Figure 1.

Pedigrees of representative families and ECGs of JWS patients carrying CACNA1C-G37R. (a) The family members included proband 1 and his deceased younger brother with a history of SCD. (b) Proband 2 and his deceased younger brother both presented with ERS/SQT, and the father died of SCD at age of 50 years. (c) The ECG of proband 3 shows an ajmaline-induced Type 1 pattern in leads V1 and V2. (d) After ajmaline provocation, ECG of proband 4 is characterized by a coved ST-segment elevation in leads V1 and V2. (e) ECG of proband 1 is presented at baseline and after treatment with quinidine. He shows significant decrease of ST-segment in leads V2–V6 and remained asymptomatic on quinidine. (f) ECG of proband 2 showing spontaneous ERP in anterior leads. Squares indicate male subjects, circles female subjects and symbols with a slash mark deceased individuals. The arrows indicate the probands and pointed triangle indicates twins. −/− wild-type (WT); +/− heterozygous for the mutation. The symptomatic subjects are labelled by black. The asymptomatic subjects are shown as white and unaffected subjects are labelled by dark grey. (Online version in colour.)