Management of Asymptomatic Wolff-Parkinson-White Pattern in Young Patients: Has Anything Changed?

The approach to pediatric asymptomatic Wolff-Parkinson-White (WPW) patients is controversial. The objective of this review is to update the last consensus of specialists of the Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society on this subject in order to summarize the most recent evidence on the management of young patients with asymptomatic WPW pattern. A systematic review of the literature published between 2008 and 2018 was performed taking into account the protocol of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) in PubMed (including Cochrane), Embase, and Web of Science. Observational, experimental, and multicentric studies were included. Out of a total of 37 articles selected, 4 were considered eligible. Most studies considered a cutoff age of 8 or greater as recommended in the 2012 consensus. The identification of a shortest pre-excitatory RR interval (SPERRI) ≤ 250 ms seems to be the best predictor for risk stratification. The importance of routine isoprenaline use to improve the sensitivity of the electrophysiological study to identify patients at high risk of sudden death was consensual. Prophylactic ablative therapy has been indicated in asymptomatic children with an accessory pathway (AP) who have a low SPERRI and/or a low effective anterograde period of the AP and/or multiple APs. Despite the evidence found in the most recent studies, more studies are warranted in this setting.

Catheter ablation in ASymptomatic PEDiatric patients with ventricular preexcitation: results from the multicenter "CASPED" study.

BACKGROUND: As there are limited data about the clinical practice of catheter ablation in asymptomatic children and adolescents with ventricular preexcitation on ECG, we performed the multicenter "CASPED" (Catheter ablation in ASymptomatic PEDiatric patients with Ventricular Preexcitation) study. METHODS AND RESULTS: In 182 consecutive children and adolescents aged between 8 and 18 years (mean age 12.9 ± 2.6 years; 65% male) with asymptomatic ventricular preexcitation, a total of 196 accessory pathways (APs) were targeted. APs were right sided (62%) or left sided (38%). The most common right-sided AP location was the posteroseptal region (38%). Ablation was performed using radiofrequency (RF) energy (93%), cryoablation (4%) or both (3%). Mean procedure time was 137.6 ± 62.0 min with a mean fluoroscopy time of 15.6 ± 13.8 min. A 3D mapping or catheter localization system was used in 32% of patients. Catheter ablation was acutely successful in 166/182 patients (91.2%). Mortality was 0% and there were no major periprocedural complications. AP recurrence was observed in 14/166 patients (8.4%) during a mean follow-up time of 19.7 ± 8.5 months. A second ablation attempt was performed in 20 patients and was successful in 16/20 patients (80%). Overall, long-term success rate was 92.3%. CONCLUSION: In this retrospective multicenter study, the outcome of catheter ablation for asymptomatic preexcitation in children and adolescents irrespective of antegrade AP conduction properties is summarized. The complication rate was low and success rate was high, the latter mainly depending on pathway location. The promising results of the study may have future impact on the ongoing risk-benefit discussion regarding catheter ablation in the setting of asymptomatic preexcitation in children and adolescents.

Life-Threatening Event Risk in Children With Wolff-Parkinson-White Syndrome: A Multicenter International Study.

OBJECTIVES: This study sought to characterize risk in children with Wolff-Parkinson-White (WPW) syndrome by comparing those who had experienced a life-threatening event (LTE) with a control population. BACKGROUND: Children with WPW syndrome are at risk of sudden death. METHODS: This retrospective multicenter pediatric study identified 912 subjects ≤21 years of age with WPW syndrome, using electrophysiology (EPS) studies. Case subjects had a history of LTE: sudden death, aborted sudden death, or atrial fibrillation (shortest pre-excited RR interval in atrial fibrillation [SPERRI] of ≤250 ms or with hemodynamic compromise); whereas subjects did not. We compared clinical and EPS data between cases and subjects. RESULTS: Case subjects (n = 96) were older and less likely than subjects (n = 816) to have symptoms or documented tachycardia. Mean age at LTE was 14.1 ± 3.9 years of age. The LTE was the sentinel symptom in 65%, consisting of rapidly conducted pre-excited atrial fibrillation (49%), aborted sudden death (45%), and sudden death (6%). Three risk components were considered at EPS: SPERRI, accessory pathway effective refractory period (APERP), and shortest paced cycle length with pre-excitation during atrial pacing (SPPCL), and all were shorter in cases than in control subjects. In multivariate analysis, risk factors for LTE included male sex, Ebstein malformation, rapid anterograde conduction (APERP, SPERRI, or SPPCL ≤250 ms), multiple pathways, and inducible atrial fibrillation. Of case subjects, 60 of 86 (69%) had ≥2 EPS risk stratification components performed; 22 of 60 (37%) did not have EPS-determined high-risk characteristics, and 15 of 60 (25%) had neither concerning pathway characteristics nor inducible atrioventricular reciprocating tachycardia. CONCLUSIONS: Young patients may experience LTE from WPW syndrome without prior symptoms or markers of high-risk on EPS.

Delta wave notching time is associated with accessory pathway localization in patients with Wolff-Parkinson-White syndrome.

PURPOSE: Our aim was to investigate the relation between delta wave notching time (DwNt) and accessory pathway location in patients with Wolff-Parkinson-White (WPW) syndrome. METHODS: The retrospective study included 149 WPW patients who underwent ablation therapy. DwNt was defined as the duration between the initial point of QRS and the notching in the delta wave. DwNt was divided by QRS duration to obtain the delta wave index (Dwi). RESULTS: Patients with left-sided accessory pathway (AP) had significantly higher DwNt (p < 0.001) and Dwi (p = 0.027) values. The R wave voltage in lead I (p = 0.037) and S wave voltage in lead V1 (p = 0.005) values were significantly higher in patients with right-sided AP compared to patients with left-sided AP. When 27 ms was taken as the DwNt cut-off value, higher durations determined the left-sided AP location with a sensitivity of 91% and a negative predictive value of 91.4%. Dwi cutoff values ≥ 0.29 were accepted to indicate a left-sided AP location with a sensitivity of 91.2% and a NPV of 91.4%. CONCLUSIONS: WPW patients with left-sided AP have longer DwNt values than patients with right-sided AP.

Radiofrequency ablation of accessory pathways in patients with the Wolff-Parkinson-White syndrome: long-term risk of mortality and coronary events.

Aims: The long-term outcomes of radiofrequency catheter ablation (RFCA) in patients with Wolff-Parkinson-White syndrome (WPW) remain unclear. We investigated the impact of RFCA on the long-term risk of coronary events and mortality in WPW patients. Methods and results: We conducted a prospective cohort study utilizing the Taiwan National Health Insurance Research Database. Between 2000 and 2003, WPW patients with no prior coronary artery disease (CAD) history, aged over 18 years, who underwent RFCA were identified. WPW patients without RFCA were matched with propensity-score 1:4 matching for confounding coronary risk factors. The study outcomes were total mortality and coronary events. A total of 1524 matched non-ablated WPW patients (Group 1) and 381 ablated WPW patients (Group 2) were included. After a mean follow-up of 9.6 ± 2.9 and 10.3 ± 1.9 years, respectively, ablation group demonstrated a lower incidence of mortality compared with non-ablation group (17 vs. 26/1000 person-years, P < 0.001; adjusted HR: 0.57, 95% CI: 0.44-0.7). However, ablation group had a higher incidence of coronary events compared with non-ablation group (47 vs. 82/1000 person-years, P < 0.001; adjusted HR: 1.69, 95% CI: 1.4-2.04). Conclusion: The ablation-treated WPW patients had lower risk of total mortality but higher risk of coronary events than non-ablated WPW patients during the long-term follow-up. Coronary artery injury produced by RFCA may account for the increased risk of coronary events. Therefore, the ablation strategies to avoid coronary artery injury should be implemented.

Intermittent versus Persistent Wolff-Parkinson-White Syndrome in Children: Electrophysiologic Properties and Clinical Outcomes.

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

Long-Term Natural History of Adult Wolff-Parkinson-White Syndrome Patients Treated With and Without Catheter Ablation.

BACKGROUND: There are a paucity of data about the long-term natural history of adult Wolff-Parkinson-White syndrome (WPW) patients in regard to risk of mortality and atrial fibrillation. We sought to describe the long-term outcomes of WPW patients and ascertain the impact of ablation on the natural history. METHODS AND RESULTS: Three groups of patients were studied: 2 WPW populations (ablation: 872, no ablation: 1461) and a 1:5 control population (n=11 175). Long-term mortality and atrial fibrillation rates were determined. The average follow-up for the WPW group was 7.9±5.9 (median: 6.9) years and was similar between the ablation and nonablation groups. Death rates were similar between the WPW group versus the control group (hazard ratio, 0.96; 95% confidence interval, 0.83-1.11; P=0.56). Nonablated WPW patients had a higher long-term death risk compared with ablated WPW patients (hazard ratio, 2.10; 95% confidence interval: 1.50-20.93; P<0.0001). Incident atrial fibrillation risk was higher in the WPW group compared with the control population (hazard ratio, 1.55; 95% confidence interval, 1.29-1.87; P<0.0001). Nonablated WPW patients had lower risk than ablated patients (hazard ratio, 0.39; 95% confidence interval, 0.28-0.53; P<0.0001). CONCLUSIONS: Long-term mortality rates in WPW patients are low and similar to an age-matched and gender-matched control population. WPW patients that underwent the multifactorial process of ablation had a lower mortality compared to nonablated WPW patients. Atrial fibrillation rates are high long-term, and ablation does not reduce this risk.

Ventricular pre-excitation: symptomatic and asymptomatic children have the same potential risk of sudden cardiac death.

AIMS: Children and adolescents with ventricular pre-excitation (VPE) are at increased risk for sudden cardiac death (SCD). Although antiarrhythmic therapy and catheter ablation are well established temporary or definitive treatments for patients with Wolff-Parkinson-White (WPW) syndrome, the optimal management of children with asymptomatic VPE remains to be clearly defined. On the basis of the most recent guidelines and recommendations, the aim of this study was to determine the electrophysiological characteristics of young patients with VPE and WPW syndrome to assess and compare their potential risk of SCD. METHODS AND RESULTS: We retrospectively investigated 124 consecutive young patients with VPE (51 with WPW syndrome and 73 asymptomatic) who underwent transoesophageal electrophysiological study. At baseline, atrioventricular reentrant tachycardia (AVRT) was induced in 13 WPW vs. 10 asymptomatic patients (25.5 vs. 13.7%, P = NS). Atrial fibrillation (AF) was induced in 13 WPW vs. 15 asymptomatic patients (25.5 vs. 20.5%, P = NS). A shortest pre-excited R-R interval (SPERRI) ≤250 ms during AF was found in four WPW vs. six asymptomatic patients (30.8 vs. 40%, P = NS). During isoproterenol infusion or stress testing, AVRT was induced in 31 of 44 WPW vs. 33 of 69 asymptomatic patients (70.4 vs. 47.8%, P = 0.018). Atrial fibrillation was induced in 12 of 44 WPW vs. 21 of 69 asymptomatic patients (27.3 vs. 30.4%, P = NS). A SPERRI ≤ 210 ms was found in 6 of 12 WPW vs. 10 of 21 asymptomatic patients (50 vs. 47.6%, P = NS). No statistically significant correlation was observed between accessory pathway location and symptoms, AVRT/AF inducibility, or mean APERP/SPERRI values. CONCLUSION: Children and adolescents with WPW syndrome have a higher rate of AVRT inducibility than asymptomatic patients. However, no differences between the two groups were found in atrial vulnerability and parameters related to the risk of SCD.

Radiofrequency ablation of accessory pathways in patients with the Wolff-Parkinson-White syndrome: the long-term mortality and risk of atrial fibrillation.

AIMS: To assess the long-term mortality and occurrence of post-ablation atrial fibrillation in patients undergoing a radiofrequency ablation for the Wolff-Parkinson-White (WPW) syndrome. METHODS AND RESULTS: A retrospective cohort study of patients (N = 362) subjected to radiofrequency ablation of the WPW syndrome at Aarhus University Hospital from 1990 to 2011. A comparison cohort (N = 3619) was generated from the Danish National Board of Health Central Population Registry. We found no significant difference in all-cause mortality when comparing the WPW group with the control group [hazard ratio (HR): 0.77 and confidence interval (CI): 0.47-1.25]. After radiofrequency ablation, the WPW group had a significantly higher risk of atrial fibrillation than the control group (HR: 4.77 and CI: 3.05-7.43). Atrial fibrillation prior to ablation (HR: 4.66 and CI: 2.09-10.41) and age over 50 years (HR: 9.79 and CI: 4.29-22.36) at the time of ablation were independent risk factors for post-ablation atrial fibrillation in the WPW group. CONCLUSION: Patients with radiofrequency ablation-treated WPW syndrome have a post-ablation mortality that is similar to the background population. The risk of atrial fibrillation remains high after radiofrequency ablation of the WPW syndrome.

Wolff-Parkinson-White syndrome in the era of catheter ablation: insights from a registry study of 2169 patients.

BACKGROUND: The management of Wolff-Parkinson-White is based on the distinction between asymptomatic and symptomatic presentations, but evidence is limited in the asymptomatic population. METHODS AND RESULTS: The Wolff-Parkinson-White registry was an 8-year prospective study of either symptomatic or asymptomatic Wolff-Parkinson-White patients referred to our Arrhythmology Department for evaluation or ablation. Inclusion criteria were a baseline electrophysiological testing with or without radiofrequency catheter ablation (RFA). Primary end points were the percentage of patients who experienced ventricular fibrillation (VF) or potentially malignant arrhythmias and risk factors. Among 2169 enrolled patients, 1001 (550 asymptomatic) did not undergo RFA (no-RFA group) and 1168 (206 asymptomatic) underwent ablation (RFA group). There were no differences in clinical and electrophysiological characteristics between the 2 groups except for symptoms. In the no-RFA group, VF occurred in 1.5% of patients, virtually exclusively (13 of 15) in children (median age, 11 years), and was associated with a short accessory pathway antegrade refractory period (P<0.001) and atrioventricular reentrant tachycardia initiating atrial fibrillation (P<0.001) but not symptoms. In the RFA group, ablation was successful in 98.5%, and after RFA, no patients developed malignant arrhythmias or VF over the 8-year follow-up. Untreated patients were more likely to experience malignant arrhythmias and VF (log-rank P<0.001). Time-dependent receiver-operating characteristic curves for predicting VF identified an optimal anterograde effective refractory period of the accessory pathway cutoff of 240 milliseconds. CONCLUSIONS: The prognosis of the Wolff-Parkinson-White syndrome essentially depends on intrinsic electrophysiological properties of AP rather than on symptoms. RFA performed during the same procedure after electrophysiological testing is of benefit in improving the long-term outcomes.

Noninvasive risk stratification for sudden death in asymptomatic patients with Wolff-Parkinson-White syndrome.

Sudden cardiac death (SCD) as the first clinical manifestation of Wolff-Parkinson-White (WPW) syndrome is a well-documented, although rare occurrence. The incidence of SCD in patients with WPW ranges from 0% to 0.39% annually. Controversy exists regarding risk stratification for patients with preexcitation on surface electrocardiogram (ECG), particularly in those who are asymptomatic. This article focuses on the role of risk stratification using exercise and pharmacologic testing in patients with WPW pattern on ECG.

Natural history of Wolff-Parkinson-White syndrome diagnosed in childhood.

Wolff-Parkinson-White (WPW) syndrome carries a risk for symptomatic arrhythmias and sudden death. The aim of this study was to examine the natural history of patients with Wolff-Parkinson-White syndrome diagnosed in childhood followed longitudinally at a single institution. The study population consisted of 446 patients. The median age of diagnosis was 7 years, and 61% were male. Associated heart disease was present in 40 patients (9%). Modes of presentation included supraventricular tachycardia (38%), palpitations (22%), chest pain (5%), syncope (4%), atrial fibrillation (0.4%), sudden death (0.2%), and incidental findings (26%); data were unavailable in 4%. During the study period, a total of 243 patients (54%) had supraventricular tachycardia, and 7 patients (1.6%) had atrial fibrillation. Of patients who presented at ≤3 months of age, 35% had resolution of manifest preexcitation compared with 5.8% who presented at >3 months of age (p <0.0001). There were 6 sudden deaths (1.3%), with an incidence of 2.8 per 1,000 patient-years. Two of these patients had structurally normal hearts (incidence 1.1 per 1,000 patient-years). Four of these patients had associated heart disease (incidence 27 per 1,000 patient-years) (p <0.01). In conclusion, in a large population of patients with Wolff-Parkinson-White syndrome diagnosed in childhood, 64% had symptoms at presentation, and an additional 20% developed symptoms during follow-up. There were 6 sudden deaths (1.3%), with an overall incidence of 1.1 per 1,000 patient-years in patients with structurally normal hearts and 27 per 1,000 patient-years in patients with associated heart disease.

The electrophysiological characteristics of accessory pathways in pediatric patients with intermittent preexcitation.

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

Prophylactic radiofrequency ablation in asymptomatic patients with Wolff-Parkinson-White is not yet a good strategy: a decision analysis.

BACKGROUND: Therapeutic management of asymptomatic patients with a Wolff-Parkinson-White (WPW) pattern is controversial. We compared the risk:benefit ratios between prophylactic radiofrequency ablation and no treatment in asymptomatic patients with WPW. METHODS AND RESULTS: Decision analysis software was used to construct a risk-benefit decision tree. The target population consisted of 20- to 40-year-old asymptomatic patients with WPW without structural fatal heart disease or a family history of sudden cardiac death. Baseline estimates of sudden death and radiofrequency ablation complication rates were obtained from the literature, an empirical data survey, and expert opinion. The outcome measure was death within 10 years. Sensitivity analyses determined the variables that significantly impacted the decision to ablate or not. Threshold analyses evaluated the effects of key variables and the optimum policy. At baseline, the decision to ablate resulted in a reduction of mortality risk of 8.8 patients for 1000 patients compared with abstention. It is necessary to treat 112 asymptomatic patients with WPW to save one life over 10 years. Sensitivity analysis showed that 3 variables significantly impacted the decision to ablate: (1) complication of radiofrequency ablation, (2) success of radiofrequency ablation, and (3) sudden death in asymptomatic patients with WPW. CONCLUSIONS: This study provides a decision aid for treating asymptomatic patients with the WPW ECG pattern. Using the model and the population we tested, prophylactic catheter ablation is not yet ready for widespread clinical use.

[Asymptomatic ventricular pre-excitation in children: a 17 year follow-up study].

Results of long term clinic-electrophysiological follow-up of a large group of children with Wolf-Parkinson-White (WPW) phenomenon are present. From 1993 to 2011 we examined 176 children - 117 boys (66,5%) and 59 girls (33,5%) - with manifesting type of WPW. Age at first examination was 12.5+/-3.2 ( 0.1 18 ). In 66.5% of children WPW phenomenon was present in the age from 10 to 18 . Hundred fifty nine children (104 [65.4%] boys and 55 [34.6%] girls) were followed up for 0.5-17 years (mean 7.4+/-4.5 years). During follow-up spontaneous attacs of atrioventricular reciprocal tachycardia appeared in 13 children (8.2%, 95% confidence interval [CI] 4.4-13.6%). Spontaneous disappearance of signs of ventricular pre-excitation on electrocardiogram and transition to intermittent form of WPW were registered in 14(8.8%, 95%CI 4.9-14.3%) and 12 children (7.5%, 95% CI 3.9-12.8%). Short lasting syncopal states were observed in 15 children (8.4%). In 3 of them atrial fibrillation with high rate of venricular rhytm was induced during transesophageal electrophysiological study. Two (1.3%) children had history of clinical death. In one of them WPW phenomenon was combined with hypertrophic cardiomyopathy and polymorphic ventricular tachycardia. This child died at the age of 11 years. One (0.6%) boy with WPW phenomenon died suddenly in 3 years after initial examination. Thus, children with WPW phenomenon are at risk of development of life threatening states and sudden cardiac death. Stratification of risk of sudden death is necessary at examination of such children.

Risk of arrhythmia and sudden death in patients with asymptomatic preexcitation: a meta-analysis.

BACKGROUND: The incidence of sudden cardiac death (SCD) and the management of this risk in patients with asymptomatic preexcitation remain controversial. The purpose of this meta-analysis was to define the incidence of SCD and supraventricular tachycardia in patients with asymptomatic Wolff-Parkinson-White ECG pattern. METHODS AND RESULTS: We performed a systematic search of prospective, retrospective, randomized, or cohort English-language studies in EMBASE and Medline through February 2011. Studies reporting asymptomatic patients with preexcitation who did not undergo ablation were included. Twenty studies involving 1869 patients met our inclusion criteria. Participants were primarily male with a mean age ranging from 7 to 43 years. Ten SCDs were reported involving 11 722 person-years of follow-up. Seven studies originated from Italy and reported 9 SCDs. The risk of SCD is estimated at 1.25 per 1000 person-years (95% confidence interval [CI], 0.57-2.19). A total of 156 supraventricular tachycardias were reported involving 9884 person-years from 18 studies. The risk of supraventricular tachycardia was 16 (95% CI, 10-24) events per 1000 person-years of follow-up. Children had numerically higher SCD (1.93 [95% CI, 0.57-4.1] versus 0.86 [95% CI, 0.28-1.75]; P=0.07) and supraventricular tachycardia (20 [95% CI, 12-31] versus 14 [95% CI, 6-25]; P=0.38) event rates compared with adults. CONCLUSION: The low incidence of SCD and low risk of supraventricular tachycardia argue against routine invasive management in most asymptomatic patients with the Wolff-Parkinson-White ECG pattern.

Behavior of Ebstein's anomaly: single-center experience and midterm follow-up.

OBJECTIVES: Ebstein's anomaly, characterized by an apical displacement of the tricuspid valve into the right ventricle, occurs in approximately 1/200,000 live births. Because long-term follow-up data of adults with Ebstein's anomaly are scarce, we evaluated the outcome of our Ebstein's anomaly patients. METHODS: All patients >16 years and registered in the congenital heart disease database of our hospital with isolated Ebstein's anomaly were selected for the study. Records were reviewed for outcome. RESULTS: Forty-nine patients (21 males, mean age at diagnosis 29.1 ± 20.7 years) were followed for a mean time of 11.4 years (range 1.1-32.4). Twenty-five patients (51%) underwent tricuspid valve surgery (16 valvuloplasty and 9 valve replacement). Eight patients (32%) required redo tricuspid valve surgery. Twenty-six patients (52.1%) exhibited supraventricular arrhythmia, and the typical Wolff-Parkinson-White syndrome occurred in 15 patients (31.2%). Seventeen patients (34.7%) underwent ablation therapy and 5 patients (10.4%) required pacemaker implantation. CONCLUSIONS: Half of the patients with Ebstein's anomaly needed tricuspid valve surgery and redo surgery was not uncommon. Supraventricular arrhythmia occurred frequently and ablation therapy was often indicated. Careful follow-up is obligate, as some complications occur for the first time in adulthood.

Asymptomatic ventricular preexcitation: a long-term prospective follow-up study of 293 adult patients.

BACKGROUND: Sudden cardiac death can be the first clinical presentation of asymptomatic ventricular preexcitation. METHODS AND RESULTS: From 1995 to 2005, we prospectively collected clinical and electrophysiological data among 293 adults with asymptomatic ventricular preexcitation (61.4% males; median age, 36 years; interquartile range [IQR], 28 to 47.5). After electrophysiological testing, patients were prospectively followed, taking no drugs. The primary end point of the study was the occurrence of a first arrhythmic event. Predictors of arrhythmic events were analyzed by univariate and multivariate Cox models. Over a median follow-up of 67 months (minimum to maximum, 8 to 90), after electrophysiological testing, 262 patients (median age, 37 years; IQR, 30 to 48) did not experience arrhythmic events, remaining totally asymptomatic, whereas 31 patients (median age, 25 years; IQR, 22 to 29; median follow-up, 27 months; minimum to maximum, 8 to 55) had a first arrhythmic event, which was potentially life-threatening in 17 of them (median age, 24 years; IQR, 20 to 28.5; median follow-up, 25 months; minimum to maximum, 9 to 55). Potentially life-threatening tachyarrhythmias resulted in resuscitated cardiac arrest (1 patient), presyncope (7 patients) syncope (4 patients), or dizziness (5 patients). In multivariate analysis age (P=0.004), inducibility (P=0.001) and anterograde effective refractory period of the accessory pathway < or = 250 ms (P=0.001) predicted potentially life-threatening arrhythmias. CONCLUSIONS: These results indicate that prognosis of adults who present with asymptomatic ventricular preexcitation is good, and the risk of a significant event is small. Short anterograde effective refractory period of the accessory pathway and inducibility at baseline are independent predictors of potentially life-threatening arrhythmic events, and the risk decreases with increasing age.