The Brugada syndrome. Outcome of one case

The Brugada syndrome is a rare condition, and due to its mutating manner of presentation it may be difficult to diagnose. We report one case and discuss the diagnostic aspects and the clinical outcome of one patient with characteristic findings of this syndrome. These findings are especially defined by J-ST elevation in the right leads of serial electrocardiographic records, wide oscillations of J points and ST segments during 24-hour Holter monitoring, and nocturnal sudden death. We stress the importance of the Holter monitor findings for diagnostic complementation. Through this method it is possible to establish a correlation between vigil activities and sleep and the variability of the degree of impairment in ventricular repolarization

Arrhythmogenic cardiomyopathy of the right ventricle. Predictive value of QT interval dispersion to assess arrhythmogenic risk and sudden death

OBJECTIVE: To determine in arrhythmogenic right ventricular cardiomyopathy the value of QT interval dispersion for identifying the induction of sustained ventricular tachycardia in the electrophysiological study or the risk of sudden cardiac death. METHODS: We assessed QT interval dispersion in the 12-lead electrocardiogram of 26 patients with arrhythmogenic right ventricular cardiomyopathy. We analyzed its association with sustained ventricular tachycardia and sudden cardiac death, and in 16 controls similar in age and sex. RESULTS: (mean ± SD). QT interval dispersion: patients = 53.8±14.1ms; control group = 35.0±10.6ms, p=0.001. Patients with induction of ventricular tachycardia: 52.5±13.8ms; without induction of ventricular tachycardia: 57.5±12.8ms, p=0.420. In a mean follow-up period of 41±11 months, five sudden cardiac deaths occurred. QT interval dispersion in this group was 62.0±17.8, and in the others it was 51.9±12.8ms, p=0.852. Using a cutoff > or = 60ms to define an increase in the degree of the QT interval dispersion, we were able to identify patients at risk of sudden cardiac death with a sensitivity of 60%, a specificity of 57%, and positive and negative predictive values of 25% and 85%, respectively. CONCLUSION: Patients with arrhythmogenic right ventricular cardiomyopathy have a significant increase in the degree of QT interval dispersion when compared with the healthy population. However it, did not identify patients with induction of ventricular tachycardia in the electrophysiological study, showing a very low predictive value for defining the risk of sudden cardiac death in the population studied

Electrical behavior of T-Wave polarity alternans in patients with congenital long QT syndrome

AbstractOBJECTIVESThis study was designed to evaluate the incidence and characteristics of onset of T-wave polarity alternans (TWPA) in patients with long QT syndrome.BACKGROUNDThe T-wave alternans is a phenomenon that consists of beat-to-beat variability in the amplitude, morphology, and sometimes polarity of the T-wave, and it may trigger life-threatening arrhythmias.METHODSThe 24-h Holter recordings of 11 patients with congenital long QT syndrome were studied. Episodes of TWPA with 10 or more consecutive cycles were selected and analyzed as follows: 1) mean cycle length (MCL) and QTc interval duration (QTcI) of the episodes of TWPA and the 10 cycles preceding and succeeding the TWPA; 2) MCL and QTcI of the third, second, and first minute before onset (Mn−3, Mn−2, Mn−1); 3) MCL and QTcI from the tenth to the first cycle immediately preceding the onset of TWPA (R−10 to R−1); 4) MCL and QTcI from the first to the fourteenth cycle during alternans (R0 to R14); 5) MCL and QTcI from the first to the tenth cycle immediately succeeding TWPA (R+1 to R+10); 6) linear correlation (Lnc) between QT interval and cycle length (CL) (LncQT/CL) during alternans and for the 10 preceding cycles; 7) Lnc between the first three alternans cycles and episode duration (Lnc 3CL/EpD); and 8) difference between the longest and shortest QTc interval. We also selected episodes consisting of four or more consecutive cycles in order to analyze daily rhythms of the phenomenon.RESULTSThe TWPA was observed in 5 (45%) out of the 11 patients studied. The alternans process is initiated by a sudden shortening of the first alternans cycle without previous heart rate changes and ends at the moment when prolongation of the cycle tends to occur. LncQT/CL–alternans: r = 0.38 ± 0.2 (p = 0.20); without alternans: r = 0.81 ± 0.06 (p = 0.01). Lnc 3CL/EpD: r = 0.002 (p = 0.992). The QTc difference during alternans: 312.0 ± 52.1 ms; without alternans: 86.0 ± 36.4 ms (p = 0.001). Daily rhythm: 71% of the episodes occurred between 8 am and 8 pm, with higher incidence during the morning.CONCLUSIONSThe TWPA was dependent on the cardiac CL; there was loss of the LncQT/CL and an increase in the QT interval variability. Like other biological variables, T-wave polarity alternans has a higher density during the morning