Table 2.

Calmodulinopathy phenotypes and symptoms

CALM-phenotype	All carriers	Any cardiac events	MAE
	(n = 140)a	(n = 103)b,c	(n = 77)c,d
LQTS	74 (53)	47 (64)	36 (49)
CPVT	36 (26)	33 (92)	20 (56)
IVF	7 (5)	5 (71)	5 (71)
LQTS/CPVT overlap	10 (7)	7 (70)	6 (60)
UD	11 (8)	11 (100)	10 (91)
Atypical	2 (1)	-	-

Data are numbers and (%) of subjects.

LQTS, long QT syndrome; CPVT, catecholaminergic polymorphic ventricular tachycardia; IVF, idiopathic ventricular fibrillation; MAE, major arrhythmic events; UD, uncertain diagnosis.

Percentage out of all 140 CALM patients.

Arrhythmic syncope, ACA, SCD, and appropriate ICD shocks.

Percentage in the corresponding phenotype group.

ACA, SCD, and appropriate ICD shocks.