Table 1.

Summary of Pedigree as Shown in Figure 1

PATIENT	SYMPTOMS	QTc (ms)	THERAPY	Pro857Arg STATUS
I.1	Died in late 60s during heart surgery	NA	NA	NA
I.2	Status Unknown	NA	NA	NA
II.1†	Asymptomatic	383	None	−
II.2	Asymptomatic	486	None	+
II.3	Out of hospital cardiac arrest	NA	ICD Implantation	+
II.4	Died during infancy	NA	NA	NA
II.5†	Syncope during pregnancy	479	None	+
II.6	Asymptomatic	454	None	+
II.7	Asymptomatic	421	None	−
III.1	Asymptomatic	406	None	−
III.2*,†	Postpartum agonal breathing, syncope	498	ICD Implantation, sympathetic denervation	+
III.3	Asymptomatic	415	None	−
IV.1	Asymptomatic	450	Prophylactic beta blocker therapy	+
IV.2	Asymptomatic	455	None	+
IV.3	Asymptomatic	NA	None	−

^*Index Case

^†Whole Exome Sequenced

NA Not available

+ Pro857Arg present

− Pro857Arg absent

QTc is the heart rate corrected QT interval and is shown in milliseconds