The link in Linking

We present 2 cases of the slow-fast form of AVNRT with initially narrow QRS complexes followed by sudden unexpected transition to persistently wide QRS complexes due to aberrant intraventricular conduction. Introduction of a properly timed extrastimulus in one case and critical oscillations in cycle length due to short-long coupling in the second case set the stage for the initial bundle branch block. However, persistence of the aberrancy pattern once the initial event abated was maintained by the “linking” phenomenon. Delayed, retrograde concealed activation from the contralateral bundle branch perpetuated the initial bundle branch block.     

Influence of left bundle branch block on left ventricular volumes, ejection fraction and regional wall motion

Background. Left ventricular volumes, ejection fraction and regional wall motion are cardiac parameters which provide valuable information for patient management in a large variety of cardiac conditions. Differences in regional wall motion are of relevance in the field of cardiac resynchronisation therapy. We quantified three-dimensional echocardiographic measurements of left ventricular volumes, ejection and regional wall motion (e.g. expressed as systolic dyssynchrony index (SDI)) in two patient cohorts: patients with normal conduction and patients with complete left bundle branch block. Methods. Thirty-five patients scheduled for routine cardiac examination underwent three-dimensional echocardiography: 23 patients with normal conduction and 12 patients with a complete left bundle branch block. Full-volume datasets were analysed and end-systolic volume (ESV), end-diastolic volume (EDV) and ejection fraction (EF) were obtained. SDI was derived from the standard deviation of the measured times to reach minimal regional volume for each of the 16 segments of the left ventricle. Results. A significant difference was observed in left ventricular volumes, ejection fraction and SDI between the two groups. Patients with complete left bundle branch block showed higher EDV (p=0.025) and ESV (p<0.01) and a lower EF (p<0.01) than patients with normal conduction. SDI is significantly higher in patients with complete left bundle branch block (p=0.004) expressing a higher amount of ventricular dyssynchrony. Intraobserver variability showed excellent correlation coefficients: r=0.99 for EDV, ESV and SDI and r=0.98 for EF. Conclusion. Three-dimensional echocardiography is a feasible and reproducible method for the quantification of left ventricular volumes, left ventricular ejection fraction and regional wall motion. Differences can be assessed between normal patients and patients with left bundle branch block. (Neth Heart J 2007;15:89-94.)     

A novel rabbit model of variably compensated complete heart block.

Complete heart block (CHB) provides a useful substrate for study of bradycardia-dependent ventricular arrhythmias and cardiac function. Existing CHB animal models are limited by surgical recovery time and reliance on intrinsic escape rhythms. We describe a novel closed-chest rabbit model of CHB involving transcatheter radiofrequency (RF) atrioventricular (AV) node ablation and ventricular rate control with chronic transvenous pacing. Permanent CHB was achieved in 34 of 38 attempts overall. Procedural mortality due to cardiac tamponade (n = 2), airway complications (n = 2), and unknown causes (n = 5) occurred in nine animals. Survivors with CHB (n = 28) were maintained for < or = 22 days, during which there were three late deaths related to infection (n = 1) or respiratory distress (n = 2). None of the survivors with CHB showed recovery of AV conduction or pacemaker capture loss during chronic ventricular pacing at about one-half normal sinus rates, and 25 animals surviving to death showed no overt signs of hemodynamic compromise such as lethargy, poor feeding, or respiratory distress. This approach provides a reproducible nonsurgical CHB model with adjustable ventricular rate control.     

Bundle branch reentrant ventricular tachycardia

Bundle branch reentrant (BBR) tachycardia is an uncommon form of ventricular tachycardia (VT) incorporating both bundle branches into the reentry circuit. The arrhythmia is usually seen in patients with an acquired heart disease and significant conduction system impairment, although patients with structurally normal heart have been described. Surface ECG in sinus rhythm (SR) characteristically shows intraventricular conduction defects. Patients typically present with presyncope, syncope or sudden death because of VT with fast rates frequently above 200 beats per minute. The QRS morphology during VT is a typical bundle branch block pattern, usually left bundle branch block, and may be identical to that in SR. Prolonged His-ventricular (H-V) interval in SR is found in the majority of patients with BBR VT, although some patients may have the H-V interval within normal limits. The diagnosis of BBR VT is based on electrophysiological findings and pacing maneuvers that prove participation of the His- Purkinje system in the tachycardia mechanism. Radiofrequency catheter ablation of a bundle branch can cure BBR VT and is currently regarded as the first line therapy. The technique of choice is ablation of the right bundle. The reported incidence of clinically significant conduction system impairment requiring implantation of a permanent pacemaker varies from 0% to 30%. Long-term outcome depends on the underlying cardiac disease. Patients with poor systolic left ventricular function are at risk of sudden death or death from progressive heart failure despite successful BBR VT ablation and should be considered for an implantable cardiovertor-defibrillator.     

Tailoring cardiac resynchronisation therapy to non-left bundle branch block: Successful cardiac resynchronisation for right bundle branch block with left posterior fascicular block without implantation of a left ventricular lead

Despite advances, cardiac resynchronisation therapy (CRT) remains fundamentally orientated to the dyssynchrony of left bundle branch block (LBBB), in which septo-lateral electrical and mechanical delays predominate. For non-LBBB patients response rates to conventional CRT are lower and mortality and rehospitalisation rates are not reduced. Despite this, alternative approaches which tailor CRT to the differing dyssynchrony patterns of non-LBBB have yet to be developed. In the specific non-LBBB subgroup of right bundle branch block (RBBB) with left posterior fascicular block (LPFB), ventricular conduction via the left anterior fascicle results in a unique early lateral, and late septal depolarisation, or lateral to septal left ventricular (LV) delay, an electrical sequence which is followed mechanically. This latero-septal delay is somewhat the reverse of LBBB and was overcome by fusing right ventricular (RV) septal pacing with intrinsic conduction via the left anterior fascicle, achieving successful resynchronisation without implantation of a left ventricular lead. A stable fusion pattern was achieved via the ‘Negative AV Hysteresis with Search’ algorithm (Abbott, St Paul, Minnesota). Improvement in all standard CRT response indices was achieved at 3 months: QRS duration was reduced from 153 to 106 ms, ejection fraction increased from 14 to 32%, and LV end-systolic and end-diastolic diameters reduced by 19% and 12.5% respectively. NYHA class improved from III-IV to class II. Cardiac resynchronisation for RBBB with LPFB can be successfully achieved with a standard pacemaker or defibrillator without left ventricular lead implantation by fusing RV septal-only pacing with intrinsic conduction.     

Normalisation of the ECG in a patient with right bundle branch block by cardiac pacing

Right ventricular apical pacing (RVA) appears to have potential deleterious effects on myocardial systolic and diastolic left ventricular function, especially in patients with intact AV conduction. Therefore, new pacing sites in the right ventricle are being explored to overcome these detrimental effects. Alternative pacing sites in the right ventricle are the right ventricular outflow tract (RVOT) and the right ventricular septum (RVS). In this case report, we demonstrate an exceptional form of ventricular fusion, namely normalisation of the QRS complex in a patient with pre-existing right bundle branch block by RVS pacing. To our knowledge, this is the first report in the literature where right ventricular pacing could restore a complete RBBB to a normal QRS complex by stimulating distally from the anatomical position of the RBBB, due to fusion between artificial right ventricular stimulation and intrinsic conduction over the left bundle of the specific His-Purkinje system.     

Sudden unexpected improvement in the atrioventricular conduction. What is the mechanism?

The 12-lead electrocardiogram (ECG) of a 79-year-old male patient with recurrent pre-syncope showed irregular sinus rhythm with constant PR interval and left bundle branch block (LBBB) with intermittently blocked P waves. The beat following the blocked P wave had a narrower QRS with a shorter PR interval. The phenomenon of bilateral bundle branch block explains the sudden improvement in the atrioventricular conduction.     

Misdiagnosing ventricular tachycardia in patients with underlying conduction disease and similar sinus and tachycardia morphologies.

Six patients presented with wide-complex tachycardias with QRS morphologies similar to those seen on their electrocardiograms that showed normal sinus rhythms. During normal sinus rhythm, each patient had an underlying intraventricular conduction abnormality or bundle branch block. Despite the similarity of the QRS complexes to those seen during sinus rhythm, the tachycardias subsequently proved to be ventricular in origin in each patient. It is important not to misdiagnose these disorders as supraventricular tachycardia as an erroneous diagnosis may result in inappropriate management.     

Noninvasive electrocardiographic imaging for cardiac electrophysiology and arrhythmia

Over 7 million people worldwide die annually from erratic heart rhythms (cardiac arrhythmias), and many more are disabled. Yet there is no imaging modality to identify patients at risk, provide accurate diagnosis and guide therapy. Standard diagnostic techniques such as the electrocardiogram (ECG) provide only low-resolution projections of cardiac electrical activity on the body surface. Here we demonstrate the successful application in humans of a new imaging modality called electrocardiographic imaging (ECGI), which noninvasively images cardiac electrical activity in the heart. In ECGI, a multielectrode vest records 224 body-surface electrocardiograms; electrical potentials, electrograms and isochrones are then reconstructed on the heart's surface using geometrical information from computed tomography (CT) and a mathematical algorithm. We provide examples of ECGI application during atrial and ventricular activation and ventricular repolarization in (i) normal heart (ii) heart with a conduction disorder (right bundle branch block) (iii) focal activation initiated by right or left ventricular pacing, and (iv) atrial flutter.     

The conduction system of the heart in mice chronically infected with Trypanosoma cruzi: histopathological lesions and electrocardiographic correlations

Chronic focal and diffuse myocarditis with interstitial fibrosis developed in Swiss outbred mice and in the inbred AKR and A/J strains of mice which were chronically infected with several Trypanosoma cruzi strains belonging to three biological types (Type I, II and III). High incidence of electrocardiographic changes with predominance of intraventricular conduction disturbances, 1st. and 2nd. degree AV block, arrhythmias, comparable with those found in human Chagas' disease, were also present. Morphological study of the conduction tissue of the heart revealed inflammatory and fibrotic changes. The presence of inflammation in the inter-atrial septum almost always coincided with the inflammatory involvement of the ventricular conduction system. Focal inflammation was associated with vacuolization and focal necrosis of the specific fibers. Most of the lesions were seen affecting the His bundle (76.3% of the cases), the right bundle branch (73.3%), AV node (43.9%) and left bundle branch (37.5%). Correlation between morphological changes in the conduction tissue and electrocardiographic alteration occurred in 53.0 to 62.5% of the cases, according to the experimental groups.     

Cardiomyopathy induced by incessant ventricular tachycardia originating in the vicinity of the His bundle

A 04-year-old boy was referred to our institution with severe, progressive heart failure of 4-months duration associated with a persistent wide QRS tachycardia with left bundle branch block and severe left ventricular dysfunction. Because of incessant wide QRS tachycardia refractory to antiarrhythmic drugs, he was referred for electrophysiological study. The ECG was suggestive of VT arising from the right ventricle near the His area. Electrophysiological study revealed that origin of tachycardia was septum of the right ventricle, near His bundle, however the procedure was not successful and an inadvertent complete atrioventricular conduction block occurred. The same ventricular tachycardia recurred. A second procedure was performed with a retrograd aortic approach to map the left side of the interventricular septum. The earliest endocardial site for ablation was localized in the anterobasal region of left ventricle near His bundle. In this location, one radiofrequency pulse interrupted VT and rendered it not inducible. The echocardiographic evaluation showed partial reversal of left ventricular function in the first 3 months. The diagnosis was idiopathic parahisian left ventricular tachycardia leading to a tachycardia mediated cardiomyopathy, an extremely rare clinical picture in children.     

Left septal fascicular block: myth or reality?

Anatomic studies have shown that the left bundle branch divides into three fascicles in most humans. Changes in the 12 lead ECG (electrocardiogram) due to conduction abnormalities of the left anterior fascicle and left posterior fascicle are now part of the standard repertoire of electrocardiographic interpretation. There are no standard criteria for detecting conduction defects involving the third left fascicle, the septal or median fascicle, and the very existence of such defects is still a matter of controversy. The purposes of this article are to review the available evidence on this subject, suggest electrocardiographic criteria for its recognition, and present examples which illustrate that left septal fascicular block does indeed exist as a specific entity. Left septal fascicular block is a polymorphic conduction defect which may explain some previously inadequately understood electrocardiographic abnormalities.     

The pathophysiology of multiple sclerosis: the mechanisms underlying the production of symptoms and the natural history of the disease

The pathophysiology of multiple sclerosis is reviewed, with emphasis on the axonal conduction properties underlying the production of symptoms, and the course of the disease. The major cause of the negative symptoms during relapses (e.g. paralysis, blindness and numbness) is conduction block, caused largely by demyelination and inflammation, and possibly by defects in synaptic transmission and putative circulating blocking factors. Recovery from symptoms during remissions is due mainly to the restoration of axonal function, either by remyelination, the resolution of inflammation, or the restoration of conduction to axons which persist in the demyelinated state. Conduction in the latter axons shows a number of deficits, particularly with regard to the conduction of trains of impulses and these contribute to weakness and sensory problems. The mechanisms underlying the sensitivity of symptoms to changes in body temperature (Uhthoff's phenomenon) are discussed. The origin of 'positive' symptoms, such as tingling sensations, are described, including the generation of ectopic trains and bursts of impulses, ephaptic interactions between axons and/or neurons, the triggering of additional, spurious impulses by the transmission of normal impulses, the mechanosensitivity of axons underlying movement-induced sensations (e.g. Lhermitte's phenomenon) and pain. The clinical course of the disease is discussed, together with its relationship to the evolution of lesions as revealed by magnetic resonance imaging and spectroscopy. The earliest detectable event in the development of most new lesions is a breakdown of the blood-brain barrier in association with inflammation. Inflammation resolves after approximately one month, at which time there is an improvement in the symptoms. Demyelination occurs during the inflammatory phase of the lesion. An important mechanism determining persistent neurological deficit is axonal degeneration, although persistent conduction block arising from the failure of repair mechanisms probably also contributes.     

Arrhythmia and sudden death associated with elevated cardiac chloride channel activity

The identification and analysis of several cationic ion channels and their associated genes have greatly improved our understanding of the molecular and cellular mechanisms of cardiac arrhythmia. Our objective in this study was to examine the involvement of anionic ion channels in cardiac arrhythmia. We used a transgenic mouse model to overexpress the human cystic fibrosis transmembrane conductance regulator (CFTR) gene, which encodes a cAMP-regulated chloride channel. We used RNase protection and in situ hybridization assays to determine the level of CFTR expression, and radiotelemetry and in vivo electrophysiological study in combination with pharmacological intervention to analyse the cardiac function. Cardiac CFTR overexpression leads to stress-related sudden death in this model. In vivo intracardiac electrophysiological studies performed in anaesthetized mice showed no significant differences in baseline conduction parameters including atrial-His bundle (AH) or His bundle-ventricular (HV) conduction intervals, atrioventricular (AV) Wenckebach or 2:1 AV block cycle length and AV nodal functional refractory period. However, following isoproterenol administration, there was marked slowing of conduction parameters, including high-grade AV block in transgenic mice, with non-sustained ventricular tachycardia easily inducible using programmed stimulation or burst pacing. Our sudden death mouse model can be a valuable tool for investigation of the role of chloride channels in arrhythmogenesis and, potentially, for future evaluation of novel anti-arrhythmic therapeutic strategies and pharmacological agents.     

Catheter ablation of fascicular ventricular tachycardia

Fascicular ventricular tachycardia (VT) is an idiopathic VT with right bundle branch block morphology and left-axis deviation occuring predominantly in young males. Fascicular tachycardia has been classified into three subtypes namely, left posterior fascicular VT, left anterior fascicular VT and upper septal fascicular VT. The mechanism of this tachycardia is believed to be localized reentry close to the fascicle of the left bundle branch. The reentrant circuit is composed of a verapamil sensitive zone, activated antegradely during tachycardia and the fast conduction Purkinje fibers activated retrogradely during tachycardia recorded as the pre Purkinje and the Purkinje potentials respectively. Catheter ablation is the preferred choice of therapy in patients with fascicular VT. Ablation is carried out during tachycardia, using conventional mapping techniques in majority of the patients, while three dimensional mapping and sinus rhythm ablation is reserved for patients with nonmappable tachycardia.