Table 1.
Baseline characteristics, comparing patients with definite and borderline ARVC diagnosis.
| All Patients | Definite Diagnosis (N = 19) |
Borderline Diagnosis (N = 4) | p | |
|---|---|---|---|---|
| Age (years) | 53 (SD 13) | 53 (SD 12) | 56 (SD 17) | 0.65 |
| Sex (females) | 8 (35%) | 7 (37%) | 1 (25%) | 1 |
| Time from diagnosis to CT (years) | 4 (1–8) | 4.5 (2–10) | 2 (0.3–5) | 0.29 |
| Pathogenic or likely pathogenic variants | 10 (43%) | 9 (47%) | 1 (33%) | 1 |
| Gene * PKP2 DSG2 |
6 (60%) 4 (40%) |
5 (56%) 4 (44%) |
1 (100%) 0 (0%) |
|
| ICD | 21 (91%) | 18 (95%) | 3 (75%) | 0.32 |
| Primary prevention ICD | 7 (33%) | 5 (28%) | 2 (67%) | 0.25 |
| Any sustained VA or aborted SD | 16 (70%) | 15 (79%) | 1 (25%) | 0.07 |
| ECG parameters | ||||
| QRS duration (ms) | 109 (SD 17) | 107 (SD 10) | 121 (SD 34) | 0.13 |
| Number of negative T waves precordial leads | 3.3 (SD 1.8) | 3.7 (SD 1.7) | 1.3 (SD 0.5) | 0.01 |
| Epsilon wave | 11 (48%) | 10 (53%) | 1 (25%) | 0.59 |
| Terminal QRS activation(ms) | 57 (SD 16) | 57 (SD 13) | 55 (SD 30) | 0.8 |
| Imaging | ||||
| TAPSE (mm) | 18 (SD 5) | 17 (SD 5) | 21 (SD 2) | 0.17 |
| LVEF (%) | 56 (SD 9) | 56 (SD 9) | 54 (SD 9) | 0.62 |
| iRVEDV (mL/m2) | 132 (SD 35) | 137 (SD 36) | 108 (SD 22) | 0.13 |
| iRVESV (mL/m2) | 85 (SD 33) | 90 (SD 34) | 63 (SD 20) | 0.09 |
| RVEF (%) | 37 (SD 10) | 35 (SD 10) | 43 (SD 9) | 0.21 |
| RV LGE ƚ | 6 (38%) | 5 (38%) | 1 (33%) | 1 |
| ADAS scar parameters | ||||
| RV scar area (cm2) | 102 (SD 37) | 108 (SD 35) | 77 (SD 37) | 0.13 |
| RV BZ area (cm2) | 42 (SD 14) | 44 (SD14) | 33 (SD 14) | 0.2 |
| RV dense scar area (cm2) | 61 (SD28) | 64 (SD 28) | 44 (SD 26) | 0.19 |
| Percentage of RV scar | 34% (SD 10) | 36% (SD9) | 27% (SD 10) | 0.11 |
| Percentage of RV BZ | 14% (SD 3) | 14% (SD 2) | 12% (SD 4) | 0.13 |
| Percentage of RV dense scar | 20% (SD 9) | 22% (SD 9) | 15% (SD8) | 0.2 |
CT, computed tomography; ICD, implantable cardioverter defibrillator; TAPSE, Tricuspid Annular Plane Systolic Excursion; LVEF, left ventricular ejection fraction; iRVEDV, indexed right ventricular end-diastolic volume; iRVESV, indexed right ventricular end systolic volume; RVEF, right ventricular ejection fraction; RV, right ventricular; LGE, late gadolinium enhancement; BZ, border zone. * Gene hosting the pathogenic or likely pathogenic variant. ƚ Information available for 16 patients who had CMR with LGE analysis.