Table 3 Clinical comparisons between patients with different gene mutations in pediatric patients with CPVT
RYR2 mutation (n = 54) | CASQ2 mutation (n = 11) | P value | |
|---|---|---|---|
Sex, males | 32/54 (60.4) | 6/11 (50.0) | 0.743 |
Age of onset, years | 8.3 ± 2.6 | 6.6 ± 1.4 | 0.048 |
Age at diagnosis, years | 11.0 ± 3.6 | 10.1 ± 3.7 | 0.453 |
Delay of diagnosis, years | 3.0 ± 3.1 | 3.4 ± 3.2 | 0.671 |
Positive family history | 8/34 (18.2) | 5/11 (45.5) | 0.035 |
Trigger | |||
Exercise | 23/30 (76.7) | 1/1 (100) | 0.750 |
Emotional stress | 19/28 (67.9) | 1/1 (100) | 0.357 |
Syncope | 54/54 (100) | 11/11 (100) | / |
Cardiac arrest | 8/17 (56.3) | 0/3 (0) | 0.145 |
Positive exercise testing | 39/39 (100) | 7/7 (100) | 0.309 |
Ventricular arrhythmia | 25/25 (100) | 5/5 (100) | / |
Sinus bradycardia | 15/54 (28.3) | 1/11 (9.1) | 0.179 |
Beta-blocker therapy | 0.301 | ||
Non-selective | 19/38 (50.0) | 3/9 (33.3) | |
Selective | 19/38 (50.0) | 6/9 (66.7) | |
Pacemaker | 2/39 (5.1) | 0/9 (0) | 0.657 |
ICD | 5/39 (10.2) | 1/9 (11.1) | 0.688 |
Deaths | 9/36 (23.1) | 1/10 (9.1) | 0.375 |