Clinical data of CPVT probands
| RYR2 mutation | Gender | Age of onset,years | Penetr-ance, % | Resting ECG pre med,bpm (age,years) | Median HR age of group9 | Deviat-ion,bpm | QTc,ms | Symptoms | Exercise test VT thres-hold,bpm | Exercise test ECG characteristics | Follow up,years | Origin | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| *Median (range); †mean (SD). | |||||||||||||
| BG, monomorphic bigeminy; CA, cardiac arrest; MPVB, monomorphic premature ventricular beat; NA, not available; PC, polymorphic couplets; PMVT, polymorphic ventricular tachycardia; PPVB, polymorphic premature ventricular beat; SPVT, sustained polymorphic ventricular tachycardia; VT, ventricular tachycardia; X syncope, exercise induced syncope. | |||||||||||||
| Family 4 | F4020L | M | 4 | NA | 70 (4) | 98 | 28 | 410 | X syncope,seizure | 150 | BG, PC, PMVT | Suddendeathat 20 | French |
| Family 8 | H4108N | F | 4 | De novo | 50 (14) | 73 | 23 | 374 | X syncope,seizure,CA | 90 | BG, PC, PMVT | 2 | Dutch |
| Family 9 | H4108Q | F | 6.5 | De novo | 70 (7) | 89 | 19 | 400 | X syncope | 140 | MPVB, PMVT | 12 | French |
| Family 7 | N4104I | M | 7 | 100 | 60 (8) | 88 | 28 | 400 | X syncope,seizure | 130 | PPVB, SPVT | 2 | French |
| Family 2 | A2254V | F | 8 | 100 | 66 (5) | 84 | 18 | 410 | CA,exercise VT | 135 | BG, PC | 22 | Dutch |
| Family 1 | E1724K | F | 9 | 100 | 43 (12) | 78 | 35 | 380 | X syncope | 110 | BG, PMVT | 15 | Dutch |
| Family 3 | A2394G | F | 9 | 100 | 60 (10) | 78 | 18 | 440 | X syncope,seizure,CA | 140 | MPVB, PMVT | 6 | French |
| Family 6 | E4076K | F | 10 | 66 | 58 (14) | 76 | 18 | 405 | X syncope | 110 | BG, PMVT | 3 | Dutch |
| Family 11 | V4771I | F | 12 | NA | – | – | – | – | X syncope,seizure | 130 | PPVB, PMVT | 7 | French |
| Family 12 | P4902S | F | 13 | 65 | 60 (13) | 76 | 16 | 414 | X syncope | 120 | PPVB, PMVT | 6 | FrenchPolynesia |
| Family 10 | H4762PG4662S | F | 13 | Com-pound | – | – | – | 410 | X syncope | 60 W | BG, PMVT | 28 | French |
| Family 5 | E4076K | M | 51 | 64 | 73 (17) | 70 | −3 | 351 | X syncope,seizure | 110 | MPVB, PC,PMVT | 4 | French |
| 9 (4–51)* | 20(10)† | 399(24)† | 124(18)† | 6 (2–28)* | |||||||||