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Purpose Patients with WPW syndrome have an increased mortality rate compared to the general population. Although asymptomatic preexcitation has previously been considered benign, recent studies have found that also asymptomatic patients have clinical and electrophysiological factors associated with increased risk of sudden cardiac death. This study compares the baseline electrophysiological characteristics of accessory pathways in symptomatic and asymptomatic patients with preexcitation. We hypothesized that a significant proportion of asymptomatic patients has inducible orthodromic tachycardia during programmed electrical stimulation. Methods This retrospective study includes 1853 patients with preexcitation who underwent invasive electrophysiological testing in two Swedish University Hospitals between 1991 and 2018. The mean age was 36 ± 17 years with a range of 3–89 years. Thirty-nine percent was women. A total of 269 patients (15%) were children younger than 18 years. Electrophysiological data included effective refractory period of the accessory pathway (APERP, in 1069 patients), tachycardia cycle length, inducibility and type of tachycardia, and AP localization. Results A total of 1703 (93%) patients reported symptoms suggesting tachyarrhythmias before the study and 128 (7%) were asymptomatic. The proportion of potentially dangerous pathways with short APERP (≤ 250 ms) were similar in symptomatic and asymptomatic patients (187/949, 20% vs. 25/108, 23%) (P = 0.40) as was the mean APERP (303 ± 68 ms vs. 307 ± 75) (P = 0.61). The proportion of patients who had inducible arrhythmia was larger in the symptomatic group (64% vs. 31%) (P Conclusion The results of this study strengthen the present guideline recommendation (IIA) to consider invasive risk assessment in patients with asymptomatic preexcitation.

Altered ventricular depolarization due to manifest accessory pathway conduction (ie, Wolff-Parkinson-White syndrome) leads to repolarization abnormalities that persist after pathway ablation. The term T-wave memory (TWM) has been applied to these changes, as the postablation T-wave vector "remembers" the pre-excited QRS vector. In adults, these abnormalities can be misinterpreted as ischemia leading to unnecessary interventions. To date, no comprehensive studies have evaluated this phenomenon in the pediatric population.The purpose of this study was to define TWM in the pediatric population, identify preablation risk factors, and delineate the timeline of recovery.Pre- and postablation electrocardiograms (ECGs) in patients ≤25 years were analyzed over a 5-year period. Frontal plane QTc interval, T-wave axis, QRST angle, and T-wave inversions were used to identify patients with TWM. Univariate analysis was performed to determine the association of preablation ECG features with the outcome of TWM.TWM was present in 42% of pediatric patients, with resolution occurring within 3 months of ablation. Preablation QRS axis0° was a strong predictor of TWM (odds ratio [OR] 15.2; 95% confidence interval [CI] 5.7-40), followed by posteroseptal pathway location (right posteroseptal-OR 8.9; 95% CI 4.2-18.8; left posteroseptal-OR 6.1; 95% CI 1.7-22.3). The degree of pre-excitation had a modest association with the development of TWM. No adverse events were observed.TWM is less common in children compared to adults, and normalization occurred within 3 months postablation. The most predictive features for the development of TWM include a leftward pre-excited QRS axis and posteroseptal pathway location.

Background: In contrast to adults, in whom cardiac rhythm disorders are mainly conditioned by coronary artery disease, in children, arrhythmias are most often associated with inherited heart disorders. Catheter ablation (CA) has an important role in the management of cardiac arrhythmias, in adults and children. The aim of the study was to assess and compare the efficacy and safety of CA in children and adults with preexcitation syndrome. Methods: The study population comprised 43 adults and 43 children diagnosed with a Wolff-Parkinson-White syndrome (WPW). The mean age of the study population was 41 ± 15 years for adults and 14 ± 2.5 years for children. In all patients, an electrophysiological study and CA were performed. Analysis with respect to the procedure duration, fluoroscopy exposure time, location of accessory pathways (AP), immediate success rate and complications were performed. Results: Electrophysiological study revealed the most frequent presence of left-sided AP (56% in children and 70% in adults). The mean procedure duration was 96 ± 36 min and 106 ± 51 min in children and adults, respectively (p = NS). The mean fluoroscopy duration was 8.5 ± 4.3 min and 5.9 ± 5.8 min in children and adults, respectively p < 0.05. The CA procedure was successful in 40 out of 43 (93%) adults and in 36 out of 43 (83.7%) children (p = NS). In 2 (4%) children minor complications occurred. Conclusions: Ablation in children and adults are equally effective with respect to short-term clinical observation.

Accessory pathway potential often indicates a highly effective ablation target in Wolff-Parkinson-White syndrome.A 27-year-old female presenting with palpitation underwent an electrophysiology study, who had mild pre-excitation in surface ECG.An accessory pathway with weak anterograde conduction was found. During isoproterenol infusion, the delta wave became prominent, an antidromic AV reentrant tachycardia was then induced. When the pathway was mapped, widely split double pathway potentials were observed at the 12 o'clock site of the tricuspid annulus during mild pre-excitation, demonstrating an example of intra-pathway conduction delay. Ablation at the site caused accelerated pathway rhythm and eliminated the pathway, rendering the tachycardia noninducible.Split pathway potentials can reflect slow conduction in patients with preexcitation.

Danon disease is typically associated with cardiomyopathy and ventricular pre-excitation. The study aimed to characterize the clinical profile of Danon disease, analyze electrocardiographic (ECG) and electrophysiologic features, and investigate their association with Wolff-Parkinson-White (WPW) syndrome and fasciculoventricular pathways (FVPs).Methods and Results:Clinical course, family history, ECG and electrophysiological data were collected from 16 patients with Danon disease. Over 0.4-8 years of follow up, 1 female patient died suddenly, and 5 male patients died of progressive heart failure by age 13-20 years. Family history analysis revealed that 3 mothers experienced hospitalization or death for heart failure at age 28-41 years. There was 100% penetrance for ECG abnormalities in 13 patients with original ECGs. Short PR intervals and delta waves were present in 9 and 8 patients, respectively. There were significant age-associated increases in the QRS complex width (r=0.556, P=0.048) and the number of leads with notched QRS (r=0.575, P=0.04). Four patients who underwent electrophysiological studies all had FVPs, and 2 of them still had left-side atrioventricular pathways.Danon disease causes a malignant clinical course characterized by early death caused by heart failure in both genders and progressive ECG changes as patients age. The pre-excited ECG pattern is related to FVPs and WPW, which is suggestive of extensive cardiac involvement.

PURPOSE The correct estimation of accessory pathway (AP) localization from surface ECG is critical before the procedure. Our study aimed to detect the predictive value of the V1r + DIIq criterion for differentiating right- from left-sided paraseptal APs. METHODS We retrospectively included 58 patients with (Wolff-Parkinson-White) WPW syndrome and paraseptal APs who underwent successful catheter ablation (37 male, 21 female; mean age 34.4 ± 13.6 years). The V1r + DIIq criterion was calculated using the following formula: V1r + DIIq (mV) = initial r wave amplitude in V1 + q wave amplitude in DII. The combined criterion included V1r + DIIq

Patients with the Wolff-Parkinson-White syndrome may experience benign and malignant arrhythmias, the most common being atrioventricular reentrant tachycardias. This arrhythmia may degenerate into atrial fibrillation, which can be conducted over an accessory pathway capable of exceptionally fast conduction to the ventricles and degenerate into ventricular fibrillation, leading to sudden cardiac death. These life-threatening events generally affect symptomatic patients in their third or fourth decade. Although rare, ventricular fibrillation may be the first clinical manifestation in subjects who are asymptomatic or unaware of their conditions. Electrophysiologic study may be useful to identify subjects at high risk of sudden cardiac death.

Background Although successful ablation of the accessory pathway (AP) eliminates atrial fibrillation (AF) in some of patients with Wolff‐Parkinson‐White (WPW) syndrome and paroxysmal AF, in other patients it can recur. Hypothesis Whether adding pulmonary vein isolation (PVI) after successful AP ablation effectively prevents AF recurrence in patients with WPW syndrome is unknown. Methods We retrospectively studied 160 patients (102 men, 58 women; mean age, 46 ± 14 years) with WPW syndrome and paroxysmal AF who underwent AP ablation, namely 103 (64.4%) undergoing only AP ablation (AP group) and 57 (35.6%) undergoing AP ablation plus PVI (AP + PVI group). Advanced interatrial block (IAB) was defined as a P‐wave duration of >120 ms and biphasic (±) morphology in the inferior leads, using 12‐lead electrocardiography (ECG). Results During the mean follow‐up period of 30.9 ± 9.2 months (range, 3‐36 months), 22 patients (13.8%) developed AF recurrence. The recurrence rate did not differ in patients in the AP + PVI group and AP group (15.5% vs 10.5%, respectively; P = .373). Univariable and multivariable Cox regression analyses showed that PVI was not associated with the risk of AF recurrence (hazard ratio, 0.66; 95% confidence interval, 0.26‐1.68; P = .380). In WPW patients with advanced IAB, the recurrence rate was lower in patients in the AP + PVI group vs the AP group (90% vs 33.3%, respectively; P = .032). Conclusions PVI after successful AP ablation significantly reduced the AF recurrence rate in WPW patients with advanced IAB. Screening of a resting 12‐lead ECG immediately after AP ablation helps identify patients in whom PVI is beneficial.

Background To investigate the relationship between the location of accessory pathways, electrophysiologic characteristics, and ablation success in Wolfe-Parkinson-White (WPW) syndrome. Methods Electrophysiologic study was performed in 178 patients for a pre-excitation syndrome. Accessory pathway location, anterograde or retrograde conduction, ablation success, and recurrence rate were evaluated. Results Among 178 patients with WPW syndrome, the most frequent location of the accessory pathway was left lateral (39.3%) which had high ablation success rate (97%) and low recurrence rate (1%). A meaningful relationship exists between accessory pathway location and electrophysiologic characteristics. Ablation success rate was 89.7% and was statistically related to accessory pathway location. Recurrent occurred in 2.9% of our patients and was more frequent in right free wall and PJRT. Conclusions The location of accessory pathways has a great impact on conductivity, ablation success, and recurrence rate in WPW syndrome.

BACKGROUND: Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1000 individuals. Mutations in PRKAG2 have been described in rare patients in association with cardiomyopathy. However, the genetic basis of WPW in individuals with a structurally normal heart remains poorly understood. Sudden death due to atrial fibrillation (AF) can also occur in these individuals. Several studies have indicated that despite ablation of an accessory pathway, the risk of AF remains high in patients compared to general population. METHODS: We applied exome sequencing in 305 subjects, including 65 trios, 80 singletons, and 6 multiple affected families. We used de novo analysis, candidate gene approach, and burden testing to explore the genetic contributions to WPW. RESULTS: A heterozygous deleterious variant in PRKAG2 was identified in one subject, accounting for 0.6% (1/151) of the genetic basis of WPW in this study. Another individual with WPW and left ventricular hypertrophy carried a known pathogenic variant in MYH7. We found rare de novo variants in genes associated with arrhythmia and cardiomyopathy (ANK2, NEBL, PITX2, and PRDM16) in this cohort. There was an increased burden of rare deleterious variants (MAF

Background Both atrial fibrillation (AF) and early repolarization (ER) are highly prevalent in patients with Wolff–Parkinson–White (WPW) syndrome. Methods We retrospectively identified 123 WPW patients with manifest accessory pathway (mAP) and 105 patients with concealed accessory pathways (cAP) who underwent successful ablation of the APs. AF history prior to the ablation was investigated. The presence of ER was evaluated from the 12-lead electrocardiograms performed both before and immediately after the ablation. Patients were further followed up for AF occurrence after the AP ablation. Results Compared with cAP patients, WPW patients presented with higher incidence of previous AF, as well as ER after the AP ablation. Compared with those without AF history, WPW patients with comorbid AF were more prone to presenting ER, especially in the inferior leads, after the mAP ablation. Regression analysis suggested that the post-ablation ER in the inferior leads was strongly associated with AF in WPW patients [OR = 5.85; 95% confidence interval (CI): 2.29–14.96], even after adjusting for age and left atrial diameter (OR = 5.14; 95% CI: 1.80–14.74). Moreover, post-ablation inferior-lead ER was predictive of AF recurrence after mAP ablation during the follow-up of 22.6 ± 11.1 months. In comparison, ER was correlated with neither AF history nor AF recurrence in patients undergoing cAP ablation. Conclusions ER in the inferior leads after the mAP ablation is highly correlated with AF history and recurrence in WPW patients.

Case series Patients: Male, 15-year-old • Female, 19-year-old Final Diagnosis: Wolff-Parkinson-White (WPW) syndrome Symptoms: Palpitation Medication: — Clinical Procedure: — Specialty: Anatomy • Cardiology Objective: Unknown etiology Background: Radiofrequency ablation in cases of Wolff-Parkinson-White (WPW) syndrome is a relatively safe procedure that yields good results. However, the electrical characteristics of WPW syndrome have not yet been fully elucidated. Herein, we report 2 cases of WPW syndrome, wherein antegrade conduction was abolished first, followed by retrograde conduction. Case Reports: Case 1: A 15-year-old boy who recently reported experiencing frequent palpitations was diagnosed with type A WPW syndrome by electrocardiography (ECG). Radiofrequency energy was delivered to the earliest activation site using an ablation catheter. This procedure abolished antegrade accessory pathway conduction in 6 seconds, and then the ablation was continued for 60 seconds; however, retrograde accessory pathway conduction remained intact. Hence, radiofrequency ablation was performed to further deliver radiofrequency energy to abolish the retrograde accessory pathway conduction. Case 2: A 19-year-old woman with palpitations since elementary school was diagnosed with type A WPW syndrome by ECG. Radiofrequency energy was delivered to the earliest activation site through an ablation catheter to abolish antegrade accessory pathway conduction in approximately 1 second, and then the ablation was continued for 60 seconds. Retrograde accessory pathway conduction was preserved, and further radiofrequency ablation performed multiple times in the same vicinity abolished retrograde accessory pathway conduction. Conclusions: We managed 2 cases of WPW syndrome wherein antegrade and retrograde accessory pathway conduction were individually abolished. This phenomenon may have been caused by an incomplete lesion that resulted in a functional block.

BACKGROUND Catheter ablation is a routine procedure in patients with WPW syndrome. Silent cerebral infarcts (SCI) detected in magnetic resonance imaging may be a complication of the ablation procedure, but it is well documented only in atrial fibrillation ablation. Ablation of left-sided accessory pathways (L-AP) has a similar target area, but WPW patients differ from those with atrial fibrillation, due to lower initial risk of cerebral embolic events. The aim of this study was to determine whether the ablation of left-sided accessory pathways carries the risk of SCI. MATERIAL AND METHODS Twenty consecutive patients with overt L-AP referred for RF ablation in our center were included in the study. An irrigated ablation catheter was used in 8 patients, and a non-irrigated ablation catheter was used in 12 patients. Diffusion-weighted magnetic resonance imaging was performed pre-procedurally and on the next day after the ablation in all patients. RESULTS Ablation procedures were completed without complications and there were no neurological symptoms following the procedure, although in 2 patients (10%), post-procedural diffusion-weighted magnetic resonance revealed new acute silent cerebral infarcts. Both patients with new cerebral lesions were female, and a non-irrigated catheter was used in both cases. CONCLUSIONS This is the first study documenting the presence of silent cerebral infarcts after WPW ablation. Further investigations are needed to evaluate the risk of silent cerebral infarcts associated with L-AP ablation.

Background Mutations in the PRKAG2 gene encoding the 5′ Adenosine Monophosphate-Activated Protein Kinase (AMPK), specifically in its γ2 regulatory subunit, lead to Glycogen storage disease of heart, fetal congenital disorder (PRKAG2 syndrome). These mutations are rare, and their functional roles have not been fully elucidated. PRKAG2 syndrome is autosomal dominant disorder inherited with full penetrance. It is characterized by the accumulation of glycogen in the heart tissue, which is clinically manifested as hypertrophic cardiomyopathy. There is little knowledge about the characteristics of this disease. This study reports a genetic defect in an Iranian family with liver problems using targeted-gene sequencing. Case presentation A 4-year-old girl presented with short stature, hepatomegaly, and liver cirrhosis. As there was no specific diagnosis made based on the laboratory data and liver biopsy results, targeted-gene sequencing (TGS) was performed to detect the molecular basis of the disease. It was confirmed that this patient carried a novel heterozygous variant in the PRKAG2 gene. The echocardiography was a normal. Conclusion A novel heterozygous variant c.592A > T (p.Met198Leu) expands the mutational spectrum of the PRKAG2 gene in this family. Also, liver damage in patients with PRKAG2 syndrome has never been reported, which deserves further discussion.

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.

Background Wolff-Parkinson-White (WPW) is a congenital defect of the cardiac conduction system (CCS), with proliferation of extra embryologic conduction pathways and rapid conduction of electrical impulses. The estimated neonatal incidence of 0.1% to 0.2% may be misrepresented secondary to missed or misdiagnosis. Undiagnosed WPW can result in sudden cardiac death. Purpose To discuss the pathogenesis, manifestations, diagnosis, management, and lifespan implications of WPW in the prenatal and postnatal periods. Methods/search strategy A literature review was conducted using PubMed, CINAHL, and Google Scholar (2013-2019). Search terms included (newborn OR infant), wolff parkinson white, pathogenesis, management, and ventricular preexcitation. After removal of duplicates, 267 references were identified, abstracts reviewed, and 30 publications fully evaluated. Findings/results Separation of the heart chambers begins around 7 weeks' gestation with formation of the annulus fibrosis complete after term. The unknown external environmental influence on the development of the preterm infant's CCS places neonates at risk for persistent atrioventricular reentrant tachycardia with WPW development. Ensuring an appropriate diagnosis is crucial, as an incorrect diagnosis could mean death. Implications for practice Due to the rarity of WPW, any fetal or neonatal supraventricular tachycardia requires further evaluation with an electrocardiogram and involvement of an experienced cardiologist for diagnosis. One episode of supraventricular tachycardia warrants evaluation for WPW, as recurring episodes may result in irreversible damage. Implications for research The recommendations for treatment of WPW in the prenatal and immediate postnatal periods are based heavily on standards of care for the adult population. A paucity of evidenced-based literature exists and future research is crucial to understand the true incidence, physiologic effects, and lifespan implications of WPW on neonates.

Background Right free wall accessory pathways (AP) are difficult to treat with catheter ablation as ablation catheter (AC) instability at the tricuspid annulus often precludes successful procedure. The aim of our study was to test a novel intra-cardiac echocardiography (ICE) guided technique for AC placement. Feasibility and success rates were observed. Methods Eight consecutive patients (aged 29 ± 21 years, 4 female) with Wolff-Parkinson-White syndrome and a right free wall AP were included in the study. ICE, three-dimensional (3D) electro-anatomic mapping (EAM) system, and a steerable long sheath were used together with either an irrigated or a non-irrigated tip radio-frequency AC to achieve a “loop” manoeuvre which provided AC tip stability at the ventricular aspect of the tricuspid annulus. X-ray fluoroscopy was not used. Results Three patients had an anterior and five had a lateral location of the right free wall AP. Procedures were successful in all patients, without recurrences during the mean follow-up of 397 ± 363 days. Average procedural duration was 90 ± 31 min. On average, 6.6 ± 5.7 ablations were needed. Average time to terminate AP conduction after the start of ablation was 4.8 ± 4.2 s. In five patients (62%) AP conduction was successfully terminated with the first ablation. There were no procedural complications. Conclusions The novel ICE-guided approach with concomitant use of the steerable sheath and the 3D EAM system for zero-fluoroscopy mapping and ablation of the right free wall APs proved feasible and resulted in excellent acute and long-term outcomes.

Cardiac arrest in Wolff-Parkinson-White (WPW) is a rare event, and although some patients appear to be at greater risk, there is no consensus on clear risk factors. We present a case of a 23-year-old male patient, with a known history of WPW pattern, who suffered an out of hospital ventricular fibrillation after the consumption of rather small dose of (meth)amphetamines. The use of illegal drug can predispose WPW patients to fatal arrhythmia and cardiac arrest. Patients with WPW pattern should be well informed about the risks of (meth)amphetamines and some might be considered for medical therapy or catheter ablation.

Ablation of septal accessory pathways (SAPs) is associated with an increased risk of heart block. Data on outcomes of SAP ablation in adults are limited.To describe outcomes of SAP ablation in our center.Patients with Wolff-Parkinson-White syndrome (WPW) undergoing an EP study at our center between January 2008 and August 2019 were identified from our institutional database. Location of the pathway was noted as anteroseptal (AS), midseptal (MS), or posteroseptal (PS). Outcomes of the ablation including success, complication rates, and recurrences were also recorded.Thirty-three patients with SAP underwent 35 EP studies: AS (n = 13), MS (n = 5), and PS (n = 15). Thirty pathways were targeted for ablation, two of which required a 2nd procedure resulting in 32 attempts at ablation in 30 patients. In the remaining 3 patients, SAP did not have malignant features and were not targeted for ablation. Single-procedure success rate was 28/30 (93.33%): 9/10 AS, 5/5 MS, and 14/15 PS ablations. One AS pathway was successfully ablated during a 2nd procedure. Two complications were observed: 1 pericardial effusion in a patient who underwent epicardial mapping and ablation of both PS and right free wall APs. Additionally, transient 2:1 AV block occurred during an MS pathway ablation that recovered during follow-up and did not require permanent pacing procedure.In this single-center experience, ablation of manifest SAP was associated with high success rates and low complication rates. No instances of permanent heart block requiring pacing occurred.

Background Inserting an electrophysiological (EP) catheter into the coronary sinus (CS) via the femoral vein can be difficult and time‐consuming in patients with variants of the CS orifice or lumen curve. Our experience with such patients inspired us to develop two new techniques: the Asclepius and Yellow Ribbon techniques. Methods Data from a 4‐year period were retrieved from records of patients undergoing radiofrequency ablation for paroxysmal supraventricular tachycardia (PSVT) or Wolff–Parkinson–White (WPW) syndrome. Data were analyzed to determine the success and complication rates of conventional and alternative techniques for catheter placement. Results The success rate of the Asclepius technique was 96.7% (30/31) and that of the Yellow Ribbon technique was 100.0% (7/7). The overall success rate of these two techniques was 97.3% (37/38). Conclusions With a high success rate, shorter procedure time, and no complications, the Asclepius and Yellow ribbon techniques may be safe, inexpensive, and effective alternative strategies for EP catheter placement in patients with difficult coronary sinus orifice access.

Catheter ablation is the established treatment for patients with symptomatic Wolff-Parkinson-White syndrome (WPW). However, some patients undergo a challenging ablation or have recurrences during the early post-ablation phase. The aim of this study was to evaluate the clinical factors associated with an unsuccessful ablation outcome or repeated sessions.Four hundred seventy-five symptomatic consecutive WPW patients (38.2±16.2 years old, 61% men, 69% with pre-excitation) who underwent an accessory pathway (AP) ablation from August 2005 to December 2015 were enrolled. When APs recurred, a redo ablation procedure was performed according to the patients' desire.Four hundred thirty-nine patients (92.4%) were cured by ablation, but it failed in 36 (7.6%) after the first procedure. Seventeen patients had AP recurrences during the acute phase within 36h post-ablation. On the other hand, 4 were identified after more than one year. In a multivariate logistic regression analysis, multiple, parahisian, and broad APs were significant independent predictors of recurrences after the 1st procedure, with odds ratios of 14.88 (p0.001), 10.14 (p0.001), and 6.88 (p0.001), respectively. Finally, 468 patients (98.5%) received a successful ablation during a mean follow-up of 8.3±3.0 years. However, after the final procedure no significant predictors were recognized. Out of 508 total procedures, three major (0.6%) complications occurred.Symptomatic WPW patients with multiple, parahisian, and broad APs had a significantly higher risk of recurrence. In half of the recurrence patients, AP recurrences were confirmed during the acute phase, but were rarely recorded in the very late phase.

Danon disease is a severe multisystem disorder clinically characterized by hypertrophic cardiomyopathy, skeletal myopathy and mental retardation in male patients, and by a milder phenotype (predominantly involving cardiac muscle) in female patients. The disease is inherited as an X-linked dominant trait. The primary deficiency of lysosome-associated membrane protein-2 (LAMP-2) causes disruption of autophagy, leading to an impaired fusion of lysosomes to autophagosomes and biogenesis of lysosomes. We surveyed over 500 Danon disease patients reported in the literature from the first description to the present, in order to summarize the clinical, pathological and molecular data and treatment perspectives. An early molecular diagnosis is of crucial importance for genetic counselling and for therapeutic interventions: in male patients, the prognosis is poor due to rapid progression towards heart failure, and only heart transplantation modifies the disease course.

A limited number of case reports of coronary sinus (CS) diverticula complicating catheter ablation have been published. We retrospectively analysed 2245 patients who underwent ablation of an accessory pathway (AP) at our institution between 1/11/1993 and 31/10/2016. Eight patients (0.36%) were found to have a CS diverticulum in venography. APs showed a mean antegrade conduction time of 276 ± 23 ms (range 220–310 ms) and a mean retrograde conduction of 301 ± 45 ms (230–350 ms). Four patients had 1 (n = 2), 2 (n = 1), or 3 (n = 1) previously failed ablation attempts. Pathways could not be ablated with a conventional 4 mm tip catheter in 7 of 8 cases. In seven patients, ablation was successful, in two using an 8-mm ablation catheter, in two using cryoablation, and in the remaining three with an irrigated tip ablation catheter. After failed femoral approach, one 9-year-old female was successfully ablated via the right jugular vein. In one 75-year-old female, ablation was not successful. During a mean follow-up of 8.9 ± 6.4 years, all patients remained free of recurrences. In inferoseptal pathways, especially with previous failed ablation attempts, venographies of the CS should be performed. After successful ablation long-term prognosis is excellent.

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. 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). Patients with left-sided accessory pathway (AP) had significantly higher DwNt (p

Case series Patient: — Final Diagnosis: PRKAG2 syndrome Symptoms: Palpitation • dyspnea and fatigue • syncope Medication: — Clinical Procedure: Radiofrequency catheter ablation • pacemaker implantion • antiarrhythmic drugs Specialty: Cardiology Objective: Rare disease Background: PRKAG2 syndrome diagnosis is already well-defined as Wolff-Parkinson-White syndrome (WPW), ventricular hypertrophy (VH) due to glycogen accumulation, and conduction system disease (CSD). Because of its rarity, there is a lack of literature focused on the treatment. The present study aimed to describe appropriate strategies for the treatment of affected family members with PRKAG2 syndrome with a long follow-up period. Case Report: We studied 60 selected individuals from 84 family members (32 males, 53.3%) (mean age 27±16 years). Patients with WPW and/or VH were placed in a group of 18 individuals, in which 11 (61.1%) had VH and WPW, 6 (33.3%) had isolated WPW, and 1 (5.6%) had isolated VH. Palpitations occurred in 16 patients (88.9%), chest pain in 11 (61.1%), dizziness in 13 (72.2%), syncope in 15 (83.3%), and dyspnea in 13 (72%). Sudden cardiac death (SCD) occurred in 2 (11.1%), and 2 patients with cardiac arrest (CA) had asystole and pre-excited atrial flutter-fibrillation (AFL and AF) as the documented mechanism. Transient ischemic attack (TIA) and learning/language disabilities with delayed development were observed. Genetic analysis identified a new missense pathogenic variant (p.K290I) in the PRKAG2 gene. Cardiac histopathology demonstrated the predominance of vacuoles containing glycogen derivative and fibrosis. The treatment was based on hypertension and diabetes mellitus (DM) control, antiarrhythmic drugs (AD), anticoagulation, and radiofrequency catheter ablation (RCA). Six patients (33.3%) underwent pacemaker implantation (PM). Conclusions: The present study describes the clinical treatment for a rare cardiac syndrome caused by a PRKAG2 mutation.

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.

Background Atrial fibrillation (AF) often coexists with Wolff-Parkinson-White (WPW) syndrome. We compared the efficacy of Kent bundle ablation alone and additional AF ablation on accompanying AF, and examined which patients would still have a risk of AF after successful Kent bundle ablation. Methods This retrospective multicenter study included 96 patients (56 ± 15 years, 72 male) with WPW syndrome and AF undergoing Kent bundle ablation. Some patients underwent simultaneous pulmonary vein isolation (PVI) for AF. The incidence of post-procedural AF was examined. Results Sixty-four patients underwent only Kent bundle ablation (Kent-only group) and 32 also underwent PVI (+PVI group). There was no significant difference in the basic patient characteristics between the groups. Additional PVI did not improve the freedom from residual AF compared to Kent bundle ablation alone (p = 0.53). In the Kent-only group, AF episodes remained in 25.0% during the follow-up (709 days). A univariate analysis showed that age ≥60 years, left atrial dimension ≥38 mm, B-type natriuretic peptide (BNP) ≥40 pg/ml, and concomitant hypertension were predictive factors for residual AF. However, in the multivariate analysis, only BNP ≥40 pg/ml remained as an independent predictive factor (HR = 17.1 and CI: 2.3–128.2; p = 0.006). Conclusions Among patients with WPW syndrome and AF, Kent bundle ablation alone may have a sufficient clinical impact of preventing recurrence of AF in select patients. Screening the BNP level would help decide the strategy to manage those patients.

Kidney is a highly adenosine triphosphate dependent organ in human body. Healthy and functional mitochondria are essential for normal kidney function. Clinical and genetic variability are the hallmarks of mitochondrial disorders. We report here the involvement of two MT-ND5 pathogenic variants encoding for ND5 subunit of respiratory chain complex I, the m.13513G>A and the m.13514A>G, in adult-onset kidney disease in three unrelated patients. The first patient had myopathy encephalopathy lactic acidosis and stroke syndrome, left ventricular hypertrophy with Wolff-Parkinson-White syndrome and tubulo-interstitial kidney disease. The second presented Leber hereditary optic neuropathy associated with tubulo-interstitial kidney disease. The third presented with an isolated chronic tubulo-interstitial kidney disease. These mutations have never been associated with adulthood mitochondrial nephropathy. These case reports highlight the importance to consider mitochondrial dysfunction in tubulo-interstitial kidney disease.

Background The efficacy of nifekalant in preexcited atrial fibrillation ( AF ) has not been assessed. Methods and Results The study populations consisted of patients with sustained preexcited AF (n=51), paroxysmal supraventricular tachycardia (n=201), and persistent AF (n=87). Effects of intravenous infusion of nifekalant were assessed on electrophysiological and clinical parameters. Nifekalant prolonged the shortest preexcited R‐R, the average preexcited R‐R, and the average R‐R intervals from 290±35 to 333±44 ms, 353±49 to 443±64 ms, and 356±53 to 467±75 ms, respectively, in patients with preexcited AF (all P QRS complexes, heart rate, and increased systolic pressure (all P AF in 33 of 51 patients (65%). Similar effects were also observed in a subgroup of 12 patients with preexcited AF and impaired left ventricular function. In patients with paroxysmal supraventricular tachycardia, nifekalant significantly prolonged the effective refractory period, the block cycle length of the antegrade accessory pathway, and the atrial effective refractory period (all P AF without an accessory pathway, nifekalant did not significantly decrease the ventricular rate of AF . One patient developed Torsades de Pointes. No other adverse effects were observed. Conclusions Nifekalant prolongs the effective refractory period of the antegrade accessory pathway and atrium without blocking antegrade conduction through the atrioventricular node, leading to slowing and/or to termination of preexcited AF . Thus, nifekalant might be an effective and a relatively safe drug in patients with preexcited AF .

ObjectivesThis study determined the recurrence and complication rates after radiofrequency catheter ablation (RFCA) for those with paroxysmal supraventricular tachycardia (PSVT), Wolff-Parkinson-White syndrome (WPW), atrial flutter (AFL), atrial fibrillation (AF) and ventricular tachycardia (VT).Study design and settingThis retrospective study included RFCAs for 2001–2010 in the Taiwan National Health Insurance Research Database. Primary outcomes included perioperative complications (pericardial effusion and new-onset stroke), RFCA recurrence and long-term outcomes (high-grade atrioventricular block (AVB) and pacemaker implantation).ResultsOf 19,475 patients who underwent RFCA, prevalence rates were 56.7% for PSVT, 13.5% for WPW, 9.5% for AFL, 5.1% for AF and 2.7% for VT. Prevalence rates increased in AF, AFL and VT over the study years. During an average follow-up period of 4.3 years (SD: 2.8 years), recurrence rates for PSVT, WPW, VT, AFL and AF were 2.0%, 4.9%, 5.7%, 5.8% and 16.1%, respectively. Compared with the PSVT group, the WPW and AF groups had significantly higher risk of pericardial effusion during admission (adjusted OR (aOR) 2.98, 95% CI (CI) 1.24 to 7.15; aOR 4.09, 95% CI 1.90 to 8.79, respectively); the AFL group had a higher risk of new-onset stroke during admission (aOR 4.07, 95% CI 1.39 to 11.91); the WPW group had a lower risk of high-grade AVB during follow-up (adjusted HR (aHR) 0.37, 95% CI 0.19 to 0.71) while the AFL group had a greater risk (aHR 1.74, 95% CI 1.17 to 2.60); and the AFL group had a higher risk of permanent pacemaker (aHR 2.14, 95% CI 1.27 to 3.62).ConclusionsThe RFCA rate increased rapidly during 2001–2010 for AF, AFL and VT. Recurrence was associated with congenital heart disease in PSVT and WPW, and with age in AF and AFL. AFL had a higher risk of permanent pacemaker implantation and new stroke. AF had a higher risk of life-threatening pericardial effusion.

This study was conducted to establish the accuracy of R/S ratios in localizing accessory pathways in Wolff Parkinson White Syndrome on surface ECGs.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.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.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.

PRKAG2 cardiac syndrome (PS) is a rare inherited disease due to PRKAG2 gene mutation and characterized by Wolff-Parkinson-White syndrome (WPWs), conduction system lesions and myocardial hypertrophy. It can also lead to serious consequences, such as sudden death. But the genetic and clinical heterogeneity makes the early diagnosis of PS difficult. Here we studied a family with familial hypertrophic cardiomyopathy and other diverse manifestations. Gene analysis identified a missense mutation (Arg302Gln) in the five affected subjects of the family. The electrocardiograph performance of the five was composed of sinus bradycardia (SB), WPWs, right bundle branch block (RBBB), atrioventricular block (AVB), left bundle branch block (LBBB), supraventricular tachycardia (SVT) and atrial premature beat (APB). Among them, the youngest one began to show paroxysmal palpitation at the age of nine and was confirmed to have WPWs at 17 years old; two members progressed over time to serious conduction damage, and the proband received a pacemaker at the age of 27 due to AVB. Besides, according to cardiac magnetic resonance and echocardiography, the youngest one showed symmetric hypertrophy; three older members showed asymmetric myocardial hypertrophy characterized with a diffuse pattern of middle-anterior-lateral-inferior wall hypertrophy and especially interventricular septal hypertrophy; all five affected patients showed atrial enlargement regardless of myocardial hypertrophy at an earlier stage. In conclusion, the conduction system disorder, familial atrial enlargement and symmetric cardiac hypertrophy may occur in the early stage of PRKAG2 R302Q mutation.

Rtionale: Left ventricular noncompaction (LVNC) is a genetic cardiomyopathy characterized by the presence of a thin compacted layer of myocardium and a spongy subendocardial layer with trabeculations and recesses. LVNC associated Wolf–Parkinson–White syndrome is very rare. Patient concerns: A 32-year-old male presented with short episodes of palpitations and a syncope 6 months before his hospitalization. Diagnosis: His ECG revealed the presence of a right posterior accessory pathway. Echocardiography identified trabeculations of the septal, apical, and lateral wall of the left ventricle, consistent with left ventricular noncompaction. Cardiac MRI confirmed the diagnosis, as the ratio between the noncompacted and compacted myocardial layer was 2.3. Interventions: The electrophysiological study revealed a malignant right posterior accessory pathway. Catheter ablation was successfully performed at the level of posterior tricuspid annulus. Programmed ventricular stimulation could not induce any arrhythmia at the end of the procedure. Outcomes: During 15 months of follow-up, the patient presented no more episodes of palpitations or syncope. Lessons: Left ventricular noncompaction with right accessory pathway is a rare association with genetic basis and gives a higher risk of sudden cardiac death. Catheter ablation of the accessory pathway is a valuable way of treatment in this category of patients, lowering the risk of sudden cardiac death.

To review 10 years of clinical practice of cardiac electrophysiology study and radiofrequency catheter ablation in the treatment of supraventricular tachycardia.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.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.Cardiac electrophysiology studies and radiofrequency catheter ablation were found to be an effective and safe method for diagnosis and treatment of supraventricular tachycardia.

We present a young soldier presenting with aborted sudden cardiac death, who was found to have concomitant hypertrophic cardiomyopathy and Wolff–Parkinson–White syndrome. Along with pathological haemodynamic features of hypertrophic cardiomyopathy, an easily-inducible re-entrant tachycardia was clearly documented in our patient. Given the fatal potential of supraventricular tachycardia in hypertrophic cardiomyopathy, we postulated that his tachyarrhythmia could potentially trigger the event. Upon his refusal to receive implantable cardioverter/defibrillator therapy, we ablated anatomical arrhythmogenic substrate instead, and he remained uneventfully over 3 years on β-blocker.

Rationale: The Wolff–Parkinson–White syndrome (WPW) is a benign heart disease with accessory pathways, which can result in cardiac arrhythmias. The purpose of this case report is to introduce a rare case of sudden cardiac death (SCD) with a mild myocardial bridge and a history of WPW. Patient concerns: A 25-year-old man with known WPW syndrome died at night while sleeping. Diagnoses: Diagnosis of WPW syndrome is based on typical electrocardiogram findings with a documented dysrhythmia before the victim's death. Interventions: At autopsy, no traumatic injury or common poisons were found, only a slight myocardial bridge was detected. We performed whole exome sequencing and identified several genetic variations related to SCD. Outcomes: We considered that the cause of death in this case was SCD in which arrhythmia might play an important role. Lessons: This case highlights SCD can occur in WPW patients with mild or unrecognized structural abnormality. Postmortem genetic examination can assist the diagnosis of sudden cardiac death, especially when no lethal structural abnormality is found in the decedent.

PRKAG2 cardiac syndrome is an autosomal dominant inherited disease resulted from mutations in the PRKAG2 gene that encodes γ2 regulatory subunit of AMP-activated protein kinase. Affected patients usually develop ventricular tachyarrhythmia and experience progressive heart failure that is refractory to medical treatment and requires cardiac transplantation. In this study, we identify a H530R mutation in PRKAG2 from patients with familial Wolff-Parkinson-White syndrome. By generating H530R PRKAG2 transgenic and knock-in mice, we show that both models recapitulate human symptoms including cardiac hypertrophy and glycogen storage, confirming that the H530R mutation is causally related to PRKAG2 cardiac syndrome. We further combine adeno-associated virus-9 (AAV9) and the CRISPR/Cas9 gene-editing system to disrupt the mutant PRKAG2 allele encoding H530R while leaving the wild-type allele intact. A single systemic injection of AAV9-Cas9/sgRNA at postnatal day 4 or day 42 substantially restores the morphology and function of the heart in H530R PRKAG2 transgenic and knock-in mice. Together, our work suggests that in vivo CRISPR/Cas9 genome editing is an effective tool in the treatment of PRKAG2 cardiac syndrome and other dominant inherited cardiac diseases by selectively disrupting disease-causing mutations.

Hypercalcaemia is a common diagnosis with the majority of cases resulting from hyperparathyroidism or malignancy. We report a rare case of persistent symptomatic hypervitaminosis D-induced hypercalcaemia in an individual taking 50 000 IU of vitamin D supplement daily for several months following a diagnosis of vitamin D deficiency. His hypercalcaemia was initially treated with calcitonin and intravenous fluids, but due to recurrent symptomatic hypercalcaemia after discharge, additional treatment with glucocorticoids and bisphosphonates was warranted during his second admission. The pathophysiology of hypercalcaemia from vitamin D intoxication results from the long-term effects of vitamin D storage in adipose tissue. In the present case, we discuss this pathophysiology and treatment approaches in the context of increasing awareness of and testing for vitamin D deficiency, and growing access to over-the-counter supplements.

A 26-year-old asymptomatic competitive athlete was found to have a delta wave consistent with Wolff-Parkinson-White (WPW) pattern on a preparticipation screening ECG. His medical history was unremarkable and without risk factors for coronary artery disease. Physical examination was normal. An

Preexcited Atrial Fibrillation after a Blood Draw A 33-year-old man presented to the emergency department with palpitations. An electrocardiogram showed preexcited atrial fibrillation.

A previously well and active 25 year old woman with no underlying structural heart disease presented with recurrent episodes of new onset syncope over a period of six hours. Her pulse was irregularly irregular at a rate of 230 beats/min. She was hypotensive with a blood pressure of 80/60 mm Hg. Twelve lead surface electrocardiogram (ECG) taken on admission is shown in fig 1. She was immediately electrically cardioverted to sinus rhythm with 150 J. She reported no history of recreational drug use or family history of sudden cardiac death. Fig 1 Surface electrocardiogram during …

A 31-year-old male patient presented with complaints of palpitations, dizziness, and recurrent episodes of syncope. A 12-lead electrocardiogram (ECG) revealed manifest ventricular preexcitation, which suggested Wolff Parkinson White syndrome. In addition, an incomplete right bundle branch block and a 3-mm ST segment elevation ending with inverted T-waves in V2 were consistent with coved-type (type 1) Brugada pattern. An electrophysiological study was performed, and during the mapping, the earliest ventricular activation with the shortest A-V interval was found on the mitral annulus posterolateral site. After successful radiofrequency catheter ablation of the accessory pathway, the Brugada pattern on the ECG changed, which prompted an ajmaline provocation test. A type 1 Brugada ECG pattern occurred following the administration of ajmaline. Considering the probable symptom combinations of these 2 coexisting syndromes and the presence of recurrent episodes of syncope, programmed ventricular stimulation was performed and subsequently, ventricular fibrillation was induced. An implantable cardioverter-defibrillator was implanted soon after.