PMC5420038_01
Proband's ECGs before ablation. A: Baseline ECG shows positive delta waves in leads I, II, aVL, and V1-V6 and negative delta waves in leads in leads III and aVF. B: One episode of atrial fibrillation in the presence of an accessory pathway recorded before ablation. Black arrows indicate concomitant type 1 Brugada ECG. ECG = electrocardiogram.
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A representative electrocardiogram (ECG) at rest revealed WPW syndrome with a short PR interval and positive delta waves in leads I, II, aVL, and V1-V6 and negative delta waves in leads III and aVF (Figure 1A). Certain beats, which were conducted over the normal pathway, were compatible with type 1 Brugada ECG in leads V1 and V2 (Figure 1B).
Follow-up ECGs of the proband after ablation. After radiofrequency catheter ablation, the regular ECG displays disappearance of delta waves and onset of coved-type ST-segment elevation followed by negative T waves in leads V1 and V2 in 2 days (A). One month (B) later, it shows a type 2 Brugada pattern in the fourth ICS and a typical type 1 Brugada pattern in the second ICS. A similar ECG pattern is discovered after 9 and 12 years (C & D). ECG = electrocardiogram; F-U = follow-up; ICS = intercostal space.
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Two days after the procedure, the ECG showed disappearance of delta waves and appearance of coved-type ST-segment elevation followed by negative T waves in leads V1 and V2 (Figure 2A). AF were diminished, but a typical type 2 Brugada pattern in the fourth intercostal space and a type 1 Brugada pattern in the second intercostal space were observed (Figure 2B). A similar Brugada pattern is discovered in the ECG after 9 and 12 years (Figures 2C and 2D).
Summary of the genetic information and medical history of the family. A: Family pedigree of the index case. B: Baseline ECG of proband's youngest brother (II-7) in 4th ICS (left) and 2nd ICS (right) in 2004 who suffered from sudden cardiac death in 2013. C: DNA chromatogram of SCN5A-WT and SCN5A-R1193Q. The latter is discovered in the index case and his family member. D: Landmark events during follow-up for the family. ICS = intercostal space.
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Figure 3A displayed the family pedigree of the index case. His asymptomatic younger brother (II-7) also had a spontaneous type 1 Brugada ECG pattern then (Figure 3B). By screening all susceptible genes associated with BrS and WPW syndrome in this family, the SCN5A-R1193Q variant was found in the proband and his nephew (III-3, Figures 3A and 3C). He has not received any antiarrhythmic drugs and has not experienced ICD discharge by far (Figure 3D).