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 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.

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.

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.

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.

[Electrophysiological properties of asymptomatic children and adolescents with the Wolff-Parkinson-White electrocardiographic pattern]. / Wolff-Parkinson-White elektrokardiyografik paterni olan asemptomatik çocuk ve ergenlerin elektrofizyolojik özellikleri.

OBJECTIVE: Optimal management in asymptomatic children and adolescents with the Wolff-Parkinson-White (WPW) electrocardiographic pattern is still debatable. The aim of this retrospective study was to evaluate the electrophysiological properties of asymptomatic children and adolescents with WPW. METHODS: The medical records and procedural data of asymptomatic children and adolescents with the WPW electrocardiographic pattern who underwent invasive electrophysiological study (EPS) and catheter ablation between April 2012 and April 2018 were evaluated. RESULTS: In all, 149 consecutive, asymptomatic children and adolescents with WPW who underwent EPS were retrospectively investigated. In 39 (26.2%) of the patients, a shortest pre-excited R-R interval of ≤250 ms during atrial fibrillation or an accessory pathway effective refractory period of ≤270 ms was found. A total of 39 patients with high-risk antegrade conduction and 45 patients with inducible orthodromic atrioventricular re-entrant tachycardia underwent catheter ablation. In addition, 14 patients underwent catheter ablation due to family preference. CONCLUSION: High-risk antegrade conduction properties are exhibited by more than one-quarter of asymptomatic children and adolescents with WPW. Ablation should be considered as a first-line therapy in asymptomatic children and adolescents with high-risk WPW.

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.

Localization Of Accessory Pathways In Wolff Parkinson White Syndrome Using R/S Ratios On Surface Ecgs.

BACKGROUND: This study was conducted to establish the accuracy of R/S ratios in localizing accessory pathways in Wolff Parkinson White Syndrome on surface ECGs. METHODS: This was a retrospective cross-sectional study from January 2002 till December 2016 conducted at National Institute of Cardiovascular diseases in Karachi, Pakistan. The sample included 157 patients with manifesting Wolff Parkinson White (WPW) Syndrome on a 12-lead surface ECG. As per the inclusion criteria, patients who had persistent or intermittent pre-excitation on surface ECG and had undergone electrophysiological study (EP) were included in the sample. Individuals with both successful and unsuccessful ablation procedures were included. RESULTS: The sample consisted of 62.4% males (n=98) and 37.6% females (n=59) with mean age being 35.36±12.44. Accessory pathways (APs) were identified on the left side in majority of the patients with 54.1% (n=85) while right sided pathways were seen in 42.1% (n=66). Moreover, the most common accessory pathway amongst the males was Left Anterior (LA) and Left Lateral (LL) with a percentage of 48%, followed by Right Posteroseptal (RPS) at 20.4%. As opposed to this, the most common pathways amongst the females turned out to be RPS at 33.9% followed by LL and LA at 32.2%. Furthermore, the test using ratios were most effective in identifying left sided APs with sensitivity 74.1% and was least sensitive in identifying right sided pathways (sensitivity=40.7%). However, it was highly specific in locating right sided pathways with specificity of 94.6% as opposed to 83.3% in the left side. Furthermore, mid-septum regions had a sensitivity and specificity of 66.7% and 69.6% respectively. CONCLUSIONS: This establishes the effectiveness of R/S ratios in determining the location of accessory pathway based on the surface ECG prior to the ablation procedure. Since the younger population is affected more commonly, prolonged exposure to ionizing radiation can cause long term complications therefore, by this means, the duration of exposure can be reduced.

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.

Clinical Findings and Prognosis of Danon Disease. An Analysis of the Spanish Multicenter Danon Registry.

INTRODUCTION AND OBJECTIVES: Danon disease (DD) is caused by mutations in the LAMP2 gene. It is considered a multisystemic disease characterized by hypertrophic cardiomyopathy with pre-excitation and extreme hypertrophy, intellectual disability, myopathy, childhood presentation, and worse prognosis in men. There are scarce data on the clinical characteristics and prognosis of DD. METHODS: We analyzed the clinical records of patients with DD from 10 Spanish hospitals. RESULTS: Twenty-seven patients were included (mean age, 31 ± 19 years; 78% women). Male patients showed a high prevalence of extracardiac manifestations: myopathy (80%), learning disorders (83%), and visual alterations (60%), which were uncommon findings in women (5%, 0%, and 27%, respectively). Although hypertrophic cardiomyopathy was the most common form of heart disease (61%), the mean maximum wall thickness was 15 ± 7 mm and dilated cardiomyopathy was present in 12 patients (10 women). Pre-excitation was found in only 11 patients (49%). Age at presentation was older than 20 years in 16 patients (65%). After a median follow-up of 4 years (interquartile range, 2-9), 4 men (67%) and 9 women (43%) died or required a transplant. Cardiac disease and adverse events occurred later in women (37 ± 9 vs 23 ± 16 and 36 ± 20 vs 20 ± 11 years, respectively). CONCLUSIONS: The clinical characteristics of DD differ substantially from traditional descriptions: age at presentation of DD is older, the disease is not multisystemic in women, and pre-excitation is infrequent.

Prognostic Significance and Risk of Atrial Fibrillation of Wolff-Parkinson-White Syndrome in Patients With Hypertrophic Cardiomyopathy.

To assess the mid-term mortality and risk of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy (HC) and Wolff-Parkinson-White (WPW) syndrome, 40 patients with HC and WPW were enrolled in our center between 2010 and 2017. An age- and gender-matched comparison cohort of patients with HC without WPW (n = 160) was generated from the same center. The clinical profile and outcomes were assessed. Of 40 patients with WPW, 28 underwent accessory pathway (AP) elimination. Two patients (7%) had failed in AP elimination. During mid-term follow-up, 1 patient had an implantable cardioverter-defibrillator intervention. Fourteen patients had AF. A previous history of AF (hazard ratio [HR]: 4.69; 95% confidence interval [CI] 1.51 to 14.63) and left atrial dimension (HR: 1.12; 95% CI 1.03 to 1.23) at baseline were risk factors for AF occurrence during follow-up. The AP elimination significantly reduced risk for the incidence of AF (HR: 0.22; 95% CI 0.06 to 0.83). Compared with the control group, the prevalence of syncope and AF were significantly higher in the WPW group. During follow-up, no difference was identified in outcome measures consisting of all-cause death, cardiac transplantation, and implantable cardioverter-defibrillator intervention. A previous history of AF (HR: 5.20; 95% CI 2.63 to 10.30, p <0.001) and persistent existing WPW (HR: 3.64; 95% CI 1.63 to 8.11, p = 0.002) were independent risk factors for AF occurrence during follow-up in the entire cohort. In conclusion, although WPW was uncommon and might not be correlated with mid-term mortality in HC patients, WPW might increase the risk of AF occurrence. Additionally, AP elimination may reduce the risk of AF occurrence.

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.

[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.

Identification of clinically relevant phenotypes in patients with Ebstein anomaly.

BACKGROUND: Ebstein anomaly (EA) is a heterogeneous congenital heart defect (CHD), frequently accompanied by diverse cardiac and extracardiac comorbidities, resulting in a wide range of clinical outcomes. HYPOTHESIS: Phenotypic characterization of EA patients has the potential to identify variables that influence prognosis and subgroups with distinct contributing factors. METHODS: A comprehensive cross-sectional phenotypic characterization of 147 EA patients from one of the main referral institutions for CHD in Colombia was carried out. The most prevalent comorbidities and distinct subgroups within the patient cohort were identified through cluster analysis. RESULTS: The most prevalent cardiac comorbidities identified were atrial septal defect (61%), Wolff-Parkinson-White syndrome (WPW; 27%), and right ventricular outflow tract obstruction (25%). Cluster analysis showed that patients can be classified into 2 distinct subgroups with defined phenotypes that determine disease severity and survival. Patients in cluster 1 represented a particularly homogeneous subgroup with a milder spectrum of disease, including only patients with WPW and/or supraventricular tachycardia (SVT). Cluster 2 included patients with more diverse cardiovascular comorbidities. CONCLUSIONS: This study represents one of the largest phenotypic characterizations of EA patients reported. The data show that EA is a heterogeneous disease, very frequently associated with cardiovascular and noncardiovascular comorbidities. Patients with WPW and SVT represent a homogeneous subgroup that presents with a less severe spectrum of disease and better survival when adequately managed. This should be considered when searching for genetic causes of EA and in the clinical setting.

Asymptomatic Wolff-Parkinson-White Pattern ECG in USAF Aviators.

INTRODUCTION: Wolff-Parkinson-White (WPW) pattern is occasionally found in asymptomatic aviators during routine ECGs. Aeromedical concerns regarding WPW pattern include risk of dysrhythmia or sudden cardiac death (SCD), thus affecting the safety of flight. The purpose of this study was to determine the prevalence and outcomes of aviators with asymptomatic WPW pattern and assess for risk factors that contribute to progression to dysrhythmia or symptoms. METHODS: The U.S. Air Force (USAF) ECG library database containing over 1.2 million ECGs collected over the past 68 yr was used to identify 638 individual aviators with WPW pattern. Demographic, medical history, and outcome data were obtained by medical record review. Aviators who developed high risk features defined as symptoms, arrhythmia, or ablation of a high risk pathway, were compared to those who remained asymptomatic. RESULTS: Prevalence of WPW pattern was 0.30% among all USAF aviators. Of the 638 individuals, 64 (10%) progressed to the combined endpoint of SCD, arrhythmia, and/or ablation of a high risk pathway over 6868 patient years, with average follow-up of 10.5 yr. There were two sudden cardiac deaths (0.3%). Annual risk of possible sudden incapacitation was 0.95% and of SCD 0.03%. Those that progressed to high risk were significantly younger, had lower diastolic blood pressure, lower total cholesterol, and better physical fitness testing scores. DISCUSSION: WPW pattern on ECG found in asymptomatic aviators confers < 1% annual risk of arrhythmia or incapacitating events with the highest risk in the younger, healthier, and most fit populations.Davenport ED, Rupp KAN, Palileo E, Haynes J. Asymptomatic Wolff-Parkinson-White pattern ECG in USAF aviators. Aerosp Med Hum Perform. 2017; 88(1):56-60.

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.

Demographics and Epidemiology of Sudden Deaths in Young Competitive Athletes: From the United States National Registry.

BACKGROUND: Sudden deaths in young competitive athletes are tragic events, with high public visibility. The importance of race and gender with respect to sport and the diagnosis and causes of sudden death in athletes has generated substantial interest. METHODS: The US National Registry of Sudden Death in Athletes, 1980-2011, was accessed to define the epidemiology and causes of sudden deaths in competitive athletes. A total of 2406 deaths were identified in young athletes aged 19 ± 6 years engaged in 29 diverse sports. RESULTS: Among the 842 athletes with autopsy-confirmed cardiovascular diagnoses, the incidence in males exceeded that in females by 6.5-fold (1:121; 691 vs 1:787,392 athlete-years; P ≤.001). Hypertrophic cardiomyopathy was the single most common cause of sudden death, occurring in 302 of 842 athletes (36%) and accounting for 39% of male sudden deaths, almost 4-fold more common than among females (11%; P ≤.001). More frequent among females were congenital coronary artery anomalies (33% vs 17% of males; P ≤.001), arrhythmogenic right ventricular cardiomyopathy (13% vs 4%; P = .002), and clinically diagnosed long QT syndrome (7% vs 1.5%; P ≤.002). The cardiovascular death rate among African Americans/other minorities exceeded whites by almost 5-fold (1:12,778 vs 1:60; 746 athlete-years; P <.001), and hypertrophic cardiomyopathy was more common among African Americans/other minorities (42%) than in whites (31%; P ≤.001). Male and female basketball players were 3-fold more likely to be African American/other minorities than white. CONCLUSIONS: Within this large forensic registry of competitive athletes, cardiovascular sudden deaths due to genetic and/or congenital heart diseases were uncommon in females and more common in African Americans/other minorities than in whites. Hypertrophic cardiomyopathy is an under-appreciated cause of sudden death in male minority athletes.

Electrophysiology testing and catheter ablation are helpful when evaluating asymptomatic patients with Wolff-Parkinson-White pattern: the con perspective.

The association between asymptomatic Wolff-Parkinson-White (WPW) syndrome and sudden cardiac death (SCD) has been well documented. The inherent properties of the accessory pathway determine the risk of SCD in WPW, and catheter ablation essentially eliminates this risk. An approach to WPW syndrome is needed that incorporates the patient's individualized considerations into the decision making. Patients must understand that there is a trade-off of a small immediate risk of an invasive approach for elimination of a small lifetime risk of the natural history of asymptomatic WPW. Clinicians can minimize the invasive risk by only performing ablation for patients with at-risk pathways.