Table 1.

Clinical Characteristics of the LQTS Subjects.

Gene	Mutation	LQTS Type	ID	Age (YRS.)	Sex	QTc (ms)	Cardiac Events (Symptoms)	Therapy
KCNQ1	340delF	LQT1	14	40	F	485	none (found during family screen)	Toprolol
KCNQ1	340delF	LQT1	15	11	F	541	none (found during family screen)	Nadolol
KCNQ1	G26D	LQT1	19	23	F	458	VF and appropriate ICD shock	ICD*
KCNQ1	T312I	LQT1	20	59	F	520	none (found during family screen)	None
KCNQ1	T312I	LQT1	21	37	F	488	several episodes of syncope (index case for family)	Propranolol
KCNQ1	T312I	LQT1	23	7	M	563	none (found during family screen)	Atenolol
KCNQ1	Q359X	LQT1	18	39	F	516	none (found during family screen)	Metoprolol + ICD*
KCNQ1	Unknown	LQT1	13	16	F	495	none (found during family screen)	Nadolol
KCNQ1	Unknown	LQT1	24	22	F	481	none (found during family screen)	Nadolol
KCNH2	H70R	LQT2	3	28	M	469	none (found during family screen)	Nadolol + ICD*
KCNH2	H70R	LQT2	6	51	F	503	palpitations at rest	Atenolol + ICD*
KCNH2	Q376sp	LQT2	1	11	M	558	presycope (index case for family)	Nadolol
KCNH2	Q376sp	LQT2	4	39	F	463	presyncope	Nadolol
KCNH2	Q376sp	LQT2	5	10	M	481	none (found during family screen)	Nadolol
KCNH2	S783P	LQT2	25	27	F	493	several episodes of syncope	Nadolol
KCNH2	V796G	LQT2	11	33	F	488	several episodes of VF (index case for family)	Metoprolol + ICD*
KCNH2	Y652X	LQT2	16	46	M	480	syncope and seizure	Lopressor
KCNH2	Y652X	LQT2	17	16	M	500	none (found during family screen)	Nadolol
SCN5A	E1784K	LQT3	2	20	M	500	presyncope	ICD*
SCN5A	E1784K	LQT3	7	34	M	478	VF and appropriate shock	ICD*
SCN5A	E1784K	LQT3	12	59	M	533	none (found during family screen)	ICD*
SCN5A	R1644H	LQT3	8	36	F	496	palpitations and syncope (index case for family)	Metoprolol + ICD*
SCN5A	R1644H	LQT3	22	8	M	543	none (found during family screen)	Nadolol
KCNE1	D76N	LQT5	9	35	F	486	none (found during family screen)	ICD*
KCNE1	D76N	LQT5	10	58	F	500	syncope	ICD*

^*The patients with ICD were not paced. The average duration of ICD implant was 5 ± 3 years at the time of the ECGI study