supplementary material). Patients with VA had worse conventional echocardiographic parameters at inclusion, except for LVEF (Table 3). RV subtricuspid deformation patterns were similarly distributed within the two cohorts (Figure 2, panel A) and were more abnormal in patients with VA. Among VA patients, 46 (98%) had an abnormal RV deformation pattern type II (n = 16 (34%)) or type III (n = 30 (64%)). Odds for VA significantly increased with each step-up in type (OR 3.91, 95%CI (2.23 – 6.85), p <0.001), also when adjusted for age at outcome adjudication (OR 3.55, 95%CI (1.97 – 6.42), p <0.001). 3
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