A Case of Reversible Cardiomyopathy Due to Pre-Excitation.

BACKGROUND Pre-excitation cardiomyopathy is a specific type of cardiac disease related to asymptomatic pre-excitation. It is rarely reported and is prone to misdiagnosis; therefore, the actual incidence of pre-excitation cardiomyopathy may be underestimated. The purpose of this case report is to present a case of pre-excitation cardiomyopathy caused by an accessory pathway. CASE REPORT A 25-year-old woman was admitted to the hospital with concerns of recurrent chest tightness and decreased exercise tolerance for 3 months. Pre-excitation was found by electrocardiogram. Contraction of the left ventricular wall reduced diffusely, and the overall left ventricle moved asynchronously. The regional septum basal segment swung to the right ventricle like an aneurysm in systolic period. No significant myocardial fibrosis was found. Pathological examination of endomyocardial biopsy demonstrated nonspecific changes of mild interstitial edema. Pre-excitation cardiomyopathy was eventually diagnosed. A right anteroseptal para-hisian manifest accessory pathway was located in an electrophysiological study, and radiofrequency catheter ablation was subsequently performed to block the advanced conduction. During the follow-up at 6 months after ablation, left ventricular dyssynchrony and systolic dysfunction were improved and symptoms were significantly relieved. CONCLUSIONS Pre-excitation cardiomyopathy is characterized by asynchronous left ventricular motion, impaired cardiac function, and manifestations of heart failure. Asynchronous electromechanical contraction coupling plays an essential role in the pathogenesis. Blocking the accessory pathway could help to correct the dyssynchrony, reverse remodeling, improve left ventricular function, and alleviate symptoms. Patients can have a good prognosis through accurate diagnosis and appropriate treatment.

The appearance of new accessory pathway after the Cone procedure.

INTRODUCTION: Acquired Wolff-Parkinson-White (WPW) syndrome can occur after congenital heart disease (CHD) surgery. METHODS AND RESULTS: A 27-year-old male with Ebstein's anomaly and manifest WPW syndrome received catheter ablation twice. The first electrophysiology study (EPS) induced orthodromic atrioventricular reentrant tachycardia and successfully eliminated the posteroseptal accessory pathway (AP). Six months after the Cone procedure, the patient suffered from palpitation. The second EPS was performed and abolished the right lateral AP. CONCLUSION: The appearance of a new AP after the reconstruction of CHD is a rare finding and should raise suspicion of an acquired AP connection.

Combining conventional technique with fluoroscopy integration module in accessory pathway ablation.

INTRODUCTION: Accessory pathway (AP) ablation is a straightforward approach with high success rates, but the fluoroscopy time (FT) is significantly longer in conventional technique. Electroanatomical mapping systems (EMS), reduce the FT, but anatomical and activation mapping may prolong the procedure time (PT). The fluoroscopy integration module (FIM) uses prerecorded fluoroscopy images and allows ablation similar to conventional technique without creating an anatomical map. In this study, we investigated the effects of combining the FIM with traditional technique on PT, success, and radiation exposure. METHODS: A total of 131 patients who had undergone AP ablation were included in our study. In 37 patients, right and left anterior oblique (RAO-LAO) images were acquired after catheter placement and integrated with the FIM. The ablation procedure was then similar to the conventional technique, but without the use of fluoroscopy. For the purpose of acceleration, anatomical and activation maps have not been created. Contact-force catheters were not used. 94 patients underwent conventional ablation using fluoroscopy only. RESULTS: FIM into AP ablation procedures led to a significant reduction in radiation exposure, lowering FT from 7.4 to 2.8 min (p < .001) and dose-area product from 12.47 to 5.8 µGym² (p < .001). While the FIM group experienced a reasonable longer PT (69 vs. 50 min p < .001). FIM reduces FT regardless of operator experience and location of APs CONCLUSION: Combining FIM integration with conventional AP ablation offers reduced radiation exposure without compromising success rates and complication.

Coronary sinus ablations in pediatric patients with supraventricular arrhythmias.

BACKGROUND: Intracoronary sinus ablations have been performed for various arrhythmical substrates. The aim of this study is to report our experience on pediatric patients of the safety and efficacy of ablations in the coronary sinus. METHODS: This is a retrospective study of all patients who underwent ablations in the coronary sinus from October 2013 to October 2021 at a single center. Clinical presentation, type of arrhythmia causing tachycardia, ablation procedure, and outcome were recorded. RESULTS: A total of 27 patients were included in the study. Nineteen (69%) of those followed up received a diagnosis of Wolff-Parkinson-White syndrome (WPW), 4 (15%) were cases of supraventricular tachycardia with concealed accessory pathway (AP), 2 (8%) were cases of focal atrial tachycardia, and 2 (8%) were cases of permanent junctional reciprocating tachycardia. Negative delta wave was noteworthy especially in lead II in 11/19 (58%) cases and coronary sinus diverticulum was detected in the WPW cases. Of those with manifest AP (19 cases), 15 (79%) had a high-risk AP and the AP in all WPW cases was adenosine unresponsive. Radiofrequency (RF) catheter ablation was performed in 25/27 (93%) cases during the procedure, and 16/25 (64%) of these were irrigated RF catheters. No complications were observed in the follow-up, including coronary artery injury. CONCLUSIONS: Catheter ablation of supraventricular tachyarrhythmias can be accomplished effectively and potentially safely within the coronary sinus. Coronary sinus diverticula should be suspected in patients with manifest posteroseptal APs who have a previous failed ablation and typical electrocardiographic signs.

Utilization of 3D mapping systems in interventional electrophysiology and its impact on procedure time and fluoroscopy-Insights from the "Go for Zero Fluoroscopy" project.

AIM: The implementation of 3D mapping systems plays an important role in interventional electrophysiology (EP) in recent years. The aim of the present study was to evaluate use of 3D mapping systems regarding fluoroscopy and procedure duration. METHOD: In the "Go for Zero Fluoroscopy" project 25 European centers provided data of consecutive EP procedures. Data on use of 3D mapping systems as well as utilization of contact force catheters and multipolar mapping catheters were associated with fluoroscopy time, dose area product (DAP), and procedure duration. RESULT: A 3D mapping system was used in 966 (54%) cases. Use of 3D mapping for atrioventricular nodal reentry tachycardia (AVNRT) was associated with reduced fluoroscopy time (p < 0.001), DAP (p = 0.04) but increased procedure time (p = 0.029). Moreover, fluoroscopy time (p < 0.001) and DAP (p = 0.005) were significantly lower in the 3D mapping group in ablation of typical atrial flutter. However, the procedure time (p < 0.001) increased. Use of 3D mapping in the ablation of accessory pathway (AP) was associated with reduced fluoroscopy time (p < 0.001) and DAP (p < 0.001) with no significant increase in procedure time (p = 0.066). In the case of atrial fibrillation, a 3D mapping system was used in 485 patients (75.8%). Additional use of a contact force catheter was associated with lower fluoroscopy time (p < 0.001) and DAP (p < 0.001). Use of a multipolar mapping catheter was associated with lower fluoroscopy time (p = 0.002). The implementation of 3D mapping systems in the ablation of ventricular tachycardias resulted in a significant increase in the procedure time (p = 0.001) without significant differences regarding the DAP (p = 0.773) and fluoroscopy time (p = 0.249). CONCLUSION: Use of 3D mapping systems in ablation of supraventricular tachycardias is associated with lower radiation exposure. Nevertheless, the procedure time often increases, except in the case of ablation for AP. Use of contact force catheters and multipolar mapping catheters is associated with yet lower radiation exposure values. Prospective randomized studies are needed to further elucidate potential benefit of these technological tools.

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.

Early dose of Adenosine, postRadiofrequency abLation of accessory pathwaY in determining acute procedural success (EARLY study).

INTRODUCTION: Post ablation of the accessory pathway (AP), the patient is observed in the catheterization laboratory for a variable period for resumption of pathway conduction. Aim of the study was to determine whether the administration of intravenous adenosine at 10 min after ablation of AP would have the same diagnostic accuracy as waiting for 30 min in predicting the resumption of AP conduction. METHODS: This was a prospective interventional study conducted in two centers. Post ablation of the AP, intravenous adenosine was administered at 10 min to look for dormant pathway conduction. The response was recorded as positive (presence of pathway conduction), negative (absence), or indeterminate (not able to demonstrate AV and VA block and inability to ascertain AP conduction). RESULTS: The study included 110 procedures performed in 109 patients. Adenosine administration at 10 min showed positive result in 3 cases (2.7%), negative result in 99 cases (90%) and indeterminate result in 8 cases (7.3%). Reconnection of accessory pathway at 30 min postablation was seen in 8 cases (7.3%). Of these 8 cases, 10 min adenosine administration showed positive test in 3 patients and negative test in 5 patients. Adenosine test at 10 min has a sensitivity, specificity, positive predictive value, and negative predictive value of 37.5%, 100%, 100%, and 94.9% in identifying the recurrence of accessory pathway conduction at 30 min, respectively. CONCLUSION: Absence of pathway conduction on administration of adenosine 10 min postablation does not help predict the absence of resumption of conduction thereafter.

Cryoablation of para-Hisian and mid-septal accessory pathways: long-term outcomes of a specific stepwise cryoablation protocol.

BACKGROUND: Although cryoablation (CA) of septally located accessory pathways (APs) is an established treatment for Wolff-Parkinson-White Syndrome, its major limitation is the lack of data regarding long-term follow-up (FU). The present study sought to investigate long-term outcomes of a specific CA protocol targeting para-Hisian (P-H) and mid-septal (M-S) APs. METHODS: Twenty-six patients who previously underwent CA of PH or MS APs from 2004 to 2014, were prospectively considered to receive a FU during 2021. All subjects received an outpatient control visit, performing an exercise stress test and a 24-h ECG Holter monitoring. RESULTS: Acute success was achieved in 22 patients (85%). One case of recurrence was reported at short-term FU. Long-term FU, performed after a mean time of 150±37 months, did not show ventricular preexcitation recurrences, with a success rate of 81%, and without late adverse events. Symptoms reduction (12% vs. 96%, P<.001) and lower rates of antiarrhythmic drug use (12% vs. 62%, P<.001) were observed at long term-FU with respect to baseline. This clinical outcome was detected also among patients who underwent unsuccessful CA at baseline. CONCLUSIONS: Our CA protocol confirmed remarkable safety and efficacy throughout a long-term FU. Significant clinical improvement in terms of antiarrhythmic therapy discontinuation and symptoms reduction was also shown among patients who experienced acute failure of CA.

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.

Results of catheter ablation with zero or near zero fluoroscopy in pediatric patients with supraventricular tachyarrhythmias.

INTRODUCTION AND OBJECTIVES: Ionizing radiation exposure in catheter ablation procedures carries health risks, especially in pediatric patients. Our aim was to compare the safety and efficacy of catheter ablation guided by a nonfluoroscopic intracardiac navigation system (NFINS) with those of an exclusively fluoroscopy-guided approach in pediatric patients. METHODS: We analyzed catheter ablation results in pediatric patients with high-risk accessory pathways or supraventricular tachycardia referred to our center during a 6-year period. We compared fluoroscopy-guided procedures (group A) with NFINS guided procedures (group B). RESULTS: We analyzed 120 catheter ablation procedures in 110 pediatric patients (11±3.2 years, 70% male); there were 62 procedures in group A and 58 in group B. We found no significant differences between the 2 groups in procedure success (95% group A vs 93.5% group B; P=.53), complications (1.7% vs 1.6%; P=.23), or recurrences (7.3% vs 6.9%; P = .61). However, fluoroscopy time (median 1.1minutes vs 12minutes; P <.0005) and ablation time (median 96.5seconds vs 133.5seconds; P=.03) were lower in group B. The presence of structural heart disease was independently associated with recurrence (P=.03). CONCLUSIONS: The use of NFINS to guide catheter ablation procedures in pediatric patients reduces radiation exposure time. Its widespread use in pediatric ablations could decrease the risk of ionizing radiation.

Atrioventricular accessory pathway unmasked by heart valve replacement.

A 50-year-old male patient with a history of severe valvular regurgitation underwent mitral and aortic valve replacement surgery 3 months ago. Preoperative 12-lead electrocardiogram presented atrial flutter (AFL) and atrial fibrillation. AFL complicated with ventricular pre-excitation was observed on current admission. The potential mechanisms underlying these changes were considered multifaceted, and valve replacement procedure may be a rare incentive factor.

V1r + DIIq is a novel and accurate criterion to predict right vs. left paraseptal accessory pathways.

PURPOSE: The correct estimation of accessory pathway (AP) localization from surface ECG is critical before the procedure. Our study aimed to detect the predictive value of the V1r + DIIq criterion for differentiating right- from left-sided paraseptal APs. METHODS: We retrospectively included 58 patients with (Wolff-Parkinson-White) WPW syndrome and paraseptal APs who underwent successful catheter ablation (37 male, 21 female; mean age 34.4 ± 13.6 years). The V1r + DIIq criterion was calculated using the following formula: V1r + DIIq (mV) = initial r wave amplitude in V1 + q wave amplitude in DII. The combined criterion included V1r + DIIq <2.05 mV and/or no initial r wave in V1. RESULTS: Right-sided paraseptal APs were detected in 36 patients (62.1%), left-sided paraseptal APs were detected in 21 patients (36.2%), and AP from CS was detected in 1 patient (1.7%). The initial r wave amplitude in V1 (mV), q wave amplitude in DII (mV) and V1r + DIIq criterion (mV) were lower in patients with right-sided paraseptal APs (p < 0.001). The percentage of patients with no initial r wave in V1 (36.1% vs. 0%) and those meeting the combined criterion (91.7% vs. 4.5%) were increased in patients with right-sided paraseptal APs. The cutoff value of the V1r + DIIq criterion obtained by ROC curve analysis was 2.05 mV for predicting right-sided paraseptal APs (sensitivity: 86.1%, specificity: 95.5%). The area under the curve (AUC) was 0.943 (95% CI = 0.881-1.000) (p < 0.001). The sensitivity and specificity values were 36.1% and 100%, respectively, for the no initial r wave criterion and 91.7% and 95.5%, respectively, for the combined criterion. CONCLUSION: The V1r + DIIq criterion and the combined criterion represent novel and simple electrocardiographic criteria for accurately differentiating right- from left-sided paraseptal APs. This simple ECG measurement can improve the accuracy of detection of paraseptal AP localization and could be beneficial for decreasing ablation duration and radiation exposure.

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.

Orthodromic Atrioventricular Tachycardia in Wolff-Parkinson-White Syndrome with Two Accessory Pathways Participation during the Same AVT

Case report of a 49-year-old patient with Wolff-Parkinson-White syndrome, very symptomatic, with apparent parahisian pathway who, during an electrophysiological study, presented orthodromic atrioventricular tachycardia, featuring two accessory pathways, retrogradely, the parahisian pathway and a hidden left posterolateral pathway, during the same tachycardia, alternating the retrograde pathway of tachycardia without interruption.

Contemporary procedure characteristics and outcomes of accessory atrioventricular pathway ablations in an integrated community-based health care system using a tiered approach.

BACKGROUND: Since the early descriptions of large series of accessory atrioventricular pathway ablations in adults and adolescents over 20 years ago, there have been limited published reports based on more recent experiences of large referral centers. We aimed to characterize accessory pathway distribution and features in a large community-based population that influence ablation outcomes using a tiered approach to ablation. METHODS: Retrospective analysis of 289 patients (age 14-81) who underwent accessory ablation from 2015-2019 was performed. Pathways were categorized into anteroseptal, left freewall, posteroseptal, and right freewall locations. We analyzed patient and pathway features to identify factors associated with prolonged procedure time parameters. RESULTS: Initial ablation success rate was 94.7% with long-term success rate of 93.4% and median follow-up of 931 days. Accessory pathways were in left freewall (61.6%), posteroseptal (24.6%), right freewall (9.6%), and anteroseptal (4.3%) locations. Procedure outcome was dependent on pathway location. Acute success was highest for left freewall pathways (97.1%) with lowest case times (144 ± 68 min) and fluoroscopy times (15 ± 19 min). Longest procedure time parameters were seen with anteroseptal, left anterolateral, epicardial-coronary sinus, and right anterolateral pathway ablations. CONCLUSIONS: In this community-based adult and adolescent population, majority of the accessory pathways are in the left freewall and posteroseptal region and tend to be more easily ablated. A tiered approach with initial use of standard ablation equipment before the deployment of more advance tools, such as irrigated tips and 3D mapping, is cost effective without sacrificing overall efficacy.

Outcomes From Pediatric Ablation: A Review of 20 Years of National Data.

OBJECTIVES: This study set out to examine outcomes from pediatric supraventricular tachycardia ablations over a 20-year period. This study sought to examine success rates and repeat ablations over time and to evaluate whether modalities such as 3-dimensional (3D) mapping, contact force, and cryotherapy have improved outcomes. BACKGROUND: Ablation of supraventricular tachycardia in pediatric patients is commonly performed in most congenital heart centers with excellent long-term results. METHODS: Data were retrieved from the NICOR (National Institute of Clinical Outcomes Research) database in the United Kingdom. Outcomes over time were evaluated, and procedure-related details were compared. RESULTS: There were 7,069 ablations performed from January 1, 1999, to December 31, 2018, at 10 centers. Overall, ablation success rates were 92% for accessory pathways, 97% for atrioventricular node re-entry tachycardia, and 89% for atrial tachycardia. There was an improvement in procedural success rates over time (p < 0.01). The use of 3D mapping did not alter success or need for repeat ablation but was associated with a higher proportion of lower fluoroscopy cases; 55% of 3D mapping cases used <5 min of fluoroscopy (p < 0.01). Patients needing a repeat ablation were 341 (12%) for accessory pathways, 128 (7%) for atrioventricular node re-entry tachycardia, and 35 (7%) for atrial tachycardia. Overall, the risk of complete heart block was low (n = 12, <0.01%). The use of cryotherapy was associated with an increased risk of needing a repeat ablation. CONCLUSIONS: Overall success rates from pediatric ablations are excellent and compare favorably to other registries. Introduction of newer technologies have likely made procedures safer and reduced radiation exposure, but they have not changed success rates or the need for a repeat procedure.

Ultrahigh density atrio-ventricular dual-chamber mapping as a next generation tool for ablation of accessory pathways.

INTRODUCTION: Detailed three-dimensional (3D) mapping has been useful for effective radiofrequency catheter ablation. The Rhythmia system can create atrio-ventricular dual-chamber mapping, which reveals the atrial and ventricular potentials all at once in the same map. The aim of this study was to investigate the utility of mapping the atrium and ventricle simultaneously with a high-density 3D mapping system for the ablation of accessory pathways (AP). METHODS: From July 2015 to August 2020, 111 patients underwent ablation of APs. Dual-chamber maps were created in 50 patients (median age 15 [10-54], 32 male [64.0%]), while 61 patients underwent radiofrequency (RF) ablation with conventional single-chamber 3D maps. The background characteristics and procedural details were compared between the dual-chamber mapping group and the conventional single-chamber mapping group. RESULTS: The number of RF applications (median [IQR]; 1.0 [1.0-3.0] vs. 3.0 [1.0-6.0], p = .0023), RF time (median [IQR], s; 9.2 [2.0-95.7] vs. 95.6 [4.1-248.7], p = .0107), and RF energy (median [IQR], J; 248.4 [58.7-3328.2] vs. 2867.6 [134.2-7728.4], p = .0115) were significantly lower in the dual-chamber group. The fluoroscopy time (median [IQR], min; 19.9 [14.2-26.1] vs. 26.5 [17.7-43.4], p = .0025) and fluoroscopy dose (median [IQR], mGy; 52.5 [31.3-146.0] vs. 119.0 [43.7-213.5], p = .0249) were also significantly lower in the dual-chamber than single-chamber mapping group. CONCLUSION: The dual-chamber mapping was useful for ablating accessory pathways and reducing the number of RF applications, total RF energy, and radiation exposure as compared with traditional mapping techniques.

Conduction Properties and Ablation of Adenosine Sensitive Accessory Pathways in Children.

Block in accessory pathway (AP) conduction with adenosine has been previously described. However, conduction characteristics of these APs has not been well defined to date. All patients with APs

Anomalía de Ebstein y aneurisma del seno coronario / Ebstein’s anomaly and coronary sinus aneurysm

Resumen La anomalía de Ebstein es una cardiopatía congénita poco común que se asocia a la presencia de vías de conducción anómalas y episodios de taquicardia supraventricular frecuentes, algunos inestables. La asociación con alteraciones anatómicas del seno coronario es rara y no ha sido reportada. Se presenta el caso de una paciente de 58 años con enfermedad coronaria, anomalía de Ebstein, episodios de taquicardia ortodrómica y aneurisma del seno coronario, a quien se realizó ablación.

Abstract Ebstein’s disease is a congenital cardiomyopathy, with a low prevalence in the general population. This abnormality has been associated with abnormal cardiac conduction problems, one of the most important being the accessory pathways. In the presence of an accessory pathway, frequent supraventricular tachycardias may occur, some of which are poorly tolerated. The association with the anomalies of the coronary sinus is not currently reported. The case of a 58-year-old woman with Ebstein’s disease, episodes of supraventricular tachycardia, and coronary sinus aneurysm undergoing ablation therapy is presented.

Fasciculoventricular and atrioventricular accessory pathways in patients with Danon disease and preexcitation: A multicenter experience.

BACKGROUND: Studies have suggested that a fasciculoventricular pathway (FVP) may be the cause of preexcitation in patients with Danon disease, a rare X-linked dominant genetic disorder of hypertrophic cardiomyopathy. OBJECTIVE: The purpose of this study was to describe the prevalence of ventricular preexcitation on resting 12-lead electrocardiogram (ECG) in patients with Danon disease and the electrophysiological study (EPS) results of those with preexcitation. METHODS: Patients with confirmed Danon disease diagnosed with preexcitation (PR ≤120 ms, delta wave, QRS >110 ms) on ECG were included from a multicenter registry. The incidence of arrhythmias, implantable cardioverter-defibrillator (ICD) procedures, ICD shocks, and EPS results were collected. RESULTS: Thirteen of 40 patients (32.5%) with Danon disease were found to have preexcitation (mean age 17.3 years; 38% women). EPS performed in 9 of 13 patients (69%) demonstrated FVP only in 2 (22.2%), extranodal pathway without exclusion of FVP in 2 (22.2%), and both FVP and extranodal pathway in 5 (55.6%). Two patients had malignant accessory pathway (AP) properties. Over median follow-up of 842 days (interquartile range 138-1678), 11 patients (85%) had ICD placement, and 6 (46.1%) underwent heart transplantation. No patients required therapy for ventricular tachycardia, and 2 patients (15%) had paroxysmal atrial fibrillation. CONCLUSION: In a large multicenter cohort of patients with Danon disease, there was a high prevalence of FVP and extranodal pathways diagnosed on EPS in those with preexcitation. These findings suggest patients with preexcitation and Danon disease should undergo EPS to assess for FVP and potentially malignant extranodal AP.