Table 1.
Clinical characteristics of patients at diagnosis of definite ARVC
| Case | Age, years | Sex | BSA, m2 | Prediagnosis | Gene mutation | ICD placement | TFC-2010 |
Imaging (CMR) |
Endomyocardial biopsy |
Repolarization abnormalities |
Depolarization abnormalities |
Arrhythmia |
Family history of ARVC |
|||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Major | Minor | Major | Minor | Major | Minor | Major | Minor | Major | Minor | Major | Minor | Major | Minor | |||||||
| 1 | 12 | M | 1.38 | VSD | PKP2 | Yes | 3 | 1 | +, Echo | - | Not performed | + | – | – | – | – | + | + | – | |
| 2 | 17 | M | 1.89 | Myocarditis | negative | Yes | 3 | 1 | +, Echo | – | – | – | – | + | + | – | + | – | – | – |
| 3 | 18 | M | 1.99 | Myocarditis | PKP2 | Yes | 3 | 1 | +, Echo | – | Not performed | + | – | – | – | – | + | + (Mother) | – | |
| 4 | 15 | M | 2.38 | VT | PKP2 | Yes | 2 | 2 | +, Echo | – | Not performed | – | – | – | + | – | + | + | – | |
| 5 | 17 | F | 1.95 | NA | PKP2 | Yes | 3 | 1 | + | Echo | Not performed | + | – | – | – | – | + | + | – | |
| 6 | 16 | M | 1.87 | NA | PKP2 | Yes | 3 | 2 | +, Echo | – | Not performed | + | – | – | + | – | + | + | – | |
| 7 | 15 | M | 2.04 | NA | PKP2 | Yes | 3 | 0 | +, Echo | – | Not performed | + | – | – | – | – | – | + | – | |
| 8 | 18 | M | 1.97 | VT | LDB3 | Yes | 1 | 2 | –, Echo | – | Not performed | – | + | – | – | – | + | – | – | |
| 9 | 18 | F | 1.99 | NSVT | PKP2 | No | 1 | 2 | – | – | Not performed | – | + | – | – | – | + | + (Mother) | – | |
| 10 | 13 | M | 1.33 | NA | NA | No | 2 | 1 | +, Echo | – | Not performed | + | – | – | – | – | + | – | – | |
BSA, body surface area; F, female; M, male; NA, not applicable; NSVT, nonsustained VT.