The Wolff-Parkinson-White pattern in neonates: results from a large population-based cohort study.

AIMS: Wolff-Parkinson-White (WPW) syndrome is a conduction disorder characterized by an accessory electrical pathway between the atria and ventricles, which may predispose to supraventricular tachycardia (SVT) and sudden cardiac death. It can be seen as an isolated finding or associated with structural heart disease. Our aims were to determine the prevalence of a WPW pattern in a large and unselected cohort of neonates and to describe the electro- and echocardiographic characteristics as well as the natural history during early childhood. METHODS AND RESULTS: Electrocardiograms and echocardiograms of neonates (aged 0-30 days) from a large, prospective, population-based cohort study were included. Neonates with a WPW pattern were identified and matched 1:4 to controls. Localization of the accessory pathway was assessed by different algorithms. Among 17 489 neonates, we identified 17 (76% boys) with a WPW pattern consistent with a prevalence of 0.1%. One neonate had moderate mitral regurgitation while other echocardiographic parameters were similar between cases and controls (all P > 0.05). The accessory pathways were primarily predicted to be left-sided. At follow-up (available in 14/17 children; mean age 3.2 years) the pre-excitation pattern persisted in only four of the children and none of the children had experienced any episodes of SVT. CONCLUSION: The prevalence of a WPW pattern in our cohort of unselected neonates was 0.1%. The WPW pattern was more frequent in boys and generally not associated with structural heart disease, and the accessory pathways were primarily left-sided. At follow-up, the WPW pattern had disappeared in most of the children suggesting either an intermittent nature or that normalization occurs. CLINICAL TRIAL REGISTRATION: Copenhagen Baby Heart, NCT02753348.

Association of Wolff-Parkinson-White With Left Ventricular Noncompaction Cardiomyopathy in Children.

BACKGROUND: Wolff-Parkinson-White (WPW) has been associated with left ventricular noncompaction (LVNC) in children. Little is known about the prevalence of this association, clinical outcomes, and treatment options. METHODS: Retrospective review of subjects with LVNC. LVNC was defined by established criteria; those with congenital heart disease were excluded. Electrocardiograms (ECGs) were reviewed for presence of pre-excitation. Outcomes were compared between those with isolated LVNC and those with WPW and LVNC. RESULTS: A total of 348 patients with LVNC were identified. Thirty-eight (11%) were found to have WPW pattern on ECG, and 84% of those with WPW and LVNC had cardiac dysfunction. In Kaplan-Meier analysis, there was significantly lower freedom from significant dysfunction (ejection fraction ≤ 40%) among those with WPW and LVNC (P < .001). Further analysis showed a higher risk of developing significant dysfunction in patients with WPW and LVNC versus LVNC alone (hazard ratio 4.64 [2.79, 9.90]). Twelve patients underwent an ablation procedure with an acute success rate of 83%. Four patients with cardiac dysfunction were successfully ablated, 3 having improvement in function. CONCLUSION: WPW is common among children with LVNC and is associated with cardiac dysfunction. Ablation therapy can be safely and effectively performed and may result in improvement in function.

Cardiac electrophysiologic procedures - A ten years' experience at National Institute of Cardiovascular Diseases, Karachi.

OBJECTIVE: To review 10 years of clinical practice of cardiac electrophysiology study and radiofrequency catheter ablation in the treatment of supraventricular tachycardia. METHODS: The retrospective chart review was conducted at the National Institute of Cardiovascular Diseases, Karachi, and comprised records of all patients who underwent electrophysiological study and / or radiofrequency catheter ablation from January2007 to December 2016. SPSS 21 was used for data analysis. RESULTS: Of the 627 patients, 335(53.4%) were females. The overall mean age was 40.99}13.59 years. The major indication for procedure was supraventricular tachycardia 376(59.97%). Final electrophysiological study diagnosis was typical slow fast atrioventricular nodal re-entrant tachycardia in 303(48.3%) patients. The overall success rate was 472(75.3%). Procedure-related complications were reported in 25(4%) patients, and there was 1(0.15%) mortality. CONCLUSIONS: Cardiac electrophysiology studies and radiofrequency catheter ablation were found to be an effective and safe method for diagnosis and treatment of supraventricular tachycardia.

[Preexcitation Syndromes in Children and Adolescents].

Preexcitation syndromes are becoming a more often detected disease in the pediatric population. Their essential feature is the presence of additional paths / pathways in the heart, through which the impulse is conducted faster than physiologically. This leads to faster ventricular excitation and may lead to creating dangerous arrhythmias. The most common preexcitation syndrome is Wolff - Parkinson-White syndrome, which affects 2/ 1000 people. The presence of an accessory pathway could result in serious consequences, ranging from supraventricular tachycardia to sudden cardiac death. There are both invasive and non-invasive methods of diagnosing preexcitation syndromes. Therapeutic treatment includes pharmacotherapy and ablation, which makes it possible to permanently remove the cause of the arrhythmia.

Wolff-Parkinson-White syndrome: lessons learnt and lessons remaining.

The Wolff-Parkinson-White pattern refers to the electrocardiographic appearance in sinus rhythm, wherein an accessory atrioventricular pathway abbreviates the P-R interval and causes a slurring of the QRS upslope - the "delta wave". It may be asymptomatic or it may be associated with orthodromic reciprocating tachycardia; however, rarely, even in children, it is associated with sudden death due to ventricular fibrillation resulting from a rapid response by the accessory pathway to atrial fibrillation, which itself seems to result from orthodromic reciprocating tachycardia. Historically, patients at risk for sudden death were characterised by the presence of symptoms and a shortest pre- excited R-R interval during induced atrial fibrillation <250 ms. Owing to the relatively high prevalence of asymptomatic Wolff-Parkinson-White pattern and availability of catheter ablation, there has been a need to identify risk among asymptomatic patients. Recent guidelines recommend invasive evaluation for such patients where pre-excitation clearly does not disappear during exercise testing. This strategy has a high negative predictive value only. The accuracy of this approach is under continued investigation, especially in light of other considerations: Patients having intermittent pre-excitation, once thought to be at minimal risk may not be, and the role of isoproterenol in risk assessment.

Risk of malignant arrhythmias in initially symptomatic patients with Wolff-Parkinson-White syndrome: results of a prospective long-term electrophysiological follow-up study.

BACKGROUND: The available amount of detailed long-term data in patients with Wolff-Parkinson-White syndrome is limited, and no prospective electrophysiological studies looking at predictors of malignant arrhythmia are available. METHODS AND RESULTS: Among 8575 symptomatic Wolff-Parkinson-White patients with atrioventricular reentrant tachycardia referred for electrophysiological test, 369 (mean age, 23±12.5 years) declined catheter ablation and were followed up. The primary end point of the study was to evaluate over a 5-year follow-up the predictors and characteristics of patients who develop malignant arrhythmias. After a mean follow-up of 42.1±10 months, malignant arrhythmias developed in 29 patients (mean age, 13.9±5.6 years; 26 male), resulting in presyncope/syncope (25 patients), hemodynamic collapse (3 patients), or cardiac arrest caused by ventricular fibrillation (1 patient). Of the remaining 340 patients, 168 (mean age, 34.2±9.0 years) remained asymptomatic up to 5 years, and 172 (mean age, 13.6±5.1 years) had benign recurrence, including sustained atrioventricular reentrant tachycardia (132 patients) or atrial fibrillation (40 patients). Compared with the group with no malignant arrhythmias, the group with malignant arrhythmias showed shorter accessory-pathway effective refractory period (P<0.001) and more often exhibited multiple accessory pathways (P<0.001), and atrioventricular reentrant tachycardia triggering sustained pre-excited atrial fibrillation was more frequently inducible (P<0.001). Multivariable analysis demonstrated that short accessory-pathway effective refractory period (P<0.001) and atrioventricular reentrant tachycardia triggering sustained pre-excited atrial fibrillation (P<0.001) were independent predictors of malignant arrhythmias. CONCLUSIONS: Symptomatic patients with Wolff-Parkinson-White syndrome generally have a good outcome, and predictors of malignant arrhythmias are similar to those reported for asymptomatic patients with ventricular pre-excitation.

Current strategy for treatment of patients with Wolff-Parkinson-White syndrome and asymptomatic preexcitation in Europe: European Heart Rhythm Association survey.

The aims of this survey was to provide insight into treatment activity, the strategy of treatment, and risk stratification of patients with asymptomatic and symptomatic ventricular pre-excitation across Europe. Fifty-eight centres, members of the European Heart Rhythm Association EP research network, covering 20 countries answered the survey questions. All centres were high-volume ablation centres. A younger person with asymptomatic Wolff-Parkinson-White (WPW) pattern has a higher likelihood of being risk-stratified or receiving ablation therapy compared with an older subject. Two-thirds of centres report that they have observed a decline in the number of patients ablated for an accessory pathway during the last 10 years. Pre-excited atrial fibrillation is rarely seen. Discontinuation of a scheduled WPW ablation due to close vicinity of the accessory pathway to the AV node happens very rarely. Patients with a first episode of pre-excited atrial fibrillation would immediately be referred for catheter ablation to be performed within weeks by 80.4% of the centres. A significant proportion of responders (50.9%) would use electrical cardioversion to restore sinus rhythm in a patient with pre-excited atrial fibrillation. With respect to the choice of antiarrhythmic medication for a patient with pre-excited AF, the majority (80.0%) would choose class 1C antiarrhytmic drugs while waiting for a catheter ablation. A patient seen in the emergency room with a second episode of orthodromic atrioventricular reentry tachycardia would be referred for immediate ablation by 79.2-90.6% of centres depending on the presence of pre-excitation. The volume of paediatric ablations performed on children younger than 12 years was low (46.4%: 0 patients per year; 46.4%: 1-9 patients per year). The majority of responding centres (61-69%) report that their country lack national guidelines dealing with clinical strategies related to WPW. There is a need for national guidelines dealing with clinical strategy in patients with WPW syndrome. Older individuals with asymptomatic WPW pattern have a higher risk of not receiving risk stratification or curative therapy with ablation compared with younger patients, despite the higher risk of developing atrial fibrillation.

Age-related prognosis of syncope associated with a preexcitation syndrome.

BACKGROUND: 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. METHODS: 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. RESULTS: Potentially malignant form (rapid conduction in accessory pathway >240 beats/min in control state or >300 beats/min after isoproterenol and atrial fibrillation [AF] induction) was more frequent in group I (34%) than in groups II (7%) (P < 0.002) and III (0%) (P < 0.001). 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. CONCLUSIONS: 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.

Association of Weight With Ablation Outcomes in Pediatric Wolff-Parkinson-White: Analysis of the NCDR IMPACT Registry.

BACKGROUND: Guidelines for electrophysiology study (EPS) and catheter ablation in Wolff-Parkinson-White (WPW) are age based, but size may be a more relevant factor in determination of outcomes. OBJECTIVES: The goal of this study was to evaluate the association of patient weight with outcomes of catheter ablation for pediatric WPW. METHODS: A multicenter retrospective cohort study was performed on children aged 1 to 21 years with WPW and first-time EPS from April 2016 to December 2019 recorded in the IMPACT (Improving Pediatric and Adult Congenital Treatment) registry, excluding those with congenital heart disease, cardiomyopathy, and >1 ablation target. A weight threshold of 30 kg was selected, representing 1 SD below the cohort mean. The primary outcome was major adverse events (MAEs); additional outcomes included deferred ablation, use of cryoablation, and ablation success. RESULTS: A total of 4,456 subjects from 84 centers were evaluated, with 14% weighing <30 kg. Subjects weighing <30 kg were more likely to have preprocedural supraventricular tachycardia (45% vs 29%; P < 0.001) and less likely to have right septal accessory pathways (25% vs 33%; P < 0.001). MAEs were rare, although with higher incidence in the <30 kg cohort (0.3% vs 0.05%; P = 0.04). No difference was seen in likelihood of deferred ablation (9% vs 12%; P = 0.07) or use of cryoablation (11% vs 11%; P = 0.70). Success was higher in the <30 kg cohort: 95% vs 92% (P = 0.009). This effect persisted after adjusting for covariates (odds ratio: 1.6; 95% CI: 1.01-2.70; P = 0.046). CONCLUSIONS: Weight <30 kg was associated with a small but elevated risk of MAEs. Rates of deferred ablation and cryoablation were similar. Adjusting for factors (including accessory pathway type and location), weight <30 kg remained an independent predictor of acute success.

Transesophageal evaluation of asymptomatic Wolff-Parkinson-White syndrome.

BACKGROUND: 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). METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Incidence of life-threatening events in children with Wolff-Parkinson-White syndrome: Analysis of a large claims database.

BACKGROUND: Previous estimates of life-threatening event (LTE) risk in Wolff-Parkinson-White (WPW) syndrome are limited by selection bias inherent to tertiary care referral-based cohorts. OBJECTIVE: This analysis sought to measure LTE incidence in children with WPW syndrome in a large contemporary representative population. METHODS: A retrospective cohort study was conducted using claims data from the IBM MarketScan Research Databases, evaluating subjects with WPW syndrome (age 1-18 years) from any encounter between January 1, 2013, and December 31, 2018. Subjects with congenital heart disease and cardiomyopathy were excluded. The primary outcome was diagnosis of ventricular fibrillation (VF); a composite outcome, LTE, was defined as occurrence of VF and/or cardiac arrest. VF and LTE rates were compared to matched representative controls without WPW syndrome (3:1 ratio). RESULTS: The prevalence of WPW syndrome was 0.03% (8733/26,684,581) over a median follow-up of 1.6 years (interquartile range 0.7-2.9 years). Excluding congenital heart disease/cardiomyopathy, 6946 subjects were analyzed. An LTE occurred in 49 subjects (0.7%), including VF in 20 (0.3%). The incidence of VF was 0.8 events per 1000 person-years, and the incidence of LTE was 1.9 events per 1000 person-years. There were no occurrences of VF in controls; the rate of LTE was 70 times higher in subjects with WPW syndrome (0.7%; 95% confidence interval 0.5%-0.9%) than in controls (0.01%; 95% confidence interval 0%-0.02%). CONCLUSION: The use of a large claims data set allowed for an evaluation of VF and LTE risk in an unselected pediatric population with WPW syndrome. The observed range of 0.8-1.9 events per 1000 person-years is consistent with prior reports from selected populations. A comparison of event rates to matched controls confirms and quantifies the significant elevation in VF and LTE risk in pediatric WPW syndrome.

Early repolarization in Wolff-Parkinson-White syndrome: prevalence and clinical significance.

AIMS: Idiopathic ventricular fibrillation (IVF) with early repolarization (ER) has recently been reported; however, ER is a common finding in healthy subjects and is also found sporadically in patients with Wolff-Parkinson-White (WPW) syndrome. The present study was designed to evaluate the prevalence and clinical significance of ER in patients with WPW syndrome. METHODS AND RESULTS: One hundred and eleven patients with WPW syndrome were studied retrospectively. Early repolarization was defined as QRS slurring or notching with J-point elevation ≥ 1 mm. The prevalence of ER was determined before and after successful catheter ablation. Before ablation, ER was found in 35 of 75 patients with a left free wall, 6 of 23 with a right free wall, and 7 of 13 with a septal accessory pathway (48 of 111, 43% as a whole). Early repolarization was always observed in leads with positive deflection of the initial part of the delta wave. After successful ablation of accessory pathways, ER was preserved in 28 (25%), disappeared in 20 (18%), and newly developed in 8 (7%) patients. In the remaining 55 (50%) patients, ER was not observed either before or after ablation. In patients with persistent ER, the amplitude and width of ER were significantly decreased 3-7 days after the ablation (1.7 ± 0.7 vs. 1.4 ± 0.6 mm, P < 0.005 and 42 ± 11 vs. 34 ± 9 ms, P < 0.001, respectively). CONCLUSION: In patients with WPW syndrome, ER could be partly related to early depolarization through the accessory pathway. However, persistent ER and new ER appearing after the ablation were frequently found. Therefore, in these patients, mechanisms other than early depolarization may be involved in the genesis of ER.

Emergency treatment of Wolff-Parkinson-White syndrome.

This article describes the aetiology of Wolff-Parkinson-White syndrome and discusses the benefits and disadvantages of some of the available cardioversion medications with which it can be treated.

Prevalence and incidence of adverse events with catheter ablation for adults with Wolff-Parkinson-White syndrome: a systematic review protocol.

OBJECTIVE: The objective of this review is to synthesize the best available evidence to determine the prevalence and incidence of adverse events in patients with Wolff-Parkinson-White syndrome who have undergone catheter ablation. INTRODUCTION: Wolff-Parkinson-White syndrome is a rare congenital heart disease affecting the normal cardiac conduction system that predisposes an individual to tachyarrhythmias. Patients with Wolf-Parkinson-White syndrome can experience a wide range of life-threatening symptoms and frequently undergo catheter ablation procedures to treat this disease. These patients are at risk of developing a variety of adverse events, including pericardial effusion, cardiac tamponade, atrial fibrillation, stroke, cerebral hemorrhage, major bleeding or hematoma, deep vein thrombosis, atrioventricular-block, cardiac perforation, coronary artery injury, pulmonary emboli, and death. INCLUSION CRITERIA: This review will include adults aged 18 or older with a diagnosis of Wolff-Parkinson-White syndrome who have undergone catheter ablation, including radiofrequency ablation and cryoablation. METHODS: MEDLINE, CINAHL, Scopus, Embase, and Web of Science databases will be searched from 1980 to the present for English-language studies only. Prevalence and incidence data, experimental, quasi-experimental, observational, and descriptive studies will be included and critically appraised by two independent reviewers. Data will be extracted using the standardized data extraction tool for prevalence data. If sufficient data is available, a meta-analysis will be conducted; otherwise, the findings will be presented in narrative form including tables and figures to aid in data presentation where appropriate. SYSTEMATIC REVIEW REGISTRATION NUMBER: PROSPERO CRD42020180391.

Outcomes of ablation in Wolff-Parkinson-White-syndrome: Data from the German Ablation Registry.

AIMS: Catheter ablation is recommended for symptomatic WPW-syndrome. Commonly perceived low recurrence rates were challenged recently. We sought to identify patient strata at increased risk. METHOD: Of 12,566 patients enrolled at 52 German Ablation Registry sites from 2007 to 2010, 789 were treated for WPW-syndrome. Patients were included for symptomatic palpitations and tachycardia documentation. Follow-up duration was one year. Overall complications were defined as serious, access-related, and ablation-related. We adjudicated WPW-recurrence for re-ablation during follow-up. Risk strata included: admission for repeat ablation at registry entry; accessory pathway localization; antiarrhythmic medical treatment before the ablation. RESULTS: WPW-syndrome patients were 42.8 ± 16.2 years on average; 39.9% were women. A majority of 95.9% was symptomatic; in 84.4%, a tachycardia was documented. Seventy-six (9.6%) patients presented for repeat procedures. Accessory pathways were located in the left atrium (71.4%), right atrium (21.1%), septum (4.4%), or coronary sinus diverticula (2.1%). Prior antiarrhythmic medication was used in 43.7% of patients. No serious events occurred. The overall complication rate was 2.5% (ablation related 1.2%, access-related 1.3%). Major determinants for complications were presentation for re-ablation as registry index procedure (6.9% vs 2.2%; p = 0.016) and septal pathway location (left 2.0% vs septal 9.1%, p = 0.014). The overall re-ablation rate was 9.7%. Usage of prior antiarrhythmic medication was associated with higher recurrence rates (12.2% vs. 7.6%; p = 0.035). CONCLUSIONS: Patients at higher complication risk may be identified by repeat procedure and septal pathway location. Prior antiarrhythmic medication was associated with higher recurrence rates. Our findings may help improving peri-procedural patient management and information.

Incidental dual atrioventricular nodal physiology in children and adolescents: clinical follow-up and implications.

BACKGROUND: Dual atrioventricular (AV) nodal physiology is a substrate for the development of AV nodal reentrant tachycardia (AVNRT). However, the risk of developing AVNRT in patients with dual AV nodal physiology is not known. The purpose of this study is to identify the risk of developing AVNRT in children and adolescents with incidental findings of dual AV nodal physiology after accessory pathway ablation. METHODS: This is a single center retrospective study of patients who underwent intracardiac electrophysiology study at The Children's Hospital, Denver, from March 1993 to August 2008, with findings of dual AV nodal physiology after successful ablation of an accessory pathway. Follow-up was obtained by chart review with the primary outcome of recurrent supraventricular tachycardia. Extended clinical follow-up was also achieved through phone contact with patients or parents of patients. RESULTS: Mean age at initial electrophysiology study was 12.8 years (±3.7 years). Follow-up was obtained on all 66 patients for a mean duration of 3.1 years (±2.8 years). Mean age at follow-up was 15.8 years (±4.6 years). Recurrent supraventricular tachycardia occurred in nine of the 66 patients (13.6%). AVNRT was induced in two of the 66 patients (3.0%). CONCLUSION: This study supports the hypothesis that incidental dual AV nodal physiology does not predict AVNRT in children and adolescents with after successful accessory pathway ablation.

Risk factors of adverse presentation as the first arrhythmia in Wolff-Parkinson-White syndrome.

BACKGROUND: 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). METHODS: 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. RESULTS: 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%) (P < 0.001), 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%) (P < 0.001), less frequent than in group IIb (90.5%) (P < 0.001). AF was more frequent in group I (85%) than in group IIc (22%), or IIb (19%) (P < 0.001). Maximal rate through AP was higher in group I than in group II (P < 0.001). CONCLUSIONS: 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.

A multicenter, long-term study on arrhythmias in children with Ebstein anomaly.

To assess the prevalence, history, and treatment of arrhythmias, in particular preexcitation and Wolff-Parkinson-White (WPW) syndrome, in patients with Ebstein anomaly (EA) during childhood and adolescence, we performed a multicenter retrospective study of all consecutive live-born patients with EA, diagnosed, and followed by pediatric cardiologists between 1980 and 2005 in The Netherlands. During a follow-up after EA diagnosis of 13 years 3 months (range: 6 days to 28 years 2 months), 16 (17%) of the 93 pediatric EA patients exhibited rhythm disturbances. Nine patients showed arrhythmic events starting as of the neonatal period. Supraventricular tachycardia was noted in 11 patients. One patient died in the neonatal period due to intractable supraventricular tachycardia resulting in heart failure and one patient died at 5 weeks of age most probably due to an arrhythmic event. The 14 surviving patients all show preexcitation, albeit 4 of them intermittently, and all have a right-sided accessory pathway location. Nine patients underwent catheter ablation of an accessory pathway. Only four patients are currently on antiarrhythmic drugs. The 17% prevalence of rhythm disturbances in pediatric EA patients, most commonly supraventricular arrhythmias, is significantly lower than in adult EA patients. Life-threatening rhythm disturbances are not frequent early in life. Symptomatic patients are well treated with radiofrequency catheter ablation.