Anatomy of the Atrioventricular Junction, Atrioventricular Grooves, and Accessory Pathways.

Accessory pathways that bypass all or part of the normal atrioventricular conduction system traverse the atrioventricular junction. The atrioventricular junction comprises of a limited septal component and much more extensive right and left parietal components. Its composition forms a plane of insulation between atrial and ventricular myocardium, preventing direct continuity between them. Typical accessory atrioventricular pathways located anywhere along the atrioventricular junction are muscle bundles or may involve muscle around the walls of coronary sinus aneurysms or coronary veins. Increasingly, variants or unusual accessory pathways, some involving an accessory node, are reported in clinical studies.

Arrhythmias Involving Variants of Accessory Pathways.

In some cases, atrioventricular reentrant arrhythmias are sustained by accessory pathways with peculiar electrophysiologic properties related to their specific anatomy. Most of these fibers, which may be responsible for variants of ventricular preexcitation, show decremental conduction properties due to a nodelike aspect or a peculiar tortuous anatomic route across the atrioventricular groove. Moreover, some fibers do not actively sustain any reentrant circuit and can be only involved as bystander in other arrhythmias. Although rare, these accessory pathway variants should be properly diagnosed using noninvasive and invasive methods to guide catheter ablation procedures when needed.

Ablation of Accessory Pathways with Challenging Anatomy.

Although catheter ablation of accessory pathways is deemed highly safe and effective, peculiar location of these pathways might lead to complex and potentially hazardous procedures requiring ablation in anatomic regions such as para-Hisian area, coronary sinus, and epicardial surface. The electrophysiologist should know these possible scenarios to plan the best strategy for safe and effective ablation of these uncommon accessory pathways.

Examination of pathologic features of the right atrioventricular groove in hearts with Ebstein anomaly and correlation with arrhythmias.

BACKGROUND: Catheter ablation of accessory pathways (APs) in patients with Ebstein anomaly (EA) has a higher recurrence rate than in subjects with normal hearts. Anatomic features could account for suboptimal ablation outcomes. OBJECTIVE: The purpose of this study was to examine the right atrioventricular (AV) groove in autopsy hearts with EA, correlate with clinical data, and identify features relevant for catheter ablation. METHODS: Thirty-three specimens with EA from our Cardiac Registry were examined. The right AV groove was inspected for gross anatomic features. Limited microscopy was performed on selected specimens. Premortem clinical data were correlated with anatomic findings. RESULTS: A prominent ridge along the right AV groove was seen in 15 of 33 specimens (45%). Ten specimens had a clinical history of AP (AP+). The extent of ventricular atrialization did not differ between AP+ and AP- groups (64 ± 63 mm/m2 vs 76 ± 42 mm/m2; P = .61), nor did the presence of visible macroscopic AV tissue connections (45% vs 51%; P = .68). The single item that differed was the presence of an AV groove ridge itself, which was significantly more common in the AP+ group (70% vs 21%; P = .03). Microscopy of ridge tissue revealed a muscular bundle in 1 AP+ specimen penetrating deep into the fibrous AV annulus that was suggestive of an AP, although complete muscular continuity was not verified in the limited sections available for examination. CONCLUSION: A prominent ridge along the inferior right AV groove is a common feature in EA and correlates with clinical history of AP. It presents a potential obstacle to catheter ablation and may contribute to recurrence rate.

Three-dimensional histologic reconstruction of remnant functional accessory atrioventricular myocardial connections in a case of Wolff-Parkinson-White syndrome.

Myocardial bundles working as accessory pathways in Wolff-Parkinson-White (WPW) syndrome are generally tiny tissues, so elucidating the culprit histology of atrioventricular (AV) myocardial connections requires careful serial sectioning of the AV junction. We performed a postmortem examination of accessory AV myocardial connections in an 84-year-old man who died from pneumonia 20 years after surgical cryoablation for WPW syndrome. Three-dimensional reconstruction images of serial histologic sections revealed accessory AV connections between the atrial and ventricular myocardium in the vicinity of the cryoablation scar. The remnant myocardial bridge was 4 mm wide and made up of multiple discontinuous fibers. This case was informative in that it provided for visualization of the histologic morphology of a remnant bundle of Kent.

Experimental, Pathologic, and Clinical Findings of Radiofrequency Catheter Ablation of Para-Hisian Region From the Right Ventricle in Dogs and Humans.

BACKGROUND: Ablation of para-Hisian accessory pathway (AP) poses high risks of atrioventricular block. We developed a pacing technique to differentiate the near-field (NF) from far-field His activations to avoid the complication. METHODS AND RESULTS: Three-dimensional mapping of the right ventricle was performed in 15 mongrel dogs and 23 patients with para-Hisian AP. Using different pacing outputs, the NF- and far-field His activation was identified on the ventricular aspect. Radiofrequency application was delivered at the NF His site in 8 (group 1) and the far-field His site in 7 dogs (group 2), followed by pathologic examination after 14 days. NF His activation was captured with 5 mA/1 ms in 10 and 10 mA/1 ms in 5 dogs. In group 1, radiofrequency delivery resulted in complete atrioventricular block in 3, right bundle branch block with HV (His-to-ventricular) interval prolongation in 1, and only right bundle branch block in 2 dogs, whereas no changes occurred in group 2. Pathologic examination in group-1 dogs showed complete or partial necrosis of the His bundle in 4 and complete necrosis of the right bundle branch in 5 dogs. In group 2, partial necrosis in the right bundle branch was found only in 1 dog. Using this pacing technique, the APs were 5.7±1.2 mm away from the His bundle located superiorly in 20 or inferiorly in 3 patients. All APs were successfully eliminated with 1 to 3 radiofrequency applications. No complications and recurrence occurred during a follow-up of 11.8±1.4 months. CONCLUSIONS: Differentiating the NF His from far-field His activations led to a high ablation success without atrioventricular block in para-Hisian AP patients.

Accessory pathway location affects brain natriuretic peptide level in patients with Wolff-Parkinson-White syndrome.

PURPOSE: The purpose of this study was to investigate the relationship between the accessory pathway location and brain natriuretic peptide (BNP) level in patients with Wolff-Parkinson-White (WPW) syndrome. METHODS: We divided 102 WPW syndrome patients with normal left ventricular systolic function into four groups: those with manifest right (MR, n = 14), manifest septal (MS, n = 11), manifest left (ML, n = 30), and concealed (C, n = 47) accessory pathways. BNP level and electrophysiological properties, including difference in timing of the ventricular electrogram between the His bundle area and the distal coronary sinus area (His-CS delay), which indicate intraventricular dyssynchrony, were compared. RESULTS: BNP levels (pg/dl) were higher in the MR and MS groups than in the ML and C groups (MR, 64 ± 58; MS, 55 ± 45; ML, 17 ± 15; C, 25 ± 21; P < 0.001). AV intervals (ms) were shorter in the MR and MS groups than in the ML and C groups (MR, 76 ± 16; MS, 83 ± 6; ML, 101 ± 19; C, 136 ± 20; P < 0.001). His-CS delay (ms) was longer in the MR group than in the other groups (MR, 50 ± 15; MS, 21 ± 7; ML, 23 ± 10; C, 19 ± 8; P < 0.001). The AV interval (P < 0.01) and the His-CS delay (P < 0.001) were negatively and positively correlated, respectively, with the BNP level. CONCLUSION: Anterograde conduction with a right or septal accessory pathway increased the BNP level in WPW syndrome patients with normal cardiac function.

Catheter ablation of accessory pathways near the coronary sinus: value of defining coronary arterial anatomy.

BACKGROUND: Accessory pathways can lie near or within the coronary sinus (CS). Radiofrequency catheter ablation of accessory pathways is a well-established treatment option, but this procedure can cause damage to adjacent coronary arteries. OBJECTIVE: The purpose of this study was to evaluate the anatomic relationship between the coronary arteries and the CS. METHODS: Retrospective data of patients who underwent catheter ablation of supraventricular tachycardia between June 2011 and August 2013 was reviewed. In addition, detailed analysis of coronary computed tomographic angiography (CTA) data from 50 patients was performed. RESULTS: Between June 2011 and August 2013, 427 patients underwent catheter ablation of supraventricular tachycardia, of whom 105 (age 28 ± 17 years, 60% male) had accessory pathway-mediated tachycardia. Of these, 23 patients had accessory pathways near the CS, and 60% (N = 14) underwent concurrent coronary angiography. In 4 patients, the posterolateral (inferolateral) branch (PLA) of the right coronary artery was in close proximity to the CS, and 2 patients (18%) had stenosis of the PLA at the site of ablation. On CTA at their closest proximity, the PLA was 1.9 ± 1.3 mm and the left circumflex artery (LCx) was 2.0 ± 0.8 mm from the body of the CS, in right and left coronary artery-dominant patients, respectively. CS ostium and PLA were 3.6 ± 1.9 mm apart. In left-dominant patients, LCx and CS ostium were 3.8 ± 1.2 mm apart. CONCLUSION: The PLA and LCx are in close proximity to the anteroinferior aspect of the CS ostium and proximal CS. The relationship of the CS and coronary arteries should be evaluated before ablation at these sites.

Sudden death of an infant with cardiac, nervous system and genetic involvement--a case report.

We present a case of sudden death of a 1-month-old male infant with heart, brainstem and genetic polymorphism involvement. Previously considered quite healthy, the child died suddenly and unexpectedly during sleep. The autopsy protocol included an in-depth anatomopathological examination of both the autonomic nervous system and the cardiac conduction system, and molecular analysis of the serotonin transporter gene promoter region, in which a specific genetic condition seems to be associated with sudden infant death. Histological examination revealed the presence of congenital cardiac alterations (hypertrophic cardiomyopathy and an accessory Mahaim fiber in the cardiac conduction system), severe hypodevelopment of all the raphe nuclei and a heterozygous genotype L/S related to the serotonin transporter gene. The sudden death of this infant was the unavoidable outcome of a complex series of congenital anomalies, each predisposing to SIDS. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3480540091031788.

Ablation of left-sided accessory pathways with atrial insertion away from the mitral annulus using an electroanatomical mapping system.

BACKGROUND: A left-sided accessory pathway (AP) with atrial insertion away from the mitral annulus (MA) may result in difficulty or failed ablation along the MA. We report our initial experience of ablating this rare form of AP by a 3-dimensional electroanatomical mapping system (CARTO). METHODS: From January 2007 to August 2011, 29 patients with left-sided APs who failed previous ablations in other centers were enrolled in this study. Left atrium (LA) was reconstructed during orthodromic atrioventricuar reentry tachycardias (AVRTs) or ventricle pacing by using a 3-dimensional electroanatomical mapping system. The AP atrial insertion was defined as the earliest retrograde atrial activation and successful ablation of the AP at the site. RESULTS: Among the 29 patients who had failed previous ablation, 7 patients were found to have atrial insertions away from the MA. Out of the 7 patients, atrial insertions were at the base of the LA appendage in 5 patients and at the anterior roof of LA in 2 patients. Ablation at the atrial insertion successfully abolished AP conduction. The mean distance between the atrial insertion sites and the MA was 24.9 ± 4.9 mm. No patients reported recovered AP conduction or recurrent tachycardias after at least 12-month follow-up. CONCLUSIONS: Left-sided APs may have atrial insertions away from the MA. By using the CARTO system, atrial insertions can be reliably identified and ablated.

LV dyssynchrony as assessed by phase analysis of gated SPECT myocardial perfusion imaging in patients with Wolff-Parkinson-White syndrome.

PURPOSE: The purpose of this study was to evaluate left ventricular (LV) mechanical dyssynchrony in patients with Wolff-Parkinson-White (WPW) syndrome pre- and post-radiofrequency catheter ablation (RFA) using phase analysis of gated single photon emission computed tomography (SPECT) myocardial perfusion imaging (MPI). METHODS: Forty-five WPW patients were enrolled and had gated SPECT MPI pre- and 2-3 days post-RFA. Electrophysiological study (EPS) was used to locate accessory pathways (APs) and categorize the patients according to the AP locations (septal, left and right free wall). Electrocardiography (ECG) was performed pre- and post-RFA to confirm successful elimination of the APs. Phase analysis of gated SPECT MPI was used to assess LV dyssynchrony pre- and post-RFA. RESULTS: Among the 45 patients, 3 had gating errors, and thus 42 had SPECT phase analysis. Twenty-two patients (52.4%) had baseline LV dyssynchrony. Baseline LV dyssynchrony was more prominent in the patients with septal APs than in the patients with left or right APs (p < 0.05). RFA improved LV synchrony in the entire cohort and in the patients with septal APs (p < 0.01). CONCLUSION: Phase analysis of gated SPECT MPI demonstrated that LV mechanical dyssynchrony can be present in patients with WPW syndrome. Septal APs result in the greatest degree of LV mechanical dyssynchrony and afford the most benefit after RFA. This study supports further investigation in the relationship between electrical and mechanical activation using EPS and phase analysis of gated SPECT MPI.

Two-dimensional speckle-tracking echocardiography for atrioventricular accessory pathways persistent ventricular pre-excitation despite successful radiofrequency ablation.

AIMS: The present study was undertaken to investigate the concordance between longitudinal two-dimensional (2D)-speckle-tracking data and endocardial mapping for localizing atrioventricular accessory pathways (AP), and whether longitudinal 2D-speckle-tracking imaging accurately identifies the contractile abnormalities associated with AP and the effect of radiofrequency ablation. METHODS AND RESULTS: Echocardiograms were repeated twice in 40 patients with Wolff-Parkinson-White (WPW) syndrome (before and early after ablation) and in 40 healthy controls to obtain longitudinal 2D strain and strain rate data. The site of ablation was considered as the gold standard for the AP localization. While control patients had a homogeneous strain pattern, all but two patients with WPW had an abnormal deformation pattern with three peaks in one or two basal contiguous segments: an early peak concomitantly with the delta wave followed by a systolic and a post-sytolic one. The rapid increase in LV longitudinal deformation within the basal pre-excited zone resulted in a pre-systolic peak strain rate at the beginning of the delta wave by SR imaging that was not found in controls. The early basal contraction spread towards the mid-ventricle before merging with the normal activated segments in 15 patients (39%). Contractile abnormalities were no more than one adjacent segment different compared with the AP ablation site in all these 38 patients. Regional strain was impaired in the pre-excited areas especially in AP localized in the interventricular septum. The abnormal deformation pattern persisted in 16 (42%) patients despite successful radiofrequency ablation. However, the difference in the regional strain between WPW patients and controls did not remain after ablation. CONCLUSION: Longitudinal 2D-speckle-tracking data accurately match with endocardial mapping findings for localizing AP. Longitudinal 2D-speckle-tracking imaging accurately identifies AP-associated contractile abnormalities. Longitudinal 2D-speckle-tracking identifies persistence of local ventricular pre-excitation immediately after successful ablation.