. Author manuscript; available in PMC: 2012 Mar 1.

Published in final edited form as: Card Electrophysiol Clin. 2011 Mar 1;3(1):23–45. doi: 10.1016/j.ccep.2010.10.012

Table 1.

Genetic disorders causing cardiac arrhythmias in the absence of structural heart disease (Primary Electrical Disease)

		Rhythm	Inheritance	Locus	Ion Channel	Gene

LQTS	(RW)	TdP	AD
	LQT1	(Andersen-Tawil Syndrome) (Timothy Syndrome)		11p15	I_Ks	KCNQ1, KvLQT1
	LQT2			7q35	I_Kr	KCNH2, HERG
	LQT3			3p21	I_Na	SCN5A, Na_v1.5
	LQT4			4q25		ANKB, ANK2
	LQT5			21q22	I_Ks	KCNE1, minK
	LQT6			21q22	I_Kr	KCNE2, MiRP1
	LQT7			17q23	I_K1	KCNJ2, Kir 2.1
	LQT8			6q8A	I_Ca	CACNA1C, Ca_v1.2
	LQT9			3p25	I_Na	CAV3, Caveolin-3
	LQT10			11q23.3	I_Na	SCN4B. Na_vb4
	LQT11			7q21-q22	I_Ks	AKAP9, Yotiao
	LQT12			20q11.2	I_Na	SNTA1, α–1 Syntrophin
	LQT13			11q24	IK-ACh	KCNJ5, Kir3.4

LQTS	(JLN)	TdP	AR	11p15	I_Ks	KCNQ1, KvLQT1
				21q22	I_Ks	KCNE1, minK

BrS	BrS1	PVT	AD	3p21	I_Na	SCN5A, Na_v1.5
	BrS2	PVT	AD	3p24	I_Na	GPD1L
	BrS3	PVT	AD	12p13.3	I_Ca	CACNA1C, Ca_V1.2
	BrS4	PVT	AD	10p12.33	I_Ca	CACNB2b, Ca_vβ_2b
	BrS5	PVT	AD	19q13.1	I_Na	SCN1B, Na_vβ1
	BrS6	PVT	AD	11q13–14	I_Ca	KCNE3. MiRP2
	BrS7	PVT	AD	11q23.3	I_Na	SCN3B, Navb3
	BrS8	PVT	AD	7q21.11	I_Ca	CACNA2D1, Ca_vα2δ

ERS	ERS1	PVT	AD	12p11.23	IK-ATP	KCNJ8, Kir6.1
	ERS2	PVT	AD	12p13.3	I_Ca	CACNA1C, Ca_V1.2
	ERS3	PVT	AD	10p12.33	I_Ca	CACNB2b, Ca_vβ_2b
	ERS4	PVT	AD	7q21.11	I_Ca	CACNA2D1, Ca_vα2δ

SQTS	SQT1	VT/VF	AD	7q35	I_Kr	KCNH2, HERG
	SQT2			11p15	I_Ks	KCNQ1, KvLQT1
	SQT3		AD	17q23.1–24.2	I_K1	KCNJ2, Kir2.1
	SQT4			12p13.3	I_Ca	CACNA1C, Ca_V1.2
	SQT5		AD	10p12.33	I_Ca	CACNB2b, Ca_vβ_2b

Catecholaminergic Polymorphic VT
	CPVT1	VT	AD	1q42–43		RyR2

	CPVT2	VT	AR	1p13–21		CASQ2

Abbreviations: AD, autosomal dominant; AR, autosomal recessive; BrS, Brugada syndrome; ERS, early repolarization syndrome; JLN, Jervell and Lange –Nielsen; LQTS, long QT syndrome; RW, Romano-Ward; SQTS, short QT syndrome; TdP, Torsade de Pointes; VF, ventricular fibrillation; VT, ventricular tachycardia.