Electromechanical Substrate Characterization in ARVC | 67 Table 1. Clinical characteristics Controls (n = 20) Subclinical Stage (n = 18) Electrical Stage (n = 15) Structural Stage (n = 35) Age, yrs 28 ± 6 27±14 40±17$ 44±16^ p<0.001 Female 9 (45%) 11 (61%) 12 (80%) 18 (51%) BSA, m2 1.87±0.17 1.80±0.21 1.84±0.17 1.91±0.22 p=0.280 Probands 0 (0%) 0 (0%) 0 (0%) 18 (53%) AC diagnosis 0 (0%) 0 (0%) 5 (33%) 35 (100%) 2010 TFC (major or minor) Structural criteria 0 (0%) 0 (0%) 35 (100%) Depolarization criteria 1 (6%) 12 (80%) 17 (49%) Repolarization criteria 1 (6%) 3 (20%) 20 (57%) Arrhythmia criteria 0 (0%) 5 (33%) 32 (91%) Mutations PKP2 mutation 15 (83%) 14 (93%) 32 (91%) DSG2 mutation 3 (17%) 1 (7%) 2 (6%) DSP mutation 0 (0% 0 (0%) 1 (3%) CMR CO, L/min/m2 3.7±0.6 3.3±0.5 3.5±0.6 3.2±0.8 p=0.041 RV-EDVi, mL/m2 108±17 87±17 94±12 132±41* p<0.01 LV-EDVi, mL/m2 98±14 85±13 93±14 91.8±15 p=0.063 RVEF, % 54±6 56±8 51±6 36±11* p<0.001 LVEF, % 61±7 58±4 57±7 56±9 p=0.182 Presence of LGE 0 (0%) 0 (0%) 0 (0%) 20 (57%) Echocardiography HR 62±10 67±16 63±11 58±9 p=0.088 LV-GLS, % -21.1±1.7 -20.0±1.7 -19.0±3.0 -17.8±3.3* p<0.001 LVEF, % 60.6±6.9 58.4±4.3 56.6±6.8 55.8±9.2 p=0.159 RV-GLS, % -27.6±4.0 -25.4±3.6 -21.7±4.7$ -15.0±5.6* p<0.001 RV basal deformation pattern Type-I 20 (100%) 12 (67%) 4 (27%) 2 (6%) Type-II 0 (0%) 6 (33%) 8 (53%) 10 (29%) Type-III 0 (0%) 0 (0%) 3 (20%) 23 (66%) RVMD, ms - 16.4±7.5^ 32.2±16.1 50±27 p<0.01 AC: Arrhythmogenic Cardiomyopathy; TFC: Task Force Criteria; PKP2: plakphilin-2; DSG2: desmoglobin-2; DSP: desmoplakin; CMR: cardiovascular magnetic resonance imaging; CO: cardiac output; RV: right ventricle; LV: left ventricle; EDVi: end diastolic volume indexed for BSA; RVEF: right ventricular ejection fraction; LVEF: left ventricular ejection fraction; LGE: late gadolinium enhancement; HR: Heart rate; GLS: global longitudinal strain; RVMD: right ventricular mechanical dispersion; *: p<0.05 structural stage versus all other groups (Bonferroni correction) ; ^: p<0.05 structural stage versus control group and subclinical stage; $: p<0.05 electrical stage versus control 4
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