A representative case of quinidine’s therapeutic effect on Brugada syndrome and the negative impact of the lack of availability in some countries, with a brief note on the lack of benefit of cilostazol

Figures

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  Figure 1. High precordial leads: V1–V3, corresponding to right second, third, and fourth intercostal spaces, from the index case.

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  Figure 2. Precordial leads of index case before and after drug therapy. A: Pre–drug therapy. B: Under 100 mg/d of cilostazol. C: Under 150 mg/d of cilostazol. Close-up: Note the lack of a significant effect on ST-segment elevation or J point under cilostazol compared to image A. D: One week after 600 mg/d of quinidine sulfate. Close-up: Note the normalization of the ST/J point electrocardiogram morphology and disappearance of the type 1 Brugada syndrome pattern.

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  Figure 3. Implantable cardioverter-defibrillator (ICD) electrogram during the arrhythmic storm event after quinidine discontinuation showed ventricular fibrillation successfully terminated by a 36 joule ICD shock (red arrow).

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  Supplemental Figure 1. The pedigree of the proband’s family. SCD = sudden cardiac death. P=proband

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  Supplemental Figure 2. Network analysis of protein interaction between DSP, TTN, and proteins associated with cilostazol (PDE3)) and quinidine (SCN alpha and beta subunits) treatment.