Characterization and Management of Arrhythmic Events in Young Patients With Brugada Syndrome

Background

Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.

Polyarteritis nodosa and hypertrophic obstructive cardiomyopathy. A true association?

A 60-year-old man with polyarteritis nodosa under treatment presented with syncope. Echocardiography demonstrated hypertrophic obstructive cardiomyopathy; coronary arteriography revealed normal findings, and Holter monitor showed episodes of non-sustained ventricular tachycardia. This is the first report of hypertrophic obstructive cardiomyopathy developing in a patient with polyarteritis nodosa. Further studies should examine whether a true association exists.Abbreviations ESR Erythrocyte sedimentation rate - PAN Polyarteritis nodosa     

Morphology of the Cardiac Conduction System in Patientswith Electrophysiologically Proven Dual Atrioventricular Nodal Pathways

Conduction System in Dual AV Nodal Pathways. Introduction: Although the electrophysiologic criteria for dual atrioventricular nodal pathways are well established, the anatomical substrate is still unclear.
Methods and Results: We examined the hearts from 10 patients who had been studied electrophysiologically prior to cardiac transplantation. All 10 patients were male, aged 22 to 60years. Nine of the 10 patients had dual atrioventricular nodal pathways according to acceptedcriteria. Histologic studies of the atrioventricular conduction system showed normal structureof the atrioventricular node in all 10 hearts, with minor variations within the node in 3 cases, within the penetrating bundle in 3 cases, and within the nonbranching bundle in 3 cases. Theatrial approaches to the atrioventricular node were generally scanty in 6 hearts. The solitarycase that was shown electrophysiologically to lack dual pathways had no obvious difference inthe structure of the nodal area other than sparsity of transitional cells. We were unable tolocate any extranodal atrial tracts as described by other investigators.
Conclusion: The anatomical substrate for conduction over dual pathways may he too subtleto be detected by gross morphologic studies. Since dual pathways were unmasked in allpatients but one during electrophysiologic studies, it may be that the potential for these pathways is ubiquitous.     

Wavelet analysis of high-resolution signal-averaged ECGs in postinfarction patients

The authors present an original method for the discrimination of patients prone to ventricular tachycardia. The wavelet transform, which is a new time-scale technique suitable for transient signal detection, was applied to bipolar unfiltered X, Y, Z signal-averaged electrocardiograms in 20 postinfarction patients with sustained ventricular tachycardia, in 20 myocardial infarction patients without ventricular tachycardia, and in 10 healthy subjects. An improved automated algorithm for the detection and localization of sharp variations of the signal, based on coherent detection of the local maxima of the wavelet transform, was developed. A risk stratification method, based on the detection of at least one singularity at or after a point defined with reference to the QRS onset, was assessed. The optimum cutoff point, found 98 ms after the onset of QRS, provides a specificity of 90% and a sensitivity of 85%. The authors conclude that wavelet analysis makes it possible, in this group of patients, to discriminate those with ventricular tachycardia. It yields better results than those obtained from the conventional time-domain approach.     

Late QRS Activity in Signal‐Averaged Magnetocardiography,Body Surface Potential Mapping,and Orthogonal ECG in Postinfarction Ventricular Tachycardia Patients

Background: Delayed electrical activity necessary for re‐entrant ventricular tachycardia (VT) is detectable noninvasively with high resolution techniques. We compared high resolution signalaveraged analysis of magnetocardiography (MCG), body surface potential mapping (BSPM), and orthogonal three‐lead ECG (SA‐ECG) in the identification of patients prone to VT after myocardial infarction (Ml). Methods: Patients with remote myocardial infarction and cardiac dysfunction were studied, 22 with (VT group) and 22 without VT (control group). MCG with seven channels and BSPM with 63 and SA‐ECG with three orthogonal leads were registered. After signal‐averaging and highpass filtering, three time domain analysis (TDA) parameters describing late electrical activity were computed: QRS duration (QRSd), root mean square amplitude (RMS) of the last 40 ms of QRS, and the duration of the low‐amplitude QRS end (LAS). Results: All parameters by each method were significantly different between the patients’groups. For example, LAS parameter in MCG was 59 (SD 22) ms in the VT group vs. 37 (SD 13) ms in controls (P < 0.001), 77 (SD 22) ms vs. 56 (SD 19) ms in BSPM (P = 0.002), and 60 (SD 24) ms vs. 39 (SD 22) ms in SA‐ECG (P = 0.005). The combination of LAS parameter in MCG and SA‐ECG resulted in improved performance in comparison to any single parameter with 95% sensitivity and 68% specificity. Conclusions: All three high resolution methods identified VT propensity among post‐Mi patients with cardiac dysfunction and between‐method differences were small. Information in MCG and SA‐ECG may be complementary and their combination could be of value in postinfarction arrhythmia risk assessment. A.N.E. 2002;7(4):389–398     

Ventricular Tachycardia/Ventricular Fibrillation Ablation in the Setting of Ischemic Heart Disease

Recurrent ventricular tachycardia (VT) in the setting of coronary artery disease is frequently a life-threatening electrophysiologic emergency. Even in patients with an implantable defibrillator, recurrent VT is frequently accompanied by repeated and disabling shock therapy. Catheter ablative therapy offers the ability to provide immediate control of recurrent VT. Long-term elimination of VT should be anticipated in most patients. This article reviews the strategies, tools, techniques, and expected outcome for catheter ablation of stable and unstable ventricular arrhythmias in the setting ischemic heart disease.     

A novel LMNA indel mutation identified in a family with atrioventricular block and atrial fibrillation

It is well known that many genetic factors are involved in the occurrence and progression of atrioventricular block (AV block) and atrial fibrillation (AF). However, the genetic variants discovered so far have only explained parts of these processes. More genes and variants remain to be identified. In the present study, a three-generation family with an autosomal dominant form of AV block and AF was enrolled. Whole exome sequencing was conducted in three affected and one unaffected family member. A total of 64 nonsynonymous variants was shared by three affected individuals and not present in the unaffected individual. By selection of variants absent in the known databases and were predicted to be deleterious, 4 novel variants were identified. Only one novel frameshift insertion in the LMNA gene (c.825_826insCAGG) was identified in another affected family member and not detected in other non-affected family members and the 100 controls. Our finding expanded the spectrum of variants associated with AV block and AF, and was valuable in the genetic diagnosis of AV block and AF.     

The RR index test for the differentiation of atrioventricular nodal block from His--Purkinje block during incremental atrial pacing in patients with bifascicular block

AIMS: His—Purkinje block induced by incremental atrial pacingis highly predictive of an impending high degree atrioventricularblock in patients with bifascicular block. The His potentialis, however, sometimes not measurable or is lost in the ventriculardepolarization. The aim of this study was to evaluate whetherthe comparison of RR intervals before and after atrioventricularblock, induced by incremental atrial pacing, could differentiatebetween atrioventricular nodal and His—Purkinje blockin patients with bifascicular block. METHODS AND RESULTS: In 98 patients with bifascicular block, incremental atrial pacingwas performed as part of an invasive electrophysiological study.An ‘RR index’ was constructed by calculating thenumerical difference between the RR interval immediately beforeand after the atrioventricular block divided by the RR intervalimmediately before the pacing-induced block. Endocavitary recordingof the His bundle potential was used for defining the levelof atrioventricular block. The median RR index was 0·98(range 0·88–1·02) in recordings with His—Purkinjeblock and 0·49 (range 0·11–0·89)in recordings with atrioventricular nodal block (P<0·001).An RR index of 0·85 had a sensitivity of 100% and a specificityof 99% for the identification of atrioventricular block localizedto the His—Purkinje system. CONCLUSION: The use of an RR index is a helpful tool in the differentiationof His—Purkinje from atrioventricular nodal block in patientswith bifascicular block undergoing incremental atrial pacingas part of an invasive electrophysical study.     

Baroreflex modulation of ventricular rhythm in atrial fibrillation

The influence of bilateral carotid sinus nerve stimulation wasstudied in a patient with atrial fibrillation. Stimulation resultedafter approximately 1.5 s in abrupt prolongation of the minimum,median and maximum RR-interval, and in greater RR-interval dispersion.Virtually no change was observed in the random character ofthe ventricular rhythm. The absence of demonstrable baroreflexmodulation of the ventricular rhythm in atrial fibrillationis probably due to the relatively long latency of the reflexeffect on atrioventricular conduction in relation to the durationof the RR-intervals.