Table 1.

Genotype status of all the genotype‐positive patients from the eight families with AR LQT1 patients. Clinical symptoms (if any), QTc interval, pharmacological intervention (if any), and other relevant parameters of the AR LQT1 patients as well as the heterozygote family members are also mentioned

Family numbers	ID, age(yrs), gender	Age at first presentations (yrs)	Symptoms	QTc (ms)	Genotype	Medication	Response to medication
A	II:4, 3, m		Syncope	557	c.[387‐5 T>A]; [387‐5 T>A] p. [?]; [?]	Atenolol (12.5 mg bd)	Well response
	II:1, m		n.s	430	c.[387‐5 T>A]; [=] p. [?]; [=]	n.m
	I:1, m		n.s	459	c.[387‐5 T>A]; [=] p. [?]; [=]	n.m
	I:2, f		n.s	431	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
B	II:5, 16, m	1	Seizure attacks	529	c.[387‐5 T>A]; [387‐5 T>A] p. [?]; [?]	Propranolol (20 mg tid)	Well response
	II:3, m		n.s	n.a	c.[387‐5 T>A]; [=] p. [?]; [?]	n.m
	I:1, m		n.s	n.a	c.[387‐5 T>A]; [=] p. [?]; [=]	n.m
C	II:1, 16, m			500	c.[387‐5 T>A]; [387‐5 T>A] p. [?]; [ ?]
	II:2, 9, m		Dizziness	459	c.[387‐5 T>A]; [387‐5 T>A]  p. [?]; [?]	Inderal
	II:3, 8, f		n.s	498	c.[387‐5 T>A]; [387‐5 T>A]  p. [?]; [?]	Inderal
	I:1, 44, m		n.s	444	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
	I:2, 44, f		n.s	467	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
D	II:2,9, f		Episode of cardiac arrest	537	c.[387‐5 T>A]; [387‐5 T>A]  p. [?]; [?]	Propranolol (20 mg tds),
	II:1, 15, m		Bradycardia	435	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
	II:4, 2, f		n.s	474	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
	I:1, m		Bradycardia	404	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
	I :2, f		Bradycardia	457	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
E	II:1, 1, f	Since birth	Bradycardia	531	c.[387‐5 T>A]; [387‐5 T>A]  p. [?]; [?]	Propranolol (1 mg/kg)	Well response
	I:1, 32, m		n.s	399	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
	I:2, 25, f		Bradycardia	418	c.[387‐5 T>A]; [=]  p. [?]; [=]	n.m
F	II:4, 12, m	3	Palpitation, seizures, chest pain, loss of consciousness	534	c.[535G>A]; [535G>A] p.[Gly179Ser]; [Gly179Ser]	B. blocker (2 mg/kg/bd)	Well response
	II:3, 16, m		n.s	444	c.[535G>A]; [535G>A] p.[Gly179Ser]; [Gly179Ser]	B. blocker (2 mg/kg/bd)
	II:5, 8 mos, m		n.s	496	c.[535G>A]; [535G>A] p.[Gly179Ser]; [Gly179Ser]	B. blocker (2 mg/kg/bd)
	III:1, 6, m		n.s	448	c.[535G>A]; [=] p.[Gly179Ser]; [=]	ICD implanted
	I:2, 60, m		n.s	467	c.[535G>A]; [=] p.[Gly179Ser]; [=]	n.m
G	II:1, 7, m		Syncope, bradycardia	580	c.[1031 C>T]; [1031 C>T] p.[A344V]; [A344V]
	I:1,, m		n.s	443	c.[1031 C>T]; [=] p.[A344V]; [=]	n.m
	I:2,, f		n.s	478	c.[1031 C>T]; [=] p.[A344V]; [=]	n.m
H	II:1,12, m	11	Syncope	485	c.[514G>A]; [514G>A] p. [V172M];[ V172M] and c.[877C>T]; [877C>T] p. [R293C];[R293C]	Propranolol (10 mg tid)
	I:1,44, m		n.s	417	c.[514G>A; 877C>T]; [=] p.[V172M; R293C];[ =]	n.m
	I:2, 42, f		n.s	415	c.[514G>A; 877C>T]; [=] p. [V172M; R293C];[ =]	n.m

n.a, not available; n.s, no symptoms; n.m, no medication.