Table 1. Clinical and Genetic Characteristics of SCN5A 1493delK Carriers.

Patient ID	Gender	Age (y)	SCN5A Mutation	Clinical Presentation	Cardiac Imaging	Device	ECG	Ajmaline Challenge
10021_47	m	60	+	asymptomatic	normal#,§,$	ICD	increased PWD, AVB I°, CCD, RFB, LAFB	exclusion of BrS
10021_49	m	30	+	asymptomatic	normal#	–	increased PWD, AVB I°, CCD	n.p.
10021_50	f	35	+	asymptomatic	normal#	–	increased PWD, CCD	exclusion of BrS
10021_146	m	51	+	asymptomatic	normal#, mild RV-hypokinetic§,1-vessel CAD$	ICD	increased PWD, AVB I°, CCD, ST-elevation in V1/V2	n.p.
10021_149	f	18	+	asymptomatic	normal#,§,$	ICD	increased PWD, AVB I°, CCD	exclusion of BrS
10021_153	f	28	+	asymptomatic	normal#	–	increased PWD	n.p.
10021_154	f	57	+	asymptomatic	normal#	ICD	increased PWD, CCD	n.p.
10021_160	f	18	+	asymptomatic	normal#	–	increased PWD, CCD	exclusion of BrS
10021_161	f	20	+	asymptomatic	normal#	–	increased PWD, CCD	exclusion of BrS
10021_218	m	20	+	asymptomatic	normal§,$	ICD	increased PWD, AVB I°, CCD	exclusion of BrS
10021_33	m	73	(+)	non-cardiac death	n.a.	–	n.a.	n.p.
10021_142	f	79	(+)	natural death	n.a.	–	n.a.	n.p.
10021_150	f	49	(+)	SCD	n.a.	PM	n.a., sinus bradycardia reported	n.p.
10021_158	f	47	(+)	SCD	n.a.	–	increased PWD, CCD	n.p.
10021_213	f	68	(+)	non-cardiac death	n.a.	–	n.a.	n.p.
10021_215	m	44	(+)	SCD	n.a.	–	n.a.	n.p.

ECG, electrocardiogram; ICD, implantable cardioverter defibrillator; PM, Pacemaker; m, male; f, female; y, years; +, mutation carrier or ICD implanted; (+),obligate mutation carrier; SCD, sudden cardiac death; RV, right ventricle; CAD, coronary artery disease; #, by transthoracic echocardiographic; §, by magnetic resonance imaging; $, by ventriculography; n.p., not performed; n.a., not avalible; PWD, P-wave duration; AVB I°, atrioventricular block first-degree; CCD, cardiac conduction delay; RFB, right fascicular block; LAFB, left anterior fascicular block. Propositus (10021_149) printed in italics.