Table 1. Clinical and genetic characteristics of family BFC.

Code	Sex	Current age (y)	Diagnosis	Age at CPVT diagnosis	Genotype	Diagnostic criterion	SCD/RCA/VT	Syncope	ICD	App ICD Therapy	β-Blocker
I-2	F	92	Normal	NA	−/−	NA	No	No	No	NA	No
II-2	F	64	CPVT	23	+/−	Bidirectional VT on EST	Yes	Yes	Yes	Yes	Yes
II-3	M	Deceased aged 16	MVA	NA	NA	Unknown	NA	No	NA	NA	NA
II-4	M	62	Normal	NA	−/−	No	No	No	No	NA	No
II-7	F	61	CPVT	51	+/−	Polymorphic ectopy on EST	Yes	Yes	Yes	No	Yes
II-9	F	58	Normal	NA	−/−	No	No	No	No	No	No
III-1	F	41	Normal	NA	−/−	No	No	No	Yes	No	No
III-3	F	38	CPVT	31	+/−	Polymorphic ectopy on EST	Yes	Yes	Yes	Yes	Yes
III-5	M	34	Normal	NA	−/−	No	No	No	No	NA	No
III-11	M	33	Normal	NA	−/−	No	No	No	No	NA	No
III-13	F	32	CPVT	28	+/−	Polymorphic ectopy on EST	No	No	Yes	No	Yes
IV-1	F	Deceased aged 5	CPVT/SCD	5	+/−	Unexplained sudden death in CPVT family	Yes	No	No	No	No
IV-2	F	4	CPVT	3	+/−	Polymorphic ectopy on adrenaline challenge	No	No	No	NA	Yes
IV-6	F	1.5	Possible CPVT	NA	NA	Atrial arrhythmias on adrenaline challenge	No	No	No	NA	Yes

−/− = no variant identified; +/− = presence of Lysl80Arg variant; App ICD therapy = appropriate implantable cardioverter-defibrillator therapy; CPVT= catecholaminergic polymorphic ventricular tachycardia; EST= exercise stress test; F= female; ICD =implantable cardioverter-defibrillator; M = male; MVA = motor vehicle accident; NA = not applicable; RCA = resuscitated cardiac arrest; SCD = sudden cardiac death;