TABLE 1

Genes associated with the congenital long QT syndrome

Syndrome	Inheritance	Locus	Ion Channel/Protein	Gene	Frequency in Genotype + LQTS
LQT1	AD	11p15	I_Ks	KCNQ1	40–55%
LQT2	AD	7q35-q36	I_Kr	KCNH2	35–45%
LQT3	AD	3p21	I_Na	SCN5A	2–8%
LQT4	AD	4q25-q27	Ankyrin B	ANK2	<1
LQT5	AD	21q22	I_Ks	KCNE1	<1
LQT6	AD	21q22	I_Kr	KCNE2	<1
LQT7	AD	17q23	I_K1	KCNJ2	<1
LQT8	AD	12p13	I_Ca-L	CACNA1C	<1
LQT9	AD	3p25	Caveolin-3	CAV3	<1
LQT10	AD	11q23	NaV_1.5 β4	SCN4B	<0.1
LQT11	AD	7q21-7q22	Yotiao	AKAP9	<0.1
LQT12	AD	20q11	A1-syntrophin	SNTA1	<0.1
LQT13	AD	11q23-24	I_K-ACh	KCNJ5	<0.1
JLN1	AR	11p15	I_Ks	KCNQ1
JLN2	AR	21q22	I_Kr	KCNE1

AD, autosomal dominant; AR, autosomal recessive; LQT, long QT syndrome; JLN, Jervell and Lange-Nielsen syndrome.