case_images: PMC10978698_01
case_images: The initial ECG of the index patient, which was recorded on an automated external defibrillator. Defibrillation was performed for the ventricular fibrillation that occurred during transfer of the patient to the emergency room (A). After the cardioversion, ST elevation was observed in the defibrillator-patch ECG (B). The initial 12-lead ECG showed normal sinus rhythms with first-degree AV block (C). The PR interval was 240 ms. A type 2 Brugada pattern ECG was observed in V1 and V2. The V1 and V2 showed dynamic changes that is more like type 1 ECG (D). ECG, electrocardiogram.
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case_images: PMC10978698_01_fcvm-11-1334096-g001.jpg
case_images: F1
case_images: While the patient was being transferred to the emergency room, defibrillation was performed twice due to ventricular fibrillation (Figures 1A,B). The initial ECG showed a normal sinus rhythm with a first-degree AV block (PR-interval was 240 ms), and a type 2 Brugada pattern was observed in the V1 lead (Figure 1C).
case_images: ECG of the index patient during and after therapeutic hypothermia. Widespread J wave accentuation was observed during the hypothermia (A). Marked PR-interval prolongation (440 ms) was identified after therapeutic hypothermia (B). In a follow-up ECG, a spontaneous type 1 Brugada pattern was identified with an incidental atrial fibrillation (C). ECG, electrocardiogram.
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case_images: PMC10978698_01_fcvm-11-1334096-g002.jpg
case_images: F2
case_images: Prominent J wave accentuation with PR-interval prolongation was identified during the therapeutic hypothermia (Figure 2). In a follow-up ECG, a spontaneous type 1 Brugada pattern was identified with an incidental atrial fibrillation (Figure 2C) The patient was diagnosed with BrS and underwent implantable cardioverter defibrillator (ICD) therapy.
case_images: An implantable cardioverter defibrillator shock during sleep was detected and terminated successfully.
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case_images: PMC10978698_01_fcvm-11-1334096-g003.jpg
case_images: F3
case_images: After the ICD, the patient suffered ICD shocks due to ventricular fibrillation (VF) during sleep (Figure 3).
case_images: Family pedigree with ECG phenotypes in V1 and V2. The proband's ECG showed a type 1 Brugada pattern in V1. The brother's ECG showed a long PR interval of 480 ms and incomplete RBBB in the 3rd intercostal V1 and V2. The father's ECG showed a long PR interval of 240 ms. The mother's ECG showed a long PR interval of 220 ms without the Brugada pattern on the ECG. One maternal aunt showed a type 1 Brugada pattern in V2. One maternal uncle, who experienced aborted sudden cardiac death, showed a suspicious BrS-pattern on the ECG in V1. One paternal aunt showed an RBBB with a normal PR interval. One paternal female cousin showed a type 2 Brugada pattern with a PR interval of 200 ms in V2. Information about the genetic mutations of first-degree relatives of the patient is also given. The squares represent males, and the circles represent females. The squares/circles with black in the left upper quadrant represent the BrS-like ECG. The squares/circles with black in the left lower quadrant represent PR prolongation. The squares/circles with red in the right upper quadrant represent the SCN5A-R376H variant. The squares/circles with red in the right lower quadrant represent the DSP-Y1101C variant. 3ICS, third intercostal space; ECG, electrocardiogram; BrS, Brugada syndrome.
case_images: fcvm-11-1334096-g004.jpg
case_images: PMC10978698_01_fcvm-11-1334096-g004.jpg
case_images: F4
case_images: We performed genetic tests using next-generation sequencing (NGS) in all first-degree relatives and evaluated the ECGs of almost all family members (Figure 4).