Successful Ablation of Antero-septal Accessory Pathway in the Non-Coronary Cusp in a Child

A 15-year-old boy with Wolff-Parkinson-White syndrome underwent an electrophysiology study for symptoms of palpitations and persistence of pre-excitation during peak exercise. He was detected to have right antero-septal accessory pathway with relatively long effective refractory period and no inducible tachycardia. He had only transient normalization with cryoablation. Eight months later, he presented again with two episodes of seizures with preceding palpitations. Neurology evaluation was unremarkable with a normal electroencephalogram. In view of his symptoms in association with evidence of pre-excitation, he underwent a second electrophysiology study with ablation. Cryoablation in the anterior septum again achieved only transient normalization. Mapping in the non-coronary cusp identified an earliest accessory pathway potential. RF ablation was performed in the non-coronary cusp with immediate normalization of his electrocardiogram. At 6 month follow-up, he continues to have no pre-excitation on his EKG. Ablation of the anteroseptal accessory pathway in the non-coronary cusp can be safely performed in patients' refractory to conventional ablation sites and techniques.     

Successful ablation of a left-sided accessory pathway in a patient with coronary sinus atresia and arteriovenous fistula: clinical and developmental insights

Background and objectives

While radiofrequency ablation catheter ablation of accessory pathways is generally safe and effective, anatomic variants can cause considerable challenges in effecting cure. Our objective was to use an unusual case where coronary sinus was absent and arterial venous fistula was present and a left-sided pathway required mapping and ablation to develop a framework to approach difficult cases.

Method

A detailed literature search and review of contemporary cardiac embryology was undertaken to attempt and to explain a common developmental anomaly. Adjunctive approaches during the ablation procedure, including intracardiac ultrasound, were used to guide mapping and ablation despite the lack of coronary sinus access.

Results

The accessory pathway was successfully ablated using a transseptal approach and intracardiac ultrasound guided mapping of the mitral annulus. A potential common mechanism to explain the apparently disparate anatomic variants in this patient was formulated.

Conclusions

Cardiac conduction development is complex and accessory pathway conduction may occur in the setting of arteriovenous anomalies thus providing insights as to the cause of WPW syndrome. Successful mapping and targeted ablation of left-sided pathways may be accomplished even when coronary sinus access is not possible.     

Cryothermal energy ablation of cardiac arrhythmias 2005: state of the art

At the time of antiarrhythmic surgery, cryothermal energy application by a hand-held probe was used to complement dissections and resections and permanently abolish the arrhythmogenic substrate. Over the last decade, significant engineering advances allowed percutaneous cryoablation based on catheters, apparently not very different from standard radiofrequency ablation catheters. Cryothermal energy has peculiar characteristics. In fact, it allows testing in a reversible way the effects of energy application at higher temperature, before producing a permanent lesion at -75 degrees C. Moreover, slow formation of the lesion allows timely discontinuation of the application, as soon as inadvertent modifications of normal atrioventricular conduction are observed during ablation in the proximity of atrioventricular node and His bundle, avoiding its permanent damage. Over the last years, percutaneous cryothermal ablation has been widely used for a variety of cardiac arrhythmias. From the data gathered, it is unlikely that cryoablation will replace standard ablation in unselected cases. Nevertheless, for the above mentioned peculiarities, cryothermal ablation has proved very effective and safe for ablation of arrhythmogenic substrates close to the normal conduction pathways, becoming the first choice method to ablate anteroseptal and midseptal accessory pathways. It can be also the best treatment for ablation of the slow pathway to abolish atrioventricular node reentrant tachycardia in pediatrics or when particular anatomy of the Koch's triangle is observed. Cryothermal ablation of the pulmonary veins for atrial fibrillation, although longer than radiofrequency ablation, is not associated with pulmonary vein stenosis and is expected to be less thrombogenic; new catheter designs for cryothermal ablation of this challenging arrhythmia are to be tested to assess their efficacy and clinical usefulness.     

Transhepatic approach for catheter ablation of accessory pathway in a child with complex congenital heart disease

We report on a 22-month-old boy with drug-resistant atrioventricular reentrant tachycardia and complex structural heart disease consisting of right atrial isomerism, mirror image orientation of the intrathoracic veins, hemi-azygos continuation to the left superior vena cava, separate drainage of the hepatic veins into the left-sided atrium, congenitally corrected transposition, pulmonary atresia, and atrial and ventricular septal defects.Access to the heart for radiofrequency (RF) ablation was obtained by percutaneous puncture of a hepatic vein, the left internal jugular vein, and femoral artery. The accessory pathway was localised to the free wall of the left-sided AV groove and successfully ablated. There were no procedure-related complications.RF ablation of an accessory pathway is feasible in young children with complex structural heart disease and abnormal systemic venous return. In such patients access to the heart must be planned with knowledge of the anatomy and judicious use of the hepatic venous approach.     

Dyssynchronous Ventricular Activation in Asymptomatic Wolff-Parkinson-White Syndrome: A Risk Factor for Development of Dilated Cardiomyopathy

A subset of children and adults with Wolff-Parkinson-White (WPW) syndrome develop dilated cardiomyopathy (DCM). Although DCM may occur in symptomatic WPW patients with sustained tachyarrhythmias, emerging evidence suggests that significant left ventricular dysfunction may arise in WPW in the absence of incessant tachyarrhythmias. An invariable electrophysiological feature in this non-tachyarrhythmia type of DCM is the presence of a right-sided septal or paraseptal accessory pathway. It is thought that premature ventricular activation over these accessory pathways induces septal wall motion abnormalities and ventricular dyssynchrony. LV dyssynchrony induces cellular and structural ventricular remodelling, which may have detrimental effects on cardiac performance. This review summarizes recent evidence for development of DCM in asymptomatic patients with WPW, discusses its pathogenesis, clinical presentation, management and treatment. The prognosis of accessory pathway-induced DCM is excellent. LV dysfunction reverses following catheter ablation of the accessory pathway, suggesting an association between DCM and ventricular preexcitation. Accessory pathway-induced DCM should be suspected in all patients presenting with heart failure and overt ventricular preexcitation, in whom no cause for their DCM can be found.     

Ventricular tachycardia based on cardiac sarcoidosis with a narrow QRS complex,ablated on the left ventricle free-wall

A septuagenarian female with cardiac sarcoidosis suffered from drug refractory ventricular tachycardia (VT) requiring multiple implantable cardioverter-defibrillator shocks. The QRS complex during the VT was very similar to that during sinus rhythm although the QRS width during the VT (142 ms) was relatively wider than that during sinus rhythm (107 ms). The VT exit was located on the ventricular septum close to the His-bundle recording region. However, the critical pathway of this VT was detected on the anterior free wall of the left ventricle (LV), and a radiofrequency application at that site could terminate the VT. No Purkinje potentials were recorded there during the VT or sinus rhythm. According to the electrophysiological study, 3-D mapping, and the response to the ablation, the critical circuit of the VT was surrounded by a protected area of scar associated with cardiac sarcoidosis. As a result, the VT circuit was connected to the basal septal area close to the His-Purkinje system as an outer loop of the VT circuit. This unique trajectory of the VT might have caused a similar QRS morphology to that of sinus rhythm, and the relatively narrow QRS complex despite the critical isthmus was located on the anterior free wall of the LV.     

Electrical activation of the ventricular myocardium of the crocodile Crocodylus johnstoni: a combined microscopic and electrophysiological study.

We mapped the sequence of ventricular depolarization in the crocodile Crocodylus johnstoni. We also attempted to find specialized conduction tissue within the ventricular myocardium. Electrical recordings with miniature multi-point electrodes revealed two strands of rapidly conducting tissue (channels) within the interventricular septum, suggestive of conductive tissue pathways. From these septal channels, wavefronts of excitation swept around each ventricle. Electrical recordings did not indicate that there was conductive tissue in the wall of either ventricle. Similarly, microscopic studies of the septal channels provided no indication of specialized conductive tissue. We suggest that the channels of early septal depolarization provide the crocodile heart with a high speed depolarization pathway functionally analogous to a rudimentary conductive system.     

Orthodromic atrioventricular reentrant tachycardia using a concealed isoproterenol-sensitive accessory pathway

Accessory pathways (APs) represent the substrate for atrioventricular reentrant tachycardia. Catecholamine-sensitivity is an uncommon feature of APs and has been almost exclusively been described in APs with antegrade conduction. We present the rare case of a catecholamine-dependent concealed AP that was only unmasked upon isoproterenol stimulation and successfully ablated. This case highlights the importance of systematic isoproterenol stimulation in patients referred for ablation of supraventricular tachycardia - in particular if the baseline electrophysiology study is negative. Otherwise, ablation targets may be missed.Learning objectiveThe absence of retrograde ventriculo-atrial conduction does not automatically exclude the presence of a concealed accessory pathway. Systematic isoproterenol stimulation should be part of any electrophysiology study for supraventricular tachycardia, to search for catecholamine-sensitive accessory pathways that may be otherwise missed.     

The transsynaptic regulation of the septal-hippocampal cholinergic neurons

There is not yet a complete understanding of the functional interactions among various septal nuclei which regulate hippocampal function. Nevertheless, much has been learned histologically and biochemically about the major connections of the distinct areas of the septal complex and the chemical character of some of these pathways. The cholinergic septal-hippocampal pathway serves as a well defined link between these two important structures of the limbic system. Acetylcholine turnover rates in the hippocampus have been shown to increase or decrease proportionally to the activity of the cholinergic neurons originating in the septum. Moreover, these turnover rates have been shown to be modulated by intraseptal injections of agonists or antagonists of various neurotransmitters or neuromodulators which are stored in various cell groups located in the septum. By coupling this biochemical approach with techniques to study the receptor organization, greater detail concerning the transmitter and cotransmitter interactions among the various neuromodulators can be obtained.     

What Next After Failed Septal Ventricular Tachycardia Ablation?

Ablation of ventricular tachycardia (VT) by conventional radiofrequency ablation can be impossible if the ventricular wall at the targeted ablation site is very thick, as for example the ventricular septum. We present a case of a patient with incessant, non-sustained slow VT originating from the septal part of the lower outflow tracts. Radiofrequency catheter ablation from both ventricles as well as from the anterior cardiac vein were not successful. Both high power radiofrequency ablation and bipolar radiofrequency ablation neither were successfull. Finally, ethanol ablation of the first septal perforator successfully terminated arrhythmia. We discuss the possibilities to overcome failed conventional radiofrequency VT ablation of a septal focus.     

Multiple arrhythmogenic substrate for tachycardia in a patient with frequent palpitations

We report a 26-year-old woman with frequent episodes of palpitation and dizziness. Resting electrocardiography showed no evidence of ventricular preexcitation. During electrophysiologic study, a concealed right posteroseptal accessory pathway was detected and orthodromic atrioventricular reentrant tachycardia incorporating this pathway as a retrograde limb was reproducibly induced. After successful ablation of right posteroseptal accessory pathway, another tachycardia was induced using a concealed right posterolateral accessory pathway in tachycardia circuit. After loss of retrograde conduction of second accessory pathway with radiofrequency ablation, dual atrioventricular nodal physiology was detected and typical atrioventricular nodal reentrant tachycardia was repeatedly induced. Slow pathway ablation was done successfully. Finally sustained self-terminating atrial tachycardia was induced under isoproterenol infusion but no attempt was made for ablation. During 8-month follow-up, no recurrence of symptoms attributable to tachycardia was observed.     

Transcatheter leadless permanent pacemaker in complex congenital heart disease with interrupted inferior vena cava: A challenging implantation

31 years lady with complete atrioventricular canal defect, large primum atrial septal defect (ASD), inlet ventricular septal defect (VSD) and Eisenmenger syndrome, presented with atrial flutter and complete heart block. She was not suitable for corrective cardiac surgery and not yet indicated for heart-lung transplantation. She was advised single chamber permanent pacemaker and eventually Micra VR transcatheter leadless pacemaker was finalised for her. Transcatheter leadless pacemaker was deployed in her RV septum despite some unforeseen technical problems. This patient had intrahepatic interruption of IVC with Azygous continuation draining into SVC but this altered venovascular course was detected only fluoroscopically midway during the pacemaker implantation procedure and this was not detected in the preprocedural transthoracic echocardiography. This abnormal venous course was clearly demonstrated in the cardiac CT which was performed only after completion of the pacemaker implantation procedure in this patient. The technical challenges encountered mainly were mostly during the manipulation of the 27F delivery catheter of Micra through this altered cardiovascular anatomy via transfemoral approach and also due to the presence of septal defects. Thus, transcatheter leadless permanent pacemaker was implanted successfully through transfemoral access in this complex congenital heart disease with interrupted IVC and azygous continuation. Besides transthoracic echocardiography, it may be better to perform transesophageal echocardiography or even preferably radiological imaging like cardiac CT or MRI prior to transcatheter leadless pacemaker implantation in patients with complex congenital heart disease to understand the cardiovascular anatomy and plan the procedure.     

Unusual findings of asymmetrical septal hypertrophy associated with calcification of the interventricular septum: Case report

A patient with aortic regurgitation, stenosis, and calcification of the septum is reported. Results of echocardiography revealed asymmetrical septal hypertrophy without other features of idiopathic hypertrophic subaortic stenosis. There was no subaortic obstruction evident on cardiac catheterization and angiography. This case serves to emphasize that calcification of the interventricular septum is another possible cause of asymmetrical septal hypertrophy.     

The role of the septal reset in creating a youthful eyelid-cheek complex in facial rejuvenation

Resetting of the septum orbitale over the orbital rim, or "septal reset," is the latest step in achieving periorbital rejuvenation in composite rhytidectomy. The first significant step was the addition of orbicularis repositioning to conventional lateral vector deep plane rhytidectomy, followed by orbital fat preservation using the arcus marginalis release and fat transposition over the orbital rim. Those early procedures have been further refined to include the zygomaticus muscles with the orbicularis oculi in the composite flap, or zygorbicular cheek flap, and a septal reset. The septum orbitale reset has distinct advantages over transposition of orbital fat alone, as it creates a firmer undersurface for the lower eyelid. This maneuver will create a truly youthful lower eyelid-cheek complex, as the normal concave aging skeletonization of the periorbit is transformed to a convex contour of youth. The effectiveness of this operation can be demonstrated in most variations of human anatomy, whether congenital or iatrogenic, allowing the plastic surgeon to utilize the septal reset in virtually every patient undergoing and desiring a harmonious facial rejuvenation.     

Cooperation between the PDGF receptors in cardiac neural crest cell migration

Neural crest cells (NCCs) are essential components of the sympathetic nervous system, skin, craniofacial skeleton, and aortic arch. It has been known for many years that perturbation of migration, proliferation, and/or differentiation of these cells leads to birth defects such as cleft palate and persistent truncus arteriosus (PTA). Previously, we had shown that disruption of the platelet-derived growth factor receptor (PDGFR) alpha in NCCs resulted in defects in craniofacial and aortic arch development, the latter with variable penetrance. Because we observed ventricular septal defects in embryos that are null for the PDGFRbeta, we hypothesized that both PDGF receptors are involved in NCC formation. Here, we show that both receptors are expressed in cardiac NCCs and that the combined loss of the PDGFRalpha and PDGFRbeta in NCCs resulted in NCC-related heart abnormalities, including PTA and a ventricular septal defect (VSD). Using NCC lineage tracing, we observed that loss of PDGF receptor signaling resulted in reduced NCCs in the conotruncus region, leading to defects in aortic arch septation. These results indicate that while PDGFRalpha plays a predominant role in NCC development, the PDGFRbeta is expressed by and functions in cardiac NCCs. Combined PDGF receptor signaling is required for sufficient recruitment of cardiac NCCs into the conotruncal region and for formation of the aortico-pulmonary and ventricular septum.     

Antegrade or Retrograde Accessory Pathway Conduction: Who Dies First?

A 36 year-old man with Wolff Parkinson White syndrome due to a left-sided accessory pathway (AP) was referred for catheter ablation. Whether abolition of antegrade and retrograde AP conduction during ablation therapy occurs simultaneously, is unclear. At the ablation procedure, radiofrequency delivery resulted in loss of preexcitation followed by a short run of orthodromic tachycardia with eccentric atrial activation, demonstrating persistence of retrograde conduction over the AP after abolition of its antegrade conduction. During continued radiofrequency delivery at the same position, the fifth non-preexcitated beat failed to conduct retrogradely and the tachycardia ended. In this case, antegrade AP conduction was abolished earlier than retrograde conduction.     

A subpopulation of apoptosis-prone cardiac neural crest cells targets to the venous pole: multiple functions in heart development?

A well-described population of cardiac neural crest (NC) cells migrates toward the arterial pole of the embryonic heart and differentiates into various cell types, including smooth muscle cells of the pharyngeal arch arteries (but not the coronary arteries), cardiac ganglionic cells, and mesenchymal cells of the aortopulmonary septum. Using a replication-incompetent retrovirus containing the reporter gene LacZ, administered to the migratory neural crest of chicken embryos, we demonstrated another population of cardiac neural crest cells that employs the venous pole as entrance to the heart. On the basis of our present data we cannot exclude the possibility that precursors of these cells might not only originate from the dorsal part of the posterior rhombencephalon, but also from the ventral part. These NC cells migrate to locations surrounding the prospective conduction system as well as to the atrioventricular (AV) cushions. Concerning the prospective conduction system, the tagged neural crest cells can be found in regions where the atrioventricular node area, the retroaortic root bundle, the bundle of His, the left and right bundle branches, and the right atrioventricular ring bundle are positioned. The last area connects the posteriorly located AV node area with the retroaortic root bundle, which receives its neural crest cells through the arterial pole in concert with the cells giving rise to the aortopulmonary septum. The NC cells most probably do not form the conduction system proper, as they enter an apoptotic pathway as determined by concomitant TUNEL detection. It is possible that the NC cells in the heart become anoikic and, as a consequence, fail to differentiate further and merely die. However, because of the perfect timing of the arrival of crest cells, their apoptosis, and a change in electrophysiological behavior of the heart, we postulate that neural crest cells play a role in the last phase of differentiation of the cardiac conduction system. Alternatively, the separation of the central conduction system from the surrounding working myocardium is mediated by apoptotic neural crest cells. As for the presence of NC cells in both the outflow tract and the AV cushions, followed by apoptosis, a function is assigned in the muscularization of both areas, resulting in proper septation of the outflow tract and of the AV region. Failure of normal neural crest development may not only play a role in cardiac outflow tract anomalies but also in inflow tract abnormalities, such as atrioventricular septal defects.     

Vascular anatomy of the gills of the stingarees Urolophus mucosus and U. paucimaculatus (Urolophidae,Elasmobranchii)

The branchial vascular anatomy of Urolophus mucosus and U. paucimaculatus was studied by scanning electron microscopical examination of critical-point-dried tissue or of vascular corrosion casts. The vasculature could be divided into arterioarterial and arteriovenous pathways, which channel the flow of blood through the gills. The arterioarterial pathway consists of an afferent branchial artery which gives rise to afferent distributing arteries that run through the tissues of the interbranchial septum and supply the afferent filament arteries of several filaments. Afferent filament arteries open regularly into a corpus cavernosum in the core of the filament; unlike other elasmobranchs no septal corpora cavernosa are found. At the tip of the filament, channels of the corpus cavernosum connect to a channel which passes across the distal end of the filament from afferent to efferent side. This channel always connects to the afferent filament artery, and in many filaments it connects to the efferent filament artery as well. In addition, a vascular arcade connects all the afferent filament arteries along the entire length of each hemibranch. The filament corpus cavernosum supplies the secondary lamellae. The lamellae drain into efferent lamellar arterioles which in turn drain into the efferent filament artery and the efferent branchial artery. The vascular anatomy of the arteriovenous pathway is similar to that described in other elasmobranchs and consists of arteriovenous anastomoses, found only arising from efferent arterial circulation, and the venolymphatic system, which is composed of the central venous sinus and the companion vessels.     

The influence of damage to the septum pellucidum on the effects of electrical stimulation of the hypothalamus in rabbits]

In chronic experiments the influences of septal lesions on the behavioural emotional effects of electrical stimulation of various hypothalamic nuclei were investigated. The total ablation of the septum caused irreversible increase of the lateral hypothalamus self-stimulation and reversal of the negative emotional responses (escape--avoidance) to the medial hypothalamus stimulation into the positive self-stimulation behaviour. When the septal ablation was only partial, involving mainly the medial nucleus, effects were weaker and lasted only 2-3 days after the surgery. The role of the septum in the septohippocampal behavioural inhibition system (J. Gray) is discussed.