case_images: PMC10512029_01
case_images: Family pedigree. The patient's father (II-3) suddenly passed away at 36 years of age with a suspected cardiac cause. His father's nephew (III-2) had suffered heart arrest at 37 years of age and was implanted with an implantable cardioverter-defibrillator (ICD) after he tested positive for BrS [pathogenic variant, SCN5A c.4222G > A (p.Gly1408Arg)]. The nephew's daughter (IV-2) also tested positive for BrS on genetic testing. Another close relative (III-5, grandson from his grandmother's sister) passed away at 22 years of age while playing basketball.
case_images: fcvm-10-1193878-g001.jpg
case_images: PMC10512029_01_fcvm-10-1193878-g001.jpg
case_images: F1
case_images: However, there is a positive BrS family history (Figure 1) as well as a repeated occurrence of sudden cardiac deaths.
case_images: Timeline.
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case_images: PMC10512029_01_fcvm-10-1193878-g002.jpg
case_images: F2
case_images: Proband's electrocardiogram. Precordial leads show right bundle branch block morphology with Brugada pattern ST segment elevation in V1 and V2. ECGs in this study were recorded at standard ECG paper speeds of 25 mm/s and 10 mm/mV.
case_images: fcvm-10-1193878-g003.jpg
case_images: PMC10512029_01_fcvm-10-1193878-g003.jpg
case_images: F3
case_images: The V2 lead detected a "saddle-back" ST-elevation that is characteristic of the type-2 BrS ECG pattern in majority of ECG strips, however one ECG showed typical type-1 BrS ECG pattern (Figure 3).