Risk assessment in patients with symptomatic and asymptomatic pre-excitation.

AIMS: Controversy remains as to whether the exercise stress test (EST) is sufficient for risk evaluation in patients with pre-excitation. This study aims to clarify the usefulness of EST in risk stratification in both asymptomatic and symptomatic patients presenting with pre-excitation. METHODS AND RESULTS: This prospective study includes consecutive asymptomatic and symptomatic patients with pre-excitation referred for risk assessment. All participants performed an incremental EST (bicycle) prior to an electrophysiology study (EPS). Primary data from the EST included loss of pre-excitation during exercise, and primary data from the EPS included the measurement of accessory pathway effective refractory period (APERP), shortest pre-excited RR interval (SPERRI), and inducible arrhythmia with the use of a beta-adrenergic receptor agonist if deemed necessary. One hundred and sixty-four patients (59 asymptomatic, 105 symptomatic) completed an EST and EPS. Forty-five patients (27%) demonstrated low-risk findings on EST, of which 19 were asymptomatic and 26 were symptomatic. Six patients with low-risk EST findings had SPERRI/APERP ≤ 250 ms at EPS, and two of them were asymptomatic. The sensitivity, specificity, positive predictive value, negative predictive value (NPV), and accuracy of low-risk EST for excluding patients with SPERRI/APERP ≤ 250 ms were 40, 91, 87, 51, and 60%, respectively. The number of patients with inducible arrhythmia at EPS was similar in the asymptomatic (36, 69%) and symptomatic (73, 61%) groups. CONCLUSION: Sudden loss of pre-excitation during EST has a low NPV in excluding high-risk APs. The EPS with the use of isoproterenol should be considered to accurately assess the risk of patients with pre-excitation regardless of symptoms (ClinicalTrials.gov Identifier: NCT03301935).

Ventricular preexcitation of every other beat or ventricular bigeminy or both?

Bigeminy is a cardiac arrhythmia in which there is a single ectopic beat, or irregular heartbeat, following each regular heartbeat. This case demonstrates the diagnostic challenges that can be encountered while interpreting ECGs of patients with "apparent ventricular bigeminy'.

EPM algorithm: A stepwise approach to accessory pathway localization in ventricular pre-excitation.

BACKGROUND: Accurate estimation of accessory pathway (AP) localization in patients with ventricular pre-excitation or Wolff-Parkinson-White (WPW) syndrome remains a diagnostic challenge. Existing algorithms have contributed significantly to this area, but alternative algorithms can offer additional perspectives and approaches to AP localization. OBJECTIVE: This study introduces and evaluates the diagnostic accuracy of the EPM algorithm in AP localization, comparing it with established algorithms Arruda and EASY. METHODS: A retrospective analysis was conducted on 138 patients from Hospital São Paulo who underwent catheter ablation. Three blinded examiners assessed the EPM algorithm's diagnostic accuracy against the Arruda and EASY algorithms. The gold standard for comparison was the radioscopic position of the AP where radiofrequency ablation led to pre-excitation disappearance on the ECG. RESULTS: EPM showed a diagnostic accuracy of 51.45%, closely aligning with Arruda (53.29%) and EASY (44.69%). Adjacency accuracy for EPM was 70.67%, with Arruda at 66.18% and EASY at 72.22%. Sensitivity for EPM in distinguishing left vs. right APs was 95.73%, with a specificity of 74.33%. For identifying septal vs. lateral right APs, EPM sensitivity was 82.79% with a specificity of 46.15%. These measures were comparable to those of Arruda and EASY. Inter-observer variability was excellent for EPM, with Kappa statistics over 0.9. CONCLUSION: The EPM algorithm emerges as a reliable tool for AP localization, offering a systematic approach beneficial for therapeutic decision-making in electrophysiology. Its comparable diagnostic accuracy and excellent inter-observer variability underscore its potential clinical applicability. Future research may further validate its efficacy in a broader clinical setting.

Pregnancy outcomes in patients complicated with pre-excitation syndrome.

INTRODUCTION: Pregnant women with pre-excitation syndrome are more likely to develop supraventricular tachycardia (SVT) during pregnancy and delivery, leading to an increased risk of adverse events. METHOD: This was a retrospective study of 309 pregnancies in 280 women (29 women had two pregnancies in this series) with pre-excitation syndrome who delivered at West China Second University Hospital from June 2011 to October 2021. All the 309 pregnant women with pre-excitation syndrome were divided into SVT and non-SVT groups to analyze the cardiac and obstetric complications. RESULTS: Among the included pregnant women in the past 10 years, the prevalence of pre-excitation syndrome was 0.24% (309/127725). There were 309 cases with pre-excitation syndrome in all hospitalized pregnant women. Among them, 62 (20.1%, 62/309) had a history of SVT. In the 62 cases with SVT during pregnancy, 22 (35.5%) cases had a history of SVT. Gestational diabetes mellitus was associated with SVT during pregnancy. The cesarean section rate was 88.7% in the SVT group, which was significantly higher than that in the non-SVT group (64.8%) (P < 0.001). Cases with SVT during pregnancy had more cardiac and obstetric complications. Four fetal deaths were recorded in the SVT group. Additionally, 29 women experienced two pregnancies during the study period, among whom, five received radiofrequency ablation after the first delivery and obtained better outcomes in the second pregnancy. CONCLUSION: The adverse outcomes such as cardiac complications, maternal and fetal complications (PROM, prematurity, SGA, fetal distress, etc.) in pregnant women with pre-excitation syndrome were closely related to SVT, with possible risk factors including history of SVT before pregnancy, cardiac function, heart organic abnormalities, and gestational diabetes mellitus.

Iatrogenic cardiomyopathy in patients with manifest right supero-paraseptal accessory pathways.

INTRODUCTION: We describe two patients with right supero-paraseptal accessory pathway (SPAP) who developed left ventricular dysfunction associated with an increased degree of ventricular pre-excitation and frequent orthodromic reciprocating tachycardia (ORT) due to worsening atrioventricular (AV) node conduction. METHODS AND RESULTS: Case 1: 48-year-old female with a history of normally functioning mechanical mitral valve, CABG, and ventricular pre-excitation that worsened after her open heart surgery. She presented with frequent palpitations with documented supraventricular tachycardia (SVT) and found to have a new left ventricular dysfunction with decrease in left ventricular ejection fraction (LVEF) from 55% to 46% with dyssynchrony. An electrophysiological study confirmed a right SPAP and ORT. The pathway was successfully ablated from the antegrade approach after careful mapping. After ablation and 6-month follow up echocardiogram showed improvement of EF to 54% and the LV dyssynchrony resolved. Case 2: 51-year-old male with a history of frequent SVT with recent unsuccessful ablations that resulted in worsening ventricular pre-excitation, more frequent SVT, and new left ventricular dysfunction (LVEF from 60% to 40%). He was started on amiodarone which resulted in significant sinus bradycardia, intermittent ventricular pre-excitation, and first degree AV block with significant increase in ORT events. His electrophysiology study confirmed SPAP which was successfully ablated from the antegrade approach after careful mapping. After 1 month, follow-up echocardiogram showed an improved ejection fraction to 60%. CONCLUSION: Left ventricular dysfunction due to dyssynchrony and symptomatic frequent ORT of right SPAP can develop in the setting of new iatrogenic diminished AV node conduction. Successful ablation will result in LV function recovery to baseline.

Intermittent ventricular pre-excitation in symptomatic adults: Always a marker of low risk?

BACKGROUND: Intermittent ventricular pre-excitation was considered a low-risk marker for sudden death. However, to date, some studies do not exclude the existence of accessory pathways (APs) with high-risk intermittent antegrade conductive properties. According to current European Guidelines, high-risk features of APs are antegrade pathway conduction ≤250 ms in baseline or during the adrenergic stimulus, inducibility of atrioventricular reciprocating tachycardias (AVRT), inducibility of pre-excited atrial fibrillation (AF), and presence of multiple APs. For all of these transcatheter ablation is recommended. The aim of our study was to evaluate the existence of differences in risk characteristics between patients with intermittent pre-excitation (IPX) and those with persistent pre-excitation (PPX), from a sample of adults with ventricular pre-excitation and symptoms like palpitations. METHODS: 293 adults [IPX: 51 (17.4%); PPX: 242 (82.6%)] underwent electrophysiological study and then catheter ablation of their APs if arrhythmia inducibility (AVRT/AF) was noted, or, conversely, if it was appreciated a fast AP antegrade conduction, in baseline or during intravenous isoproterenol infusion, or if multiple APs were detected. RESULTS: There were no statistically significant differences in demographic characteristics (age and gender), AVRT/AF inducibility, antegrade conductive properties, the prevalence of multiple APs, and APs locations between IPX and PPX patients. CONCLUSIONS: In our study, patients with IPX did not show significant differences in clinical and electrophysiological features versus PPX patients.

Left ventricular dysfunction in a child with asymptomatic pre-excitation: Cure with catheter ablation.

Wolff-Parkinson-White syndrome, is known to cause left ventricular dysfunction or dilated cardiomyopathy secondary to sustained tachycardia in infants and children. However, left ventricular dysfunction secondary to pre-excitation related abnormal ventricular activation has been reported in a limited number of cases. This condition should be recognized early, as catheter ablation of the accessory pathway can permit rapid ventricular function improvement. In this paper, we present a 2.5-year-old patient diagnosed with tachycardia-free Wolff-Parkinson-White syndrome with a right free wall accessory pathway and depressed cardiac function, whose left ventricular function is completely restored after successful catheter cryoablation.

Wolff-Parkinson-White syndrome and dilated cardiomyopathy: Not only an electrical issue?

The present case describes a dilated cardiomyopathy associated with both antidromic and orthodromic atrio-ventricular reentrant tachycardias supported by multiple right accessory pathways. Both right accessory pathways were successfully eliminated by catheter ablation and the patient progressively recovered during the follow up. The following etiologies might be involved: 1) primitive dilated cardiomyopathy (or post-inflammatory); 2) septal dyssinchrony due to ventricular pre-excitation; 3) tachycardiomyopathy.

[Clinical characteristics of Danon disease].

Objective: To review the clinical data of 7 patients with Danon disease and analyze their clinical characteristics. Methods: The medical records of 7 patients with Danon disease, who were hospitalized in Peking Union Medical College Hospital of Chinese Academy of Medical Sciences from April 2008 to July 2021, were reviewed and summarized, of which 6 cases were diagnosed as Danon disease by lysosomal-associated membrane protein-2 (LAMP-2) gene mutation detection and 1 case was diagnosed by clinicopathological features. Clinical manifestations, biochemical indexes, electrocardiogram, echocardiography, skeletal muscle and myocardial biopsy and gene detection results were analyzed, and patients received clinical follow-up after discharge. Results: Six patients were male and average age was (15.4±3.5) years and the average follow-up time was (27.7±17.0) months. The main clinical manifestations were myocardial hypertrophy (6/7), decreased myodynamia (2/7) and poor academic performance (3/7). Electrocardiogram features included pre-excitation syndrome (6/7) and left ventricular hypertrophy (7/7). Echocardiography examination evidenced myocardial hypertrophy (6/7), and left ventricular dilatation and systolic dysfunction during the disease course (1/7). The results of skeletal muscle biopsy in 6 patients were consistent with autophagy vacuolar myopathy. Subendocardial myocardial biopsy was performed in 3 patients, and a large amount of glycogen deposition with autophagosome formation was found in cardiomyocytes. LAMP-2 gene was detected in 6 patients, and missense mutations were found in all these patients. During the follow-up period, implantable cardioverter defibrillator implantation was performed in 1 patient because of high atrioventricular block 4 years after diagnosis, and there was no death or hospitalization for cardiovascular events in the other patients. Conclusion: The main clinical manifestations of Danon disease are cardiomyopathy, myopathy and mental retardation. Pre-excitation syndrome is a common electrocardiographic manifestation. Autophagy vacuoles can be seen in skeletal muscle and myocardial pathological biopsies. LAMP-2 gene mutation analysis is helpful in the diagnose of this disease.

Positive QRS complex in limb lead 2 with negative QRS in lead 3 on surface electrocardiogram: A novel predictor for anterior location of right-sided accessory pathways (10-1 o'clock on tricuspid annulus).

BACKGROUND: Localization of atrioventricular accessory pathways (AP) from Electrocardiogram (ECG) is crucial for successful ablation. We analyzed the value of limb lead 2 versus 3 QRS vector discordance on surface ECG among right-sided pathways. METHODS: Data from consecutive patients undergoing successful ablation of manifest AP were analyzed. They were categorized into two groups-Gr I: Endocardial ablation from anterior and anterolateral tricuspid annulus (TA, 10-1 o'clock, right anterolateral [RAL]); Gr II: Ablation outside this region (1-10 o'clock of TA). Inferior lead discordance (ILD) was defined as positive QRS complex (monophasic R, Rs) in lead 2 with negative/equiphasic QRS vector in lead 3 (rS, S, RS). Maximally pre-excited ECGs during electrophysiology study were compared for presence of ILD. RESULT: Among total 22 cases (Age 36 ± 18 years, 12 males), ILD was noted in 4/4 cases of Gr I. It was absent among 17/18 cases of right-sided AP in Gr II. The only case in Gr II having ILD was ablated near 8 o'clock (posterolateral). In contrast to the other four cases, aVF was negative, along with lead 3. A close differential was mid-septal AP (MSAP). However, the MSAP had absence of r in V1 and lead 2 having rS/RS complex in contrast to strongly positive QRS in RAL pathways. The sensitivity and specificity of ILD for RAL are 100% and 95%, respectively. The positive, negative predictive value, and accuracy are 80%, 100%, and 95%, respectively. CONCLUSION: Positive QRS complex in lead 2 with negative QRS in lead 3 in maximally pre-excited ECG is often predictive of anterior and anterolateral location among right-sided pathways.

Assessment of the physical performance in children with asymptomatic pre-excitation.

AIMS: Pre-excitation syndrome can lead to recurrent supraventricular tachycardias (SVTs) and carries a risk of sudden cardiac death (SCD). However, an underestimated consequence of antegrade conduction through an accessory pathway is fusion of intrinsic and accessory conduction that causes asynchronous activation and myocardial contraction that could be a cause for cardiac dysfunction and dilation. It is not known to what extent pre-excitation affects myocardial and physical performance in those patients. The aim of the study was to assess to what degree ventricular pre-excitation affects physical performance in children, using cardio-pulmonary exercise testing (CPET). METHODS AND RESULTS: The study group consisted of 30 asymptomatic children, aged 8-17 years, with pre-excitation and no history or documentation of SVT compared to 31 healthy controls matched according to sex and age. All patients underwent routine cardiology assessment and then CPET. Echocardiography showed there were no differences in the left ventricular size and function between the study and control group. During the CPET both, patients and controls achieved maximal effort. Patients in the study group showed significantly lower values of VO2max and anaerobic threshold when compared to controls. The most affected subgroup was patients with persistent pre-excitation throughout the exercise. CONCLUSIONS: Physical performance is affected in children with pre-excitation. This effect is stronger in patients with persistent delta wave observed throughout the exercise.

Miniseries 1-Part IV: How frequent are fasciculo-ventricular connections in the normal heart?

AIMS: Seeking to account for accessory atrioventricular conduction potentially leading to ventricular pre-excitation, Mahaim in the mid-20th century had described pathways between the atrioventricular conduction axis and the muscular ventricular septum. We aimed to look for such 'paraspecific' connections in adult human hearts. METHODS AND RESULTS: We serially sectioned 21 hearts, covering the triangle of Koch and the aortic root, and assessing the atrioventricular node, the penetration of the conduction axis, and the bundle branches in our search for fasciculo-ventricular connections. We also calculated the length of the non-branching bundle, and if present the origin of the fasciculo-ventricular connections. The non-branching bundle was 3.6 ± 1.7 mmin length, varying from 1.7 mm to 7.2 mm. Fasciculo-ventricular connections were found in more than half of the hearts, making direct contact with the muscular septum at an average of 3.5 ± 1.7 mm from the origin of the left bundle branch, with the site of origin varying from 1.1 mm to 5.5 mm from the first fascicle of the left bundle branch. In three hearts, additional fasciculo-fascicular connections were observed in the left bundle branch. Two loops were small, but one loop extended over 9.5 mm. CONCLUSION: We endorse the finding of Mahaim that fasciculo-ventricular pathways exist in most human hearts. We presume the identified connections had the capability of producing ventricular pre-excitation. More studies are needed to determine the potential clinical manifestations.

Miniseries 2-Septal and paraseptal accessory pathways-Part I: The anatomic basis for the understanding of para-Hisian accessory atrioventricular pathways.

AIMS: Although the anatomy of the atrioventricular conduction axis was well described over a century ago, the precise arrangement in the regions surrounding its transition from the atrioventricular node to the so-called bundle of His remain uncertain. We aimed to clarify these relationships. METHODS AND RESULTS: We have used our various datasets to examine the development and anatomical arrangement of the atrioventricular conduction axis, paying particular attention to the regions surrounding the point of penetration of the bundle of His. It is the areas directly adjacent to the transition of the atrioventricular conduction axis from the atrioventricular node to the non-branching atrioventricular bundle that constitute the para-Hisian areas. The atrioventricular conduction axis itself traverses the membranous part of the ventricular septum as it extends from the node to become the bundle, but the para-Hisian areas themselves are paraseptal. This is because they incorporate the fibrofatty tissues of the inferior pyramidal space and the superior atrioventricular groove. In this initial overarching review, we summarize the developmental and anatomical features of these areas along with the location and landmarks of the atrioventricular conduction axis. We emphasize the relationships between the inferior pyramidal space and the infero-septal recess of the subaortic outflow tract. The details are then explored in greater detail in the additional reviews provided within our miniseries. CONCLUSION: Our anatomical findings, described here, provide the basis for our concomitant clinical review of the so-called para-Hisian arrhythmias. The findings also provide the basis for understanding the other variants of ventricular pre-excitation.

Miniseries 2-Septal and paraseptal accessory pathways-Part III: Mid-paraseptal accessory pathways-revisiting bypass tracts crossing the pyramidal space.

The mid-paraseptal region corresponds to the portion of the pyramidal space whose right atrial aspect is known as the triangle of Koch. The superior area of this mid-paraseptal region is also para-Hisian, and is close to the compact atrioventricular node and the His bundle. The inferior sector of the mid-paraseptal area is unrelated to the normal atrioventricular conduction pathways. It is, therefore, a safe zone in which, if necessary, to perform catheter ablation. The middle part of the mid-paraseptal zone may, however, in some patients, house components of the compact atrioventricular node. This suggests the need for adopting a prudent attitude when considering catheter ablation in this area. The inferior extensions of the atrioventricular node, which may represent the substrate for the slow atrioventricular nodal pathway, take their course through the middle, and even the inferior, sectors of the mid-paraseptal region. In this review, we contend that the middle and inferior areas of the mid-paraseptal region correspond to what, in the past, was labelled by most groups as the 'midseptal' zone. We describe the electrocardiographic patterns observed during pre-excitation and orthodromic reciprocating tachycardia in patients with pathways ablated in the middle or inferior sectors of the region. We discuss the modification of the ventriculo-atrial conduction times during tachycardia after the development of bundle branch block aberrancy. We conclude that the so-called 'intermediate septal' pathways, as described in the era of surgical ablation, were insufficiently characterized. They should not be considered the surrogate of the 'midseptal' pathways defined using endocardial catheter electrode mapping.

Miniseries 2-Septal and paraseptal accessory pathways-Part II: Para-Hisian accessory pathways-so-called anteroseptal pathways revisited.

Surgeons, when dividing bypass tracts adjacent to the His bundle, considered them to be 'anteroseptal'. The area was subsequently recognized to be superior and paraseptal, although this description is not entirely accurate anatomically, and conveys little about the potential risk during catheter interventions. We now describe the area as being para-Hisian, and it harbours two types of accessory pathways. The first variant crosses the membranous septum to insert into the muscular ventricular septum without exiting the heart, and hence being truly septal. The second variant inserts distally in the paraseptal components of the supraventricular crest, and consequently is crestal. The site of ventricular insertion determines the electrocardiographic expression of pre-excitation during sinus rhythm, with the two types producing distinct patterns. In both instances, the QRS and the delta wave are positive in leads I, II, and aVF. In crestal pathways, however, the QRS is ≥ 140 ms, and exhibits an rS configuration in V1-2. The delta wave in V1-2 precedes by 20-50 ms the apparent onset of the QRS in I, II, III, and aVF. In the true septal pathways, the QRS complex occupies ∼120 ms, presenting a QS, W-shaped, morphology in V1-2. The delta wave has a simultaneous onset in all leads. Our proposed terminology facilitates the understanding of the electrocardiographic manifestations of both types of para-Hisian pathways during pre-excitation and orthodromic tachycardia, and informs on the level of risk during catheter ablation.

A Frequent Observation of Wolff-Parkinson-White Syndrome and Fasciculoventricular Pathways in Patients With Danon Disease.

BACKGROUND: Danon disease is typically associated with cardiomyopathy and ventricular pre-excitation. The study aimed to characterize the clinical profile of Danon disease, analyze electrocardiographic (ECG) and electrophysiologic features, and investigate their association with Wolff-Parkinson-White (WPW) syndrome and fasciculoventricular pathways (FVPs).Methods and Results:Clinical course, family history, ECG and electrophysiological data were collected from 16 patients with Danon disease. Over 0.4-8 years of follow up, 1 female patient died suddenly, and 5 male patients died of progressive heart failure by age 13-20 years. Family history analysis revealed that 3 mothers experienced hospitalization or death for heart failure at age 28-41 years. There was 100% penetrance for ECG abnormalities in 13 patients with original ECGs. Short PR intervals and delta waves were present in 9 and 8 patients, respectively. There were significant age-associated increases in the QRS complex width (r=0.556, P=0.048) and the number of leads with notched QRS (r=0.575, P=0.04). Four patients who underwent electrophysiological studies all had FVPs, and 2 of them still had left-side atrioventricular pathways. CONCLUSIONS: Danon disease causes a malignant clinical course characterized by early death caused by heart failure in both genders and progressive ECG changes as patients age. The pre-excited ECG pattern is related to FVPs and WPW, which is suggestive of extensive cardiac involvement.

The characteristics of pre-excitation syndrome concomitant with atrial tachyarrhythmia and the effect of radiofrequency ablation.

BACKGROUND: Wolff-Parkinson-White (WPW) concomitant with atrial tachyarrhythmia (ATA) has not been systemically characterized. METHODS: Detailed electroanatomical mapping of the right atrium (RA) and/or left atrium (LA) was performed using three-dimensional mapping and the accessory pathway (AP) was mapped. RESULTS: WPW syndrome with ATA was diagnosed in 11 patients (median age 60 years). The characteristic of unidirectional anterograde conduction over the AP was displayed in nine patients, six of whom were intermittent. Sustained atrial tachycardia, that is, counterclockwise atrial flutter (AFL) with a median tachycardia cycle length (TCL) of 225 (220-275) ms, was mapped in eight patients; furthermore, "figure 8" right atrial reentry was mapped with TCL 250 ms in one patient with a surgical history of ventricular septal defect repair. The remaining two patients underwent mitral annulus-dependent AT after paroxysmal atrial fibrillation (PAF) ablation and LA micro-reentry AT, respectively. In four patients, the location of the APs was left posterior. Left-lateral APs were identified in four patients. The locations of the APs in the remaining three patients were the right posterior and middle septum. All ATAs and APs were successfully ablated. After a median follow-up of 37 (15-72) months, no anterograde conduction over the AP was recorded, new onset of PAF was recorded in three patients, and all of them underwent circumferential pulmonary vein isolation. CONCLUSIONS: WPW with concomitant ATA frequently had continuous anterograde conduction over the AP with a rapid ventricular rate. Most WPWs displayed the characteristic of unidirectional anterograde conduction.

Distal type of nodo-ventricular pathway: Unique electrophysiological characteristics mimicking fasciculo-ventricular pathway.

Fasciculo-ventricular and nodo-ventricular pathways (FVP and NVP) are rare preexcitation variants. Normally, NVP is electrophysiologically different from FVP. We describe a unique type of NVP emerging from the distal part of the slow pathway, designated as "distal type" NVP. The distal type NVP resembled FVP but was proven by unexpected elimination of the NVP during the slow pathway ablation. Also, NVP was distinguishable from FVP by a careful comparison of the HV intervals during conduction over the fast and slow pathways. Demonstration of this novel type NVP provides insights into how the insertion site of NVP affects its electrophysiologic behaviors.

Wide complex tachycardia in a patient with non-ischemic cardiomyopathy: What is the diagnosis?

A 55-year-old male presented with acute heart failure and incessant wide complex tachycardia resembling an outflow tract ventricular tachycardia. Meticulous analysis of the electrocardiograms established the diagnosis of pre-excitation with prolonged atrio-ventricular (A-V) conduction over a decrementally conducting accessory A-V pathway. "Linking" between the accessory A-V pathway and normal A-V conduction system resulted in sustained maximal pre-excitation as well as periodic transition to normal A-V conduction without appreciable change in heart rate. Successful radiofrequency ablation of this unusual accessory A-V pathway was performed at the aortic-mitral junction. This ameliorated the mechanical dysynchrony, allowed discontinuation of hemodynamic/inotropic support, and resulted in sustained symptomatic improvement.

Electrocardiographic and electrophysiological characteristics of fasciculoventricular fibers in children.

OBJECTIVES: Fasciculoventricular fiber (FVF) that does not cause tachyarrhythmia is a rare form of ventricular preexcitation, which is important to distinguish from Kent fibers. Although, adenosine and some electrocardiographic features are important in the differentiation of Wolff Parkinson White (WPW) than FVF, a clear distinction may not always be possible without an electrophysiological study (EPS). In this study, we aimed to present the clinical and electrophysiological features of our pediatric patients with fasciculoventricular fiber. PATIENTS AND METHOD: Between October 2013 and September 2021, 565 patients who underwent electrophysiological studies due to ventricular preexcitation in our clinic were screened in the study, and 27 (4.7%) patients with fasciculoventricular fiber were included. The data of the patients were obtained from the file records using the electronic internet database system Filemaker® . Electrophysiological study age, weight, gender, symptom, and presence of congenital heart disease of the patients were obtained from the file records. Accessory pathway localization was evaluated according to the modified Arruda algorithm in pre-procedural electrocardiography. In addition, delta wave amplitudes were measured in the first 40 ms from the surface ECG. PR interval, QRS interval, and delta wave amplitude were recorded before and after ablation in patients with additional accessory pathways. Post-procedure values were included in the FVF group. RESULTS: The mean age of the patients was 11.47 ± 4.25 years. All 70.4% of the reasons for admission were symptoms such as palpitations and syncope. Two patients had hypertrophic cardiomyopathy and 1 patient had ccTGA. In the electrophysiological study, additional manifest WPW was found in 9 (33%) patients (3 patients with high risk, 6 patients with orthodromic supraventricular tachycardia), focal atrial tachycardia in a patient, and atrioventricular nodal reentry tachycardia in a patient. While the delta wave amplitude was found to be 2.56 ± 1.38(1-5.5) mm in the first 40 ms in surface electrocardiography in 9 patients with additional accessory pathway, it was found to be 1.64 ± 0.67(0.5-3) mm in the FVF group. There was no statistically significant difference between the 2 groups (p = .398). Delta wave amplitude > 3.5 mm was not detected in any patient with isolated FVF. Interestingly, delta wave amplitude was < 3.5 mm in 7 (78%) of 9 patients who were identified and ablated with an additional accessory pathway. Total 19 of the patients (59.3%) were adenosine-responsive (18 isolated FVF, 1 manifest AP+FVF adenosine-responsive. 8 patients with other manifest AP + FVF had no pre-procedural adenosine-asystole response, and all of them QRS were expanded). CONCLUSION: Although, the fasciculoventricular fibers themselves are not the cause of tachyarrhythmia, the accessory pathway and other tachyarrhythmia substrate frequency accompanying these cases are quite high (approximately 40%) in EPS. The delta wave characteristics of ablated patients are very similar to FVF patients. While all patients with isolated FVF were adenosine responsive, most of those with additional manifest WPW were unresponsive. Therefore, performing EPS in patients with suspected FVF based on surface ECG features seems to be important for the detection of additional tachyarrhythmias and risky accessory pathways.