Table 2.

Clinical comparisons between patients with CPVT1 by pathogenicity of RYR2 variant

	Pathogenic /probable pathogenic RYR2 variant	RYR2 variant of undetermined /unknown significance
	n = 90	n = 27
Male sex	42 (47%)	17 (63%)
Probands	60 (67%)	17 (63%)
Median age at diagnosis (years)	11.7 (8.0–14.7)	11.8 (9.8–14.9)
Median delay to diagnosis (years)	0.5 (0.1–4.1)	0.7 (0.1–4.1)
Syncope	30 (33%)	6 (22%)
Cardiac arrest	33 (37%)	10 (37%)
Atypical trigger for syncope	10 (11%)	3 (11%)
Atypical trigger for cardiac arrest	6 (7%)	2 (7%)
VA	69 (77%)	20 (74%)
Atrial arrhythmia	20 (22%)	6 (22%)
Beta-blocker failure	12 (13%)	3 (11%)
ICD use	46 (51%)	10 (37%)
Deaths	4 (4%)	0 (0%)