Table 2 The summary of clinical features of Chinese pediatric patients with CPVT

Sex, males

56/95 (58.9)

Age of onset, years (n = 90)

8.7 ± 3.0 (3.0–20.0)

Age at diagnosis, years (n = 92)

12.9 ± 6.8 (2.1–45.0)

Delay of diagnosis, years (n = 87)

4.3 ± 6.6 (0.0–36.0)

Positive family history (n = 62)

16/62 (25.8)

Trigger

 Exercise, n (%) (n = 53)

41/53 (77.4)

 Emotional stress, n (%) (n = 51)

32/51 (62.7)

Syncope, n (%) (n = 95)

94/95 (98.9)

Cardiac arrest, n (%) (n = 34)

13/34 (38.2)

Genetic results, n (%) (n = 67)

 RYR2

47/67 (70.1)

 CASQ2

11/67 (16.4)

 RYR2 + SCN5A

7/67 (10.4)

 Tecrl

1/67 (1.4)

 LGTs

1/67 (1.4)

RYR2 hotspot, n% (n = 29)

 I

5/29 (17.2)

 II

7/29 (24.1)

 III

6/29 (20.7)

 IV

8/29 (27.6)

 Non-spot

3/29 (10.3)

RYR2 domain, n% (n = 29)

 NTD

7/29 (24.1)

 SPRY1

1/29 (3.4)

 HD1

7/29 (24.1)

 Central domain

3/29 (10.3)

 U-motif

3/29 (10.3)

 VSC

2/29 (6.9)

 S6

2/29 (6.9)

 CTD

4/29 (13.8)

Positive exercise testing, n (%) (n = 67)

66/67 (98.5)

VT after exercise test, n (%) (n = 49)

 pVT

9/49(22.4)

 bVT

11/49 (18.4)

 pVT and bVT

29/49 (59.2)

Sinus bradycardia, n (%) (n = 95)

20/95 (22.1)

First-line therapy

Beta-blocker therapy, n (%) (n = 67)

 Non-selective

29/67 (43.3)

 Selective

38/67 (56.7)

Beta-blocker therapy in detail, n (%)

 Nadolol, n (%)

8/67 (11.9)

 Metoprolol, n (%)

36/67 (53.7)

 Propranolol, n (%)

21/67 (31.3)

 Bisoprolol, n (%)

2/67 (3.0)

Prognosis after beta-blocker therapy, n (%) (n = 47)

 Remission, n (%)

20/47 (42.5)

 Recurrent scope, n (%)

24/47 (51.1)

 Death, n (%)

3/47 (6.4)

Rescue therapy, n (%) (n = 33)

 Dosage

9/33 (27.3)

 Beta-blocker and other drugs, n (%)

10/33 (30.3)

 Beta-blocker and LCSD, n (%)

5/33 (15.1)

 Beta-blocker and ICD, n (%)

9/33 (27.3)

Follow-up duration, months (n = 51)

17.3 ± 23.6

Pacemaker, n (%)

4/68 (5.9)

Outcome, n (%) (n = 75)

 Remission, n (%)

65/75 (86.7)

 Deaths, n (%)

10/75 (13.3)