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Case Reports
. 2016 Mar 4;2(3):211-216.
doi: 10.1016/j.hrcr.2015.12.001. eCollection 2016 May.

Correlation between functional and ultrastructural substrate in Brugada syndrome

Pablo E Tauber  1   2 , Virginia Mansilla  1 , Guillermo Mercau  1 , Felix Albano  2 , Ricardo R Corbalán  1 , Sara S Sánchez  3 , Stella M Honoré  3
Affiliations
Case Reports

Correlation between functional and ultrastructural substrate in Brugada syndrome

Pablo E Tauber et al. HeartRhythm Case Rep. .
No abstract available

Keywords: Biopsy; Brugada syndrome; Electrocardiography; Electrophysiology study; Radiofrequency catheter ablation; Right ventricular outflow tract.

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Figures

Figure 1
Figure 1
A: Twelve-lead electrocardiogram (ECG) tracings baseline: patent type 2 ECG (not diagnostic) displaying a PR interval of 200 milliseconds, QRS axis of 0°, QRS duration in V2 [fourth intercostal space (ICS)] of 120 milliseconds, QT/QTc of 360/383 milliseconds, the presence of an end-QRS slur with a descending ST segment in DI and horizontal in aVL, and saddleback-type ST-segment elevation observed in V2 (second ICS). Shift of right precordial leads in second higher ICSs does not reveal a type 1 patent ECG, but an increased saddleback-type ST-segment elevation is observed in V2. Note the presence of the end-QRS slur in DI and aVL, with a J-point peak ≥0.2 mV and descending ST segment in DI and horizontal in aVL (red arrows). B: Twelve-lead ECGs after oral administration of 400 mg of flecainide: In the third hour, the type 2 ECG is converted to the diagnostic type 1 patent ECG, which consists of a coved-type ST-segment elevation, observed in V2 of the standard 12-lead ECG (fourth ICS) and in V1, V2, and V3 recorded from the second ICS. Depolarization abnormalities are present as a prolonged PR interval of 210 milliseconds, QRS axis +8°, prolonged QRS duration in V2 of 150 milliseconds, and a high final R wave in aVR of 3 mm, consistent with right end conduction delay. Repolarization disorder can also be seen, with a prolonged QT/QTc interval of 420/481 milliseconds. Note the disappearance of the end-QRS slur in DI and aVL and the appearance of S waves and leveling ST segment (red arrows).
Figure 2
Figure 2
A: Baseline AH interval of 110 milliseconds, HV-DI of 60 milliseconds, and HV-V2 of 40 milliseconds; QRS duration in DII of 90 milliseconds and QRS duration in V2 of 150 milliseconds, indicating slow conduction in the right ventricular outflow tract (RVOT). B: Note the presence of middiastolic electrograms (EGMs) during sinus rhythm (red arrows). We identified areas that contained low-amplitude late potentials, corresponding to the anterior area of the high RVOT on the voltage map, where a systolic low-voltage EGM can also be seen in the proximal ablator. C: Spontaneous ventricular tachycardia from the RVOT and diastolic EGMs preceding the onset of the QRS complex can be seen (red arrows), which correspond to presystolic Purkinje-type potentials of low amplitude and high frequency preceding the QRS for 20 or 30 milliseconds. D. Voltage and electroanatomical maps, and the 3 areas of different voltage. HV-DI = HV interval measured DI; HV-V2 = HV interval measured V2; RVOT-D = distal right ventricular outflow tract; RVOT-P = proximal right ventricular outflow tract.
Figure 3
Figure 3
Endocardial biopsy by transmission electron microscopy. Voltage and electroanatomical map of A: normal lower area of the right ventricular outflow tract (voltage ≥ 1.5 mv), and B: Purkinje cell with abundant secretory vesicles in the normal lower area. Note that when approaching the pathological area, cytoplasmic disorganization, vacuolization (*) and myofibrillar remains in the adjacent cells were evident. C: Mitochondria showing normal crest, lipofuscin deposits near the myocyte nucleus and myofibrils with classic characteristics in normal lower area. D: Voltage and electroanatomical map of peripheral area (voltage between 0.5 and 1.5 mv). E: Myofibrillar and cellular remains in the peripheral area. F: Note the disappearance of the mitochondrial crests, mitochondrial swelling, and myofibrillar disorganization (*) in the same area. G: Voltage and electroanatomical map of central area (voltage ≤ 0.5 mv). Note the bioptome connected to the navigation system. H: Strong vacuolization and cell destruction in the central area. I: Intense cytoplasmic disorganization, vacuolization, and remains of myocardial fibers (*) in the central area. lp = lipofuscin deposits; mi = mitochondria; mf = myofibrils; nu = nucleus; pc = Purkinje cell. Scale bar: B 2.2 μm; C, F, I: 1.42 μm; E, H: 3.33 μm.
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