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We report a case of a patient with right axillary ventricular. Similar congenital anomaly of the right atrium was reported as "right appendage diverticulum or right atrial diverticulum." However, this independent chamber has its own annulus, synchronizes with the right ventricular, and generates large ventricular potential. Under the guidance of the CARTO mapping system (Biosense Webster, Diamond Bar, CA, USA), a right atrioventricular accessory pathway associated with type B Wolff-Parkinson-White syndrome was ablated successfully. This pathway was close to the annulus of the axillary ventricular. The patient remained free of arrhythmia at 1-year follow-up.

Background Successful ablation sites in Wolff-Parkinson-White syndrome (WPW) are characterized by short atrioventricular (AV) intervals. Approximately 15% of patients with WPW have adenosine-sensitive accessory pathways (APs). We sought to determine if local AV intervals of adenosine-sensitive APs are different from those of adenosine-insensitive APs in patients with WPW. Methods Patients ≤21 years with WPW and adenosine-sensitive APs who underwent successful ablation over a 9-year period were included. Patients with WPW and adenosine-insensitive APs were matched by age and weight in a 1:2 case-control design. AP location, antegrade and retrograde conduction properties, supraventricular tachycardia (SVT) inducibility, local AV interval, interval from delta wave onset to local ventricular activation (del-V), and time to loss of preexcitation were reviewed. Results Fourteen patients with adenosine-sensitive APs and 28 with adenosine-insensitive APs were included. Patients with adenosine-sensitive APs had minimum 1:1 antegrade AP conduction at a longer median paced cycle length (380, interquartile range [IQR] 295 to 585 ms vs 290, IQR 250 to 330 ms, P = 0.046), were less likely to have inducible SVT (35.7% vs 75.0%, P = 0.035), and had a longer median local AV interval (40.5, IQR 30.8 to 58.3 ms vs 32.0, IQR 29.3 to 37.8 ms, P = 0.029) when compared to those with adenosine-insensitive APs. Conclusion Patients with WPW and adenosine-sensitive APs have 1:1 antegrade AP conduction at longer cycle lengths, lower likelihood of SVT induction, and longer local AV intervals when compared to those with adenosine-insensitive APs. In patients with WPW, it may be important to consider adenosine response when selecting appropriate ablation targets.

Anisotropy in conduction velocity (CV) is a key substrate abnormality influencing atrial arrhythmias. In skeletal muscle fibers, CV and frequency content of the surface electromyogram signal are directly related. We hypothesized that in human atria the frequency content of the bipolar signal, recorded on the endocardial surface, is directly related to the local CV.In 15 patients submitted to ablation of supraventricular arrhythmias, incremental pacing was performed through an octapolar catheter inserted into the coronary sinus (CS), alternatively from both extremities in two different sequences: CS bipole 1-2 as the pacing site and CS bipole 7-8 as the detection site in the first, and vice versa in the second. The pacing cycle length (PCL) was stepwise decreased from 600 ms to 500 ms, 400 ms, 300 ms, until 250 ms. Estimation of the CV was performed as the ratio between the distance traveled by the propagating pulse and the propagation time. The frequency distribution of the signal energy was estimated using the fast Fourier transform, and the characteristic frequency (CF) was estimated as the barycenter of the frequency spectrum.A total of 2,496 bipolar signals were analyzed; CV and CF were estimated and compared. The single patient and group data analysis showed a significant direct correlation between CV and CF of the local bipolar signal.Comparing the degree of spectral compression among signals registered in different points of the endocardial cardiac surface in response to decreasing PCL enables to map local differences in CV, a useful arrhythmogenic substrate index.

Background Risk stratification for Wolff-Parkinson-White (WPW) pattern either by noninvasive or invasive tests is important to determine whether an ablation is necessary or not. The aim was to compare noninvasive tests and invasive studies in a pediatric WPW population. Methods A total of 71 WPW patients (median age 14 years [interquartile range, 11-16 years]; 43 male) underwent Holter monitoring, exercise stress test (EST), and transesophageal electrophysiological study (TEEPS). In the case of a ≤270-ms effective refractory period of accessory pathway or induction of supraventricular tachycardia using TEEPS, patients were classified as high risk and underwent invasive electrophysiological study (EPS). Results Nine of 23 patients with low risk by Holter and eight of 26 patients with low risk by EST had high risk in TEEPS. Risky TEEPS results were detected in 42 of 71 patients and those patients underwent EPS. High-risk characteristic in EPS was detected in seven of nine patients with low risk by Holter and in seven of eight with low risk by EST. Both tests yielded moderate specificity (52-69%) and negative predictive value (61-69%) according to TEEPS. Conclusion Holter and EST have low predictive value in WPW risk stratification. However, TEEPS and EPS are extremely valuable in WPW risk stratification. TEEPS may reveal important and useful results for WPW risk determination, especially in small children not having undergone EPS in order to avoid its complications.

To compare the efficacy, safety, and cost-effectiveness of a three-catheter approach with a conventional five-catheter approach for the mapping and ablation of supraventricular tachycardia in pediatric patients with Wolff-Parkinson-White Syndrome (WPW) and concealed accessory pathways (APs).A retrospective review from 2008 to 2012 of patients less than 21 years with WPW who underwent a three-catheter radiofrequency (RF) ablation of a left-sided AP (ablation, right ventricular [RV] apical, and coronary sinus [CS] decapolar catheters) was performed. The three-catheter group was compared to a control group who underwent a standard five-catheter (ablation, RV apical, CS decapolar, His catheter, and right atrial catheter) ablation for the treatment of left-sided WPW or concealed AP. Demographics, ablation outcomes, and costs were compared between groups.Twenty-eight patients met inclusion criteria with 28 control patients. The groups did not differ in gender, age, weight, or body surface area. Locations of the AP on the mitral annulus were similar between the groups. All patients were ablated via transseptal approach. Note that 28 of 28 in the three-catheter group (100%) and 27 of 28 (96%) controls were acutely successfully ablated (P = 0.31). No complications were encountered. There was no difference in procedural time, time to loss of AP conduction, or number of RF applications. Use of the three-catheter technique resulted in a total savings of $2,465/case, which includes the $680 savings from using fewer catheters as well as the savings from a shortened procedure time.Ablation in patients with WPW and a left-sided AP can be performed using three catheters with similar efficacy and safety while offering significant cost savings compared to a conventional five-catheter approach.

Intermittent Wolff-Parkinson-White (WPW) syndrome is considered to have a lower risk of sudden death. Fewer data exist regarding electrophysiologic (EP) characteristics and the natural history of intermittent WPW in children.All patients with WPW age 1-18 years at a single institution (1996-2013) were reviewed. Patients with intermittent preexcitation were compared to those with loss of preexcitation on Holter/exercise testing and those with persistent preexcitation. High-risk accessory pathway (AP) was defined as AP effective refractory period (APERP), block cycle length, or shortest preexcited RR interval during atrial fibrillation ≤250 ms.A total of 295 patients were included: 226 (76.6%) persistent, 39 (13.2%) intermittent, and 30 (10.2%) loss of preexcitation Holter/exercise. There were no differences in symptoms between groups. Median interquartile range APERP was significantly longer in intermittent WPW (380 [320, 488] ms vs 320 [300, 350] ms persistent, 310 [290, 330] ms loss of preexcitation Holter/exercise; P = 0.0008). At baseline, there was no difference between groups in frequency of high-risk pathways. However, when isoproterenol values were included, high-risk pathways were more frequent among patients with loss of preexcitation on Holter/exercise (54% vs 16% persistent, 11% intermittent; P = 0.005). There was one death in a patient with loss of preexcitation on exercise testing, no EP study, and prior drug use. A second patient with persistent WPW and APERP 270 ms required resuscitation following a methadone overdose.Intermittent preexcitation in children does not connote a lower risk AP by EP criteria or reduced symptoms. The low number of pediatric WPW patients who develop preexcited atrial fibrillation or sudden death warrants larger studies to investigate these outcomes.

Accessory pathways (APs) with intermittent preexcitation (IPX) are thought to be of lower risk, but there are reports of IPX patients presenting with rapidly conducted atrial fibrillation.Retrospective study performed on patients with preexcitation who underwent an electro-physiological study (EPS). IPX was defined as loss of the delta wave on electrocardiogram prior to EPS. Patients with IPX were compared with those with persistent preexcitation (PPX) or suppression of the delta wave on exercise test (IPX-ET). Congenital heart disease and prior ablations were excluded.Of 328 patients with preexcitation, 41 (12.5%) had IPX. Patients with IPX or PPX were similar in age (12.9 years vs 13.0 years, P = 0.8) and AP location (left-sided 54% vs 50%, P = 0.7; septal 32% vs 35%, P = 0.4). Testing on isoproterenol was performed in 17 (41%) IPX and 41 (14%) PPX patients. Although IPX patients had a longer median refractory period compared to PPX patients (340 ms vs 310 ms, P = 0.001), the incidence of APs with refractory periods ≤250 ms was similar (10% vs 12%, P = 1.0). Exercise tests were performed on 208 patients and 24 (12%) had IPX-ET. Compared with IPX patients, IPX-ET had similar median AP refractory periods (320 ms, P = 0.4) and incidence of APs with refractory periods ≤250 ms (13%, P = 1.0).Patients with IPX had longer AP refractory periods than those with PPX, but the incidence of pathways with refractory periods ≤250 ms was not significantly different. The finding of IPX on a baseline electrocardiogram does not rule out potentially high-risk pathways.

BACKGround : Left lateral accessory pathway (AP) location along the mitral annulus (MA) can influence ablation strategy, including choice of a transseptal or retrograde aortic approach and the use of deflectable sheaths and/or bidirectional catheters. We aimed to develop electrocardiographic (ECG) criteria to accurately localize a left lateral AP, hypothesizing that the relationship of QRS amplitudes in limb leads II and III could be used to differentiate left anterolateral (LAL) from left posterolateral (LPL) AP locations.The ECGs from patients who underwent ablation of a left-sided AP between 2001 and 2008 were evaluated for the relationship of QRS amplitudes in limb leads II and III. A LAL-AP was defined by successful ablation between 12 and 3 o'clock on the MA, as seen in left anterior oblique (LAO) fluoroscopic projection. A LPL-AP was defined by successful ablation between 3 and 6 o'clock in the LAO projection.In 249 consecutive patients undergoing AP ablation, 23 met the prespecified inclusion criteria: manifest preexcitation due to single AP, ablated successfully in a LAL or LPL location. The ratio of dominant QRS amplitude in lead II to lead III was ≥ 1 in 10/11 patients with LAL-AP, compared with 3/12 patients with a LPL-AP (P = 0.002). Using these criteria, two blinded reviewers predicted a LAL or LPL location with 87% accuracy and 100% interobserver agreement.We report new ECG criteria that can be used to accurately predict the anterior and posterior location of a left lateral AP. Such localization may facilitate procedural planning.

In Wolff-Parkinson-White (WPW) syndrome, rapid antegrade conduction of atrial tachyarrhythmias can result in ventricular fibrillation and sudden death. Antegrade conduction can be assessed through noninvasive testing or invasive electrophysiology study (EPS). We aimed to determine the correlation between noninvasive testing and EPS in a pediatric WPW population.All WPW patients21 years who underwent EPS over a 10-year period were identified. Noninvasive testing reviewed included electrocardiogram, Holter, and exercise stress test (EST). Patients were classified as low-risk if preexcitation was lost during any test. EPS data reviewed included antegrade conduction during atrial pacing and atrial fibrillation. Conduction through the accessory pathway (AP) to a cycle length ≤ 250 ms was considered rapid, otherwise patients were nonrapid. Sensitivity, specificity, positive (PPV), and negative predictive value (NPV) of noninvasive testing to correctly identify nonrapid conduction was calculated.There were 135 EPS. Twenty-four patients (18%) were classified low-risk noninvasively. Two of the 24 (8%) had rapid conduction at baseline EPS. The sensitivity, specificity, PPV, and NPV of low-risk noninvasive testing to predict nonrapid conduction was 22%, 94%, 92%, and 31%, respectively. Sixteen of the 24 had low-risk EST and none had rapid conduction at baseline EPS. The specificity and PPV of low-risk EST were 100%.Loss of preexcitation during noninvasive testing had high specificity and PPV for nonrapid antegrade conduction during baseline EPS. Abrupt loss of preexcitation during EST was a highly reliable noninvasive marker of nonrapid AP conduction at baseline in our pediatric WPW patients.

Syncope in Wolff-Parkinson-White syndrome (WPW) is without relationship with WPW or reveals a poorly tolerated arrhythmia. Electrophysiologic study (EPS) is recommended. The purpose of the study was to evaluate the influence of the patient's age on the causes and prognosis of syncope.A total of 98 patients, mean age 35 ± 18 years, with WPW were admitted for syncope. Note that 29 were aged between 9 and 19 years (mean 15 ± 3) (children and teenagers/group I), 45 between 20 and 49 years (mean 34 ± 8) (adults/group II), and 24 between 50 and 70 years (mean 60 ± 8) (elderly/group III). EPS consisted of atrial pacing and programmed atrial stimulation in control state and after isoproterenol.Potentially malignant form (rapid conduction in accessory pathway240 beats/min in control state or300 beats/min after isoproterenol and atrial fibrillation [AF] induction) was more frequent in group I (34%) than in groups II (7%) (P0.002) and III (0%) (P0.001). Orthodromic atrioventricular reentrant tachycardia (AVRT) and AF were induced as frequently in groups I (59, 34%), II (47, 15.5%), and III (54, 17%). AVRT was induced in all but one patient with malignant form. EPS was as frequently negative in groups I (27.5%), II (44%), and III (37.5%). Natural follow-up (mean 8 ± 6 years) indicated a favorable prognosis, only related to AVRT induction. Induced AF was without significance.Data in syncope and WPW syndrome depended on age: electrophysiological malignant form was frequent in children/teenagers, rare in adults, and absent in elderly. AVRT, the main cause of syncope, was as frequent in all ranges of age. AF's induction alone had no significance. Final prognosis was favorable.

Irrigated-tip catheter technology has been used for the elimination of resistant accessory pathways (AP) in adults with Wolff-Parkinson-White (WPW) syndrome. However, there are persistent concerns regarding the safety of irrigated catheters in the pediatric population. In this report we present our experience, in terms of effectiveness and safety, of irrigated catheter technology in children and adolescents who underwent ablation of WPW.We prospectively followed up all patients less than 18 years old (n = 41, mean age of 12.8 years old) who were referred to our center for radiofrequency (RF) catheter ablation of WPW between January 2010 and July 2011. Catheter ablation was performed in all patients using an open irrigated-tip catheter (Celsius Thermocool 3.5 mm, 7F, B-type, Biosense Webster, Diamond Bar, CA, USA). Power was started from 15 W up to 30 W in right-sided AP; RF pulses in left-sided APs were delivered at 40 W while 20 W was delivered inside the coronary sinus.Mean procedure time was 26.4 minutes and mean fluoroscopy time was 12.2 minutes. Overall procedural success was obtained in 39/41 (95.1%) patients after the first procedure. No complications were observed after the procedure. All patients attended their scheduled follow-up visit at 3, 6, and 12 months and no recurrences were observed based on 12-lead electrocardiogram and 24-hour Holter monitoring.RF ablation of APs using open irrigated-tip catheters can be performed in children and adolescents with a high acute and long-term success rate, very short procedure times, and acceptable fluoroscopy times.

Adenosine administration to patients with Wolff-Parkinson-White (WPW) usually increases preexcitation and therefore may be diagnostic for WPW syndrome when the electrocardiogram (ECG) is questionable. We aimed to determine the adenosine response in pediatric patients with WPW pattern on ECG and whether blocked accessory pathway (AP) conduction with adenosine correlated with nonrapid AP conduction measured by invasive electrophysiology study (EPS).All patients with WPW ≤ 18 years of age who underwent EPS over a 5-year period were identified. The adenosine response during atrial pacing was characterized as blocked or continued AP conduction. Invasive data were obtained during atrial pacing and atrial fibrillation. Conduction through the AP to a cycle length ≤ 250 ms was considered rapid; otherwise patients were nonrapid. The sensitivity, specificity, and positive (PPV) and negative predictive value were calculated for blocked AP conduction to identify nonrapid baseline AP conduction during EPS.There were 59 patients included and nine (15%) had blocked AP conduction with adenosine. Five of these nine had WPW syndrome and four had fasciculoventricular APs. All nine patients had nonrapid conduction on baseline EPS. Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 100%, a PPV of 100%.In these pediatric patients with WPW pattern on ECG, a significant minority blocked AP conduction with adenosine and this finding had 100% specificity and PPV for nonrapid baseline antegrade AP conduction. The finding of blocked AP conduction with adenosine may aid in risk stratification.

We report two cases of intermittent Wolff-Parkinson-White (WPW) syndrome. In one patient, early repolarization (ER) was masked during preexcitation whereas in the other, J wave-like notches were observed in the right precordial leads only during preexcitation. The clinical significance of ER is not apparent in WPW syndrome but some possible mechanisms are discussed.

Risk stratification for Wolff-Parkinson-White (WPW) by intracardiac electrophysiology study (ICEPS) carries risks related to catheterization. We describe an alternative approach by using transesophageal electrophysiology study (TEEPS).The pediatric electrophysiology database was reviewed for patients with WPW and no documented clinical supraventricular tachycardia (SVT) who underwent risk stratification by TEEPS from October 2005 to November 2010. Of those who underwent subsequent ICEPS, only those with data available to compare accessory pathway (AP) conduction during ICEPS and TEEPS were included.Of 65 patients who underwent TEEPS, 42 were found to have an indication for ablation. The most common indication for ICEPS was inducible SVT, which was induced in 67% of patients. Of 42 patients who underwent subsequent ICEPS, 23 had sufficient data for comparison of AP conduction between ICEPS and TEEPS. There was no difference between the baseline minimum 1:1 antegrade conduction through the accessory pathway found at TEEPS versus ICEPS (312 ± 51 ms vs 316 ± 66 ms, P = 0.5). There was no significant difference between the baseline antegrade AP-effective refractory period found at TEEPS versus ICEPS (308 ± 34 ms vs 297 ± 37 ms, P = 0.07). There were no complications related to TEEPS or ICEPS.TEEPS is a safe and feasible alternative to ICEPS for risk stratification in patients with asymptomatic WPW and should be considered before ICEPS and ablation.

Antidromic reciprocating tachycardia (ART) is a rare form of wide complex tachycardia in children with Wolff-Parkinson-White syndrome (WPW). The incidence and electrophysiologic characteristics of ART in children with WPW have not been well described.A multicenter retrospective analysis of all patients with WPW undergoing electrophysiology (EP) study from 1990 to 2009 was performed. Patients with clinical or inducible ART were included.A total of 1,147 patients with WPW underwent EP study and 30 patients had ART (2.6%) and were the subject of this analysis. The mean age was 16±3 years, weight was 65±16 kg, and tachycardia cycle length was 305±55 ms. There were two patients (7%) with congenital heart disease (both with Ebstein's anomaly). Four patients (13%) had more than one accessory pathway (AP). The location of the AP was left sided in 53% of patients and right sided in 47%, with septal location and left lateral pathways most commonly involved. AP conduction was found to be high risk in 17 patients (57%). Ablation was not attempted in two patients (7%) due to proximity to the HIS and risk of heart block. Ablation was acutely successful in 93% of the patients in whom it was attempted.ART is a rare finding in children undergoing EP study. Over half of the patients with ART were found to be high risk and multiple AP were uncommon. Unlike the adult population, ART occurred commonly with septal APs.

The coexistence of Brugada syndrome and Wolff-Parkinson-White (WPW) syndrome is a very rare phenomenon. We describe a 31-year-old patient without any previous cardiac disorder admitted to our hospital due to palpitations and concomitantly diagnosed as WPW syndrome and treated with radiofrequency catheter ablation. He was later diagnosed with Brugada syndrome and followed-up 2 years without any symptoms. We discuss other previously reported cases in literature, in which these two conditions exist simultaneously.

Wolff-Parkinson-White (WPW) syndrome is a disorder characterized by presence of an accessory pathway that predisposes patients to tachyarrhythmias and sudden death. Radiofrequency ablation (RFA) is both effective and safe for patients with symptomatic tachyarrhythmias mediated by accessory atrioventricular pathways. During the procedure, fatal complications may occur but it is considered low in relation to the morbidity associated with the WPW syndrome. Coronary artery occlusion, as a complication of an RF catheter ablation, is quite rare. In this report, we present a 56-year-old male patient with a left main coronary artery (LMCA) occlusion during the ablation of left anterolateral wall accessory pathway. It should be kept in mind if the patient complains of new onset of severe chest pain, an immediate angiography should be performed.

The aim of the study was the evaluation of the predictors of adverse presentation as first arrhythmia in Wolff-Parkinson-White syndrome; they usually affect young patients with septal or multiple accessory pathways (AP).Our population comprised 645 patients with a preexcitation syndrome. Among them, adverse presentation (sudden death, hemodynamically not tolerated atrial fibrillation [AF]) occurred in 60 (9%) (group I). Their clinical and electrophysiological features were compared to group II patients, which consisted of 75 patients with syncope (IIa), 287 with reentrant tachycardia (RT) (IIb), 211 asymptomatic patients (IIc), and 12 with well-tolerated AF.Sixteen group I patients had triggering factors. Group I patients were older (40 ± 18.5) than group II (34 ± 16) (P = 0.02). Male gender was as frequent in both groups (63%, 59%). Free wall left AP was more frequent in group I (65%) than in group II (37%) (P0.001), septal AP less frequent (27% vs 47%) (P = 0.004), multiple APs exceptional. RT was more frequent in group I (57%) than in group IIc (12%) (P0.001), less frequent than in group IIb (90.5%) (P0.001). AF was more frequent in group I (85%) than in group IIc (22%), or IIb (19%) (P0.001). Maximal rate through AP was higher in group I than in group II (P0.001).Adverse presentation in WPW may affect patients older than 35 years of both sexes, with a single free wall lateral AP. All could have been identified by an electrophysiological study.

We present a case of a conscript who was referred for ablation of asymptomatic Wolff-Parkinson-White syndrome. However, the electrophysiologic study revealed the presence of an accessory pathway arising infranodally in the His-Purkinje system, and inserting into the nearby ventricle. The case emphasizes the limitations of the surface ECG and the importance of a thorough electrophysiologic study. Our patient required no specific therapy but correction of the diagnosis before being declared fit for military service. A second interesting finding not described before with this type of pathway was the occurrence of transient mechanical interruption of accessory pathway conduction.

A 33-year-old woman presented with exercise-related palpitations after an apparently successful catheter-ablation of overt midseptal accessory pathway. Post procedure, the electrocardiogram at rest was normal, while the progressive appearance of delta-wave during treadmill stress testing was recorded. In addition, the occurrence of ventricular preexcitation was reproduced by controlled administration of dobutamine. Detailed understanding of the unusual pathway electrophysiology resulted in specific planning of the second procedure. In the basal state, pacing maneuvers did not demonstrate any evidence of pathway conduction. However, during infusion of dobutamine bidirectional conduction in the right anterior pathway was restored, enabling definitive cure by radiofrequency.

Background: Health-Related Quality of Life (HRQOL) can be negatively affected in patients living with arrhythmias and many patients experience a pronounced symptom burden. The arrhythmia can cause both uncertainty and limitations, including interference with work, reluctance to perform and plan for leisure activities and leading to self-imposed restrictions in daily life situations. There are patients striving to find strategies to manage the arrhythmia and for some this can become the focus in their lives. Treatment options are often a choice between pharmaceuticals and radiofrequency ablation (RFA) where RFA is an option for many arrhythmia-patients to be cured. In the care of arrhythmia-patients it is of great importance to combine objective examinations with patient-reported outcomes (PROs) to achieve patient’s own experiences of treatment efficacy and arrhythmias interference in daily life situations. Aims: The overall aims of this thesis were to assess symptom burden and HRQOL in patients with arrhythmias and to develop and validate an arrhythmia-specific questionnaire, suitable for most arrhythmia-patients. Design and Methods: Studies I and II were single-centre studies including patients referred for RFA, with two different arrhythmia diagnoses. Assessments of patient-reported outcomes (PROs) concerning HRQOL were performed using two questionnaires, SF-36 and EQ-5D (I-II). Further, patients were asked some disease-specific questions (I). Study I describes assessments before the RFA treatment and Study II the follow-up assessments at three and twelve months after RFA. Patients’ scoring of HRQOL was compared to age and gender matched reference groups before and after RFA (I-II). Studies III and IV describe the development and validation of a disease-specific questionnaire ASTA (Arrhythmia-Specific questionnaire in Tachycardia and Arrhythmia) assessing symptom burden and HRQOL. Studies III and IV were multicentre studies. Patients planned for DC-conversion, AF patients seeking emergency care and those with different forms of arrhythmias referred for RFA were included. Results: Patients scored significantly lower HRQOL in seven of SF-36’s eight scales compared to the age and gender matched reference groups before RFA treatment. Frequent arrhythmia attacks had a great negative impact on HRQOL, and female gender and older age were factors contributing to worse HRQOL (I). Treatment with RFA restored the patients’ HRQOL. Most positive effects were seen at three months follow-up. One year after treatment patients and the matched reference group scored their HRQOL to a similar level, assessed with SF-36 and EQ-5D index (II). The validated ASTA questionnaire was found to have good psychometric properties. Construct validity was confirmed with sufficient levels of item-total correlations in the ASTA symptom burden scale and HRQOL scales. The dimensionality of the ASTA HRQOL scale was established with confirmatory factor analysis, supporting a physical and a mental subscale. The internal consistency, demonstrated with Cronbach’s alpha (α), was satisfactory for the ASTA symptom burden scale and the ASTA HRQOL scales, varying from α 0.79 to α 0.91 (III-IV). Conclusions and clinical implications: The studies in this thesis confirmed how negatively affected the arrhythmia-patients can be with a pronounced symptom burden and impaired HRQOL. Treatment with RFA was demonstrated to restore the patients HRQOL to an equal level of that of the matched reference group. PROs are important to take into consideration in the care of arrhythmia-patients, to achieve the patients’ subjective experiences of their daily life situation. To the best of our knowledge ASTA is the first arrhythmia-specific questionnaire assessing symptom burden and HRQOL, suitable for most arrhythmia forms. The newly validated ASTA questionnaire can be an important contribution to assessment of PROs in arrhythmia-patients.

Background: Atrioventricular reciprocating tachycardia (AVRT) is common in patients (pts) with WolffParkinson-White (WPW) syndrome but atrial flutter/fibrillation (AF) with rapid ventricular response (RVR) is rare. Although AF occurs in 18% of adult WPW pts, its incidence in children is unknown. We sought to determine risk factors for AVRT spontaneously degenerating to AF during electrophysiologic studies (EPS) in children with WPW. Methods: This was a retrospective study of children with WPW referred for accessory pathway (AP) ablation without clinical AF. Standard electrophysiologic protocols were performed to induce AVRT. To determine if AF degeneration was associated with patient characteristics, 2-sample t-tests, Chi-square, and Fisher’s exact were used. Results: There were 53 (31 males) WPW pts studied. During EPS, AVRT degenerated to AF in 27/53 (51%). RVR was seen in 18/27 (67%) patients. The ventricular cycle length (CL) during AF was shorter with RVR (211 + 24 ms) than without (313 + 65 ms) (P = 0.01). AF occurred more commonly among patients with right anterior AP (P = 0.05). Patient gender, age, height, weight, body surface area, persistence of preexcitation on exercise testing, baseline CL, AVRT conduction, and AP number were not significant AF determinants. The AVRT CL was significantly shorter in patients with (265.2 + 41.5 ms) versus those without (308 + 59 ms) AF (P = 0.01). Preliminary data suggest that AP location may be related to patient ethnicity. Conclusion: AF with RVR occurred following AVRT induction during EPS in 34% of our WPW patients, typically associated with right-sided AP locations. Time intervals for RVR to degenerate into ventricular fibrillation and lead to SCD are yet to be determined. (PACE 2008; 31:1307‐1312)

Prediction of accessory pathway (AP) location before radiofrequency ablation has become increasingly important for patients with AP; this is especially true for posteroseptal (PS) APs.To identify electrocardiographic and electrophysiologic predictors of pathway location in patients with manifest posteroseptal AP.A detailed electrocardiographic analysis, electrophysiologic study, and ablation were performed in 94 patients with single manifest posteroseptal AP (mean age 35.0 +/- 13.8 years; 56 males).Localization was right PS in 68 patients, left PS in 19 patients, and coronary sinus and its branches in seven patients. Common to all the patients with posteroseptal AP was a negative delta in at least two inferior leads. The most sensitive and specific parameter for differentiating left posteroseptal APs from right posteroseptal APs was an R/S ratioor=1.0 in lead V1 (sensitivity 100% and specificity 100%). The R-wave amplitude in lead I (sensitivity 54%, specificity 67%), and delta ventricularatrial interval (sensitivity 75%, specificity 87%) had much lesser sensitivity and specificity in this regard. The epicardial posteroseptal APs were discriminated from endocardial variant by the positive delta in aVR (sensitivity 71% and specificity 99%) and negative delta in II (sensitivity 100% and specificity 20%). Delta wave polarity in V1 was not helpful for differentiating right-sided from left-sided posteroseptal APs.This study demonstrated that in patients with posteroseptal AP, successful ablation site could be predicted to be on the right or left endocardial surface using R/S ratio in lead V1. Necessity for Coronary sinus catheterization and angiography is predictable using delta wave polarities in leads aVR and II.

Isolated noncompaction of the left ventricle (INLV) is a rare congenital disorder associated with multiple cardiac arrhythmias. We report a case of INLV revealed by ventricular fibrillation in a patient with Wolff-Parkinson-White syndrome. Because of the persistence of inducible ventricular arrhythmias after ablation of the accessory pathway, implantation of an ICD was decided.

Background: The majority of cardiac arrhythmias in children are supraventricular tachycardia, which is mainly related to an accessory pathway (AP)-mediated reentry mechanism. The investigation for Wolff-Parkinson-White (WPW) syndrome in adults is numerous, but there is only limited information for children. This study was designed to evaluate the specific electrophysiologic characteristics and the outcome of radiofrequency (RF) catheter ablation in children with WPW syndrome. Methods: From December 1989 to August 2005, a total of 142 children and 1,219 adults with atrioventricular reentrant tachycardia (AVRT) who underwent ablation at our institution were included. We compared the clinical and electrophysiologic characteristics between children and adults with WPW syndrome. Results: The incidence of intermittent WPW syndrome was higher in children (7% vs 3%, P=0.025). There was a higher occurrence of rapid atrial pacing needed to induce tachycardia in children (67% vs 53%, P=0.02). However, atrial fibrillation (AF) occurred more commonly in adult patients (28% vs 16%, P = 0.003). The pediatric patients had a higher incidence of multiple pathways (5% vs 1%, P < 0.001).Both the onset and duration of symptoms were significantly shorter in the pediatric patients. The antegrade 1:1 AP conduction pacing cycle length (CL) and antegrade AP effective refractory period (ERP) in children were much shorter than those in adults with manifest WPW syndrome. Furthermore, the retrograde 1:1 AP conduction pacing CL and retrograde AP ERP in children were also shorter than those in adults. The antegrade 1:1 atrioventricular (AV) node conduction pacing CL, AV nodal ERP, and the CL of the tachycardia were all shorter in the pediatric patients. Conclusion: This study demonstrated the difference in the electrophysiologic characteristics of APs and the AV node between pediatric and adult patients. RF catheter ablation was a safe and effective method to manage children with WPW syndrome.

An anomalous inferior vena cava (IVC) is often associated with patients with dextrocardia. However, radiofrequency catheter ablation in such a case with that combination has not been reported. We encountered a case of Wolff-Parkinson-White syndrome with dextrocardia associated with complete situs inversus and an azygos continuation of the IVC. A steep angulation at the junction of the azygos vein and superior vena cava precluded the precise mapping of the anatomical right cavity with a femoral vein approach. Successful catheter ablation of an accessory connection between the coronary sinus and left ventricle could be achieved with a right cubital vein approach.

While Drs. Wolff, Parkinson, and White fully described the syndrome that bears their names in 1930, prior case reports had already described the essentials. Over the ensuing century this syndrome has captivated the interest of anatomists, clinical cardiologists, and cardiac surgeons. Stanley Kent described lateral muscular connections over the atrioventricular (AV) groove, which he felt were the normal AV connections. The normal AV connections were, however, clearly described by His and Tawara. True right-sided AV connections were initially described by Wood et al., while Ohnell first described left free wall pathways. David Scherf is thought to be the first to describe our current understanding of the pathogenesis of the Wolff-Parkinson-White (WPW) syndrome in terms of a reentrant circuit involving both the AV node--His axis as well as the accessory pathway. This hypothesis was not universally accepted and many theories were applied to explain the clinical findings. The basics of our understandings were established by the brilliant work of Pick, Langendorf, and Katz who by using careful deductive analysis of ECGs were able to define the basic pathophysiological processes. Subsequently, Wellens and Durrer applied invasive electrical stimulation to the heart in order to confirm the pathophysiological processes. Sealy and his colleagues at Duke University Medical Center were the first to successfully surgically divide an accessory pathway and ushered in the modern area for curative therapy for these patients. Morady and Scheinman were the first to successfully ablate an accessory pathway (posteroseptal) using high-energy direct-current shocks. Subsequently, Jackman, Kuck, Morady, and a number of groups proved the remarkable safety and efficiency of catheter ablation for pathways in all locations using radiofrequency energy. More recently, Gallob et al. first described the gene responsible for a familial form of WPW. The current ability to cure patients with WPW is due to the splendid contributions of individuals from diverse disciplines from throughout the world.

Electrophysiological characteristics of an accessory pathway (AP) with a long ventriculoatrial (VA) interval (arbitrarily defined as > or = 50 ms and absence of continuous electrical activity) and no retrograde decremental property are described in this study. Fifteen patients (group 1) were compared with 171 patients with normal VA conduction (group 2). Mean VA conduction time was 77 +/- 24 versus 34 +/- 12 ms in group 1 versus group 2, respectively. Group 1 patients were older (55 +/- 14 vs 40 +/- 14 years), the male to female ratio was higher (2.8 vs 1.6), and APs were more prevalent on the right (60%) but manifest APs were lower (20% vs 54%) compared to group 2 patients (P < 0.05 in all cases). QRS morphology during induced atrioventricular reciprocating tachycardia was identical in both groups but the tachycardia cycle length was longer in group 1 (373 +/- 29 vs 344 +/- 50 ms, P < 0.05). Retrograde AP block cycle length and effective refractory period were greater in group 1 (362 +/- 59 vs 293 +/- 57 ms; 330 +/- 58 vs 273 +/- 55 ms, both P < 0.05). Adenosine (up to 18 mg) and verapamil (5-10 mg) failed to block the VA conduction via AP during ventricular pacing. In group 1 the number of radiofrequency lesions for a successful ablation were significantly less (3 +/- 2 vs 6 +/- 5, P < 0.05). In conclusion, APs with a long VA interval and no decremental retrograde conduction have electrophysiological characteristics that are different from those with a short VA interval. Role of aging deserves further exploration.

This case report describes a patient with Wolff-Parkinson-White syndrome in whom the ECG exhibited a typical pattern of an anteroseptal (superoparaseptal) accessory pathway. Successful radiofrequency catheter ablation was achieved from the septal side of the left ventricular outflow tract. It might be worthwhile to map the left side of the anterior septum if an accessory pathway potential is not appreciable along the tricuspid annulus to avoid the potential complication of AV block in patients with a typical anteroseptal accessory pathway ECG pattern. (PACE 2004; 27:668–670)

We report a case of a manifest left free wall accessory pathway in a patient with Kartagener's syndrome and recurrent episodes of orthodromic atrioventricular reentrant tachycardia. To the best of our knowledge, it is the first report of Wolff-Parkinson-White syndrome associated with Kartagener's syndrome. Situs inversus and mirror image dextrocardia occurred with no additional detectable cardiac structural abnormalities. Diagnostic and therapeutic electrophysiological study was carried out via transaortic approach and a left-to-right reversal of monoplane fluoroscopic image.

A case of Wolff-Parkinson-White syndrome successfully treated by transcutaneous epicardial radiofrequency ablation is described in a patient with a posteroseptal accessory pathway who had failed prior attempts of conventional endocardial and coronary venous system approaches. Simultaneous endocardial and pericardial space mapping was performed and only ablation from the pericardial space was successful, suggesting an epicardial course of the accessory pathway.

A 29-year-old man was referred for electrophysiological testing and radiofrequency ablation because of repeated episodes of palpitation over 2 years. A 12-lead electrocardiogram during sinus rhythm showed manifest Wolff-Parkinson-White syndrome and during palpitation showed narrow QRS tachycardia at a rate of 213 beats/min. Following successful radiofrequency ablation of the left anterolateral accessory pathway, sustained atrial fibrillation was induced by atrial extrastimulation. Cibenzoline (2 mg/kg body weight) was injected to terminate atrial fibrillation. ST-T segment elevation in the right precordial leads was observed following cibenzoline administration. Ventricular fibrillation was reproducibly induced by ventricular extrastimuli (S1: 600 ms, S2: 220 ms, S3: 210 ms). (PACE 2004; 27:109–111)

The interest of electrohysiological study for the prognostic evaluation of asymptomatic Wolff-Parkinson-White (WPW) syndrome remains controversial. We report the case of an asymptomatic 67-year-old man without heart disease in whom a type A WPW syndrome was noted. Because the WPW was unchanged during exercise testing, transesophageal EPS was performed. In basal state, 1/1 conduction through the Kent bundle was noted up to a rate of 210 beats/min. After infusion of 30 μg of isoproterenol, atrial pacing was associated with a 1/1 conduction throught the Kent bundle at a rate at 300 beats/min and induced rapid atrial fibrillation which was stopped by flecainide. No treatment was indicated. Nine years later, at age 76, the patient developed syncope related to rapid atrial fibrillation requiring cardioversion. In conclusion, the occurrence of a potentially lethal supraventricular tachyarrhythmia in a previously asymptomatic patient with WPW syndrome might be encountered in elderly patients. Transesophageal electrophysiological evaluation is a useful means to predict this risk. (PACE 2003; 26:1769–1770)

In clinical practice, noninvasive classification of ventricular preexcitation (VPX) is usually done with ECG algorithms, which provide only a qualitative localization of accessory pathways. Since 1984, single or multichannel magnetocardiography (MMCG) has been used for three-dimensional localization of VPX sites, but a systematic study comparing the results of ECG and MMCG methods was lacking. This study evaluated the reliability of MMCG in an unshielded electrophysiological catheterization laboratory, and compared VPX classification as achieved with the five most recent ECG algorithms with that obtained by MMCG mapping and imaging techniques. A nine-channel direct current superconducting quantum interference device (DC-SQUID) MMCG system (sensitivity is 20 fT/Hz0.5) was used for sequential MMCG from 36 points on the anterior chest wall, within an area 20 x 20 cm. Twenty-eight patients with Wolff-Parkinson-White syndrome were examined at least twice, on the same day or after several months to test the reproducibility of the measurements. In eight patients, the reproducibility of MMCG was also evaluated using different MCG instrumentation during maximal VPX and/or atrioventricular reentrant tachycardia induced by transesophageal atrial pacing via a nonmagnetic catheter. The results of VPX localization with ECG algorithms and MMCG were compared. Equivalent current dipole, effective magnetic dipole, and distributed currents imaging models were used for the inverse solution. MMCG classification of VPX was found to be more accurate than ECG methods, and also provided additional information for the identification of paraseptal pathways. Furthermore, in patients with complex activation patterns during the delta wave, distributed currents imaging revealed two different activation patterns, suggesting the existence of multiple accessory pathways.

A 62-year-old woman with Wolff-Parkinson-White syndrome had two types of tachycardia; ectopic AT and orthodromic-type AVRT. A radiofrequency application 2 cm inside the coronary sinus ostium eliminated ectopic AT and accessory pathway conduction at once.

Transcutaneous pericardial instrumentation in a patient with a left-sided posteroseptal accessory pathway associated with a large coronary sinus diverticulum was performed after three previous unsuccessful ablations. The earliest ventricular activation site was recorded epicardially using a catheterin the pericardial space, but energy delivery at this site was not possible due to high impedances. Intravascular linear lesions transecting the neck of the large coronary sinus diverticulum using the pericardial catheter as a target allowed successful radiofrequency catheter ablation of the accessory pathway. This case outlines potential problems with ablations in the pericardial space and provides an alternative solution that can spare patients from open surgical procedures.

This report describes a male patient with WPW syndrome who underwent surgical repair of sudden, severe mitral valve regurgitation through the posterior leaflet 20 months after successful RF catheter ablation for Kent bundle at the age of 14, and discusses the problem of valvular damage caused by this technique.

VF was observed in four patients (group A) with preexcited AF presenting to the emergency department who had been given 12 mg of adenosine. These patients were resuscitated and underwent electrophysiological study and catheter ablation of the accessory pathway (AP). In a control (group B) of five patients with manifest AP, sustained AF was induced by rapid atrial pacing during electrophysiological study and 12 mg of adenosine was administered. The ECG and electrophysiologic features in the two groups were compared. All patients had a single manifest AP. In group A, three patients had a left free-wall AP and one patient had a posteroseptal AP, while in the control group all had left free-wall APs. The antegrade AP effective refractory period (ERP) in groups A and B was 227 +/- 29 and 289 +/- 37 ms, respectively (P0.05). The atrial ERP was 210 +/- 17 versus 219 +/- 21 ms, respectively, in groups A and B (P0.05). The shortest R-R interval during AF in group A was 246 +/- 51 ms and 301 +/- 60 ms in group B (P value0.05). After adenosine, no patient in group B developed VF. Adenosine may cause VF when administered during preexcited AF. This phenomenon is seen in patients having APs with short refractory periods.

BREMBILLA-PERROT, B., et al.: Influence of Age on the Potential Risk of Sudden Death in Asymptomatic Wolff-Parkinson-White Syndrome. Sudden death might be the first event in patients with asymptomatic WPW. The purpose of the study was to know if the age of the patient modifies the electrophysiological characteristics of asymptomatic WPW. Transesophageal stimulation was performed on 92 asymptomatic WPW patients from the following age groups: 10–69 years (n =14), 20–29 years (n = 33), 30–39 years (n = 15), 40–49 years (n = 17), and 50–69 years (n = 13). The procedure consisted of atrial pacing up to the second AV block, programmed atrial stimulation using one and two extrastimuli delivered on two driven rhythms in the control state, and after infusion of isoproterenol. In the five age groups, paroxysmal junctional tachycardia occurred, respectively, in 1 (7%), 1 (3%), 2 (13%), 2 (12%) patients, and not at all in the oldest group. AF > 1 minute occurred, respectively, in 3 (21%), 9 (27%), 5 (33%), 3 (18%), and 4 (31%) patients. The dangerous form of WPW (rapid conduction in the Kent bundle > 240/min in the control state or > 300/min after isoproterenol + AF induction) occurred, respectively, in 3 (21%), 9 (27%), 4 (27%), 1 (6%), and 3 (23%) patients. In conclusion, electrophysiological data of asymptomatic WPW are not modified by age of the patient. Elderly patients remain at risk of a dangerous form of WPW; systematic evaluation of WPW is recommended in patients with an active life independent of age.

Previous studies have shown that with low temperature testing for RF ablation of arrhythmias, unnecessary irreversible myocardial lesions may be avoided. In children admitted for RF ablation from June 1996 to May 1999, we evaluated the method of an initial temperature setting of 50 degrees C for a maximum of 10 seconds. If accessory pathway block occurred, the temperature setting was immediately increased to 70 degrees C and continued for 45-120 seconds (group 1). If accessory pathway block did not occur after several attempts, subsequent attempts were made with initial settings at 70 degrees C-80 degrees C at the same or different sites (group 2). Eighty patients with Wolff-Parkinson-White syndrome (mean age 11 +/- 4 years) were treated using this method. Twelve patients were excluded for various reasons. Of the remaining 68 patients, 52 (76%) had successful block of the pathway at 50 degrees C; 16 patients demonstrated block only at the higher temperature setting of 70 degrees C-80 degrees C. There were no statistically significant differences between these two groups in terms of age, weight, and location of accessory pathways. Unsuccessful 50 degrees C test ablation attempts were 1.6 +/- 2.4 in group 1 and 3.1 +/- 2.9 in group 2 (P = 0.04). Total unsuccessful attempts were 1.6 +/- 2.4 in group 1 and 8.1 +/- 7.1 in group 2 (P = 0.001). The time from application of RF energy to the time of AP block in group 1 was not significantly different from group 2. In the majority of children, successful RF ablation can be achieved by using a temperature setting of 50 degrees C, then 70 degrees C. This will prevent unnecessary permanent injury at unsuccessful attempt sites.

LU, C.-W., et al.: Paroxysmal Supraventricular Tachycardia in Identical Twins with the Same Left Lateral Accessory Pathways and Innocent Dual Atrioventricular Pathways. We report on 16-year-old, female identical twins who both have atrioventricular reentrant tachycardia caused by the same left lateral atrioventricular accessory pathway. The Kent pathway in twin A was a unidirectional retrograde accessory pathway. A manifest Kent pathway was demonstrated in twin B. Both pathways were successfully ablated by radiofrequency (RF) energy and without recurrence. In addition, innocent dual AV nodal pathways were shown in both patients. These findings suggest that genetic factors may play a role in the pathogenesis of the formation of accessory atrioventricular pathways and dual AV nodal pathways.