Wolff-Parkinson-White Research Articles

Wolff Parkinson white pattern in Danon disease: When preexcitation is not what it seems

Danondisease is a rare genetic disorder with an X-linked dominant inheritance affecting both skeletal and cardiac muscle. Its characteristic cardiac phenotype consists on a severe, non-obstructive and concentric hypertrophic cardiomyopathy (HCM) usually associated with a Wolff-Parkinson-White (WPW) type preexcitation pattern. Whether this corresponds to the presence of an AV or another type of accessory pathways, such as fasciculoventricular pathways (FVP) remains controversial in the literature. However, we describe the case of a teenager with Danon disease and preexcitation who develops a first degree AV block without any change in his QRS morphology, fact that favors the hypothesis of the presence of a FVP. This finding has important clinical implications in the management and prognosis of these complex patients. The absence of an AV accessory pathway decreases their risk of potential SCD in the context of a fast atrial arrhythmia and their chances of having a reentrant AV tachycardia.

WOLFF-PARKINSON-WHITE SYNDROME

WPW syndrome is a congenital heart disease that is characterized by the presence of abnormal electrical connections between the atria and ventricles of the heart. In 1930, Louis Wolff, Sir John Parkinson, and Paul Dudley white published a seminal article describing the 11 young patients who suffered from attacks of tachycardia associated with an electrocardiographic pattern of ‘bundle branch block’ with a short PR interval. So from there onwards, it is called Wolff Parkinson white [WPW] syndrome. The normal conduction of the AV node occurs slowly than the accessory pathway conduction. Preexcitation is a process that the cardiac ventricles are activated earlier than the impulse of the AV node which leads to the shorter PR interval and formation of a delta wave. The supraventricular tachycardia associated with WPW syndrome is called AV reentrant or reciprocating tachycardia (AVRT).WPW syndrome is that there is an accessory pathway between the atrium and ventricles which cause rapid heartbeat or tachycardia.

The Pathophysiology, Diagnosis, and Management of Wolff-Parkinson-White Syndrome in the Neonate.

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. To discuss the pathogenesis, manifestations, diagnosis, management, and lifespan implications of WPW in the prenatal and postnatal periods. 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. 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. 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. 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.

New algorithm for accessory pathway localization focused on screening septal pathways in pediatric patients with Wolff-Parkinson-White syndrome

Published algorithms for accessory pathway localization in Wolff-Parkinson-White (WPW) syndrome are inaccurate in pediatric patients, especially for septal pathways. We aimed to develop a new algorithm that is sensitive for septal pathways and more applicable in pediatric patients. In 120 patients (mean age: 11.7 ± 3.9 years) who underwent catheter ablation for WPW syndrome, the candidate criteria for new algorithm were searched by comparing electrocardiography parameters and accessory pathway locations. A new algorithm was designed to increase the sensitivity for septal pathways. For validation, 142 patients (mean age: 15.8 ± 3.7 years) were additionally evaluated. New and published algorithms were applied to electrocardiography of 262 patients and the results were compared. The new algorithm achieved its best discrimination by combining several parameters together in each step: (1) QRS polarity in V1 and QRS shape in lead I for left/right discrimination, and (2) delta wave polarity in V1, QRS transition in precordial leads, and delta wave polarity in lead III for septal pathway screening. The new algorithm showed higher sensitivity for septal pathways (95.7%) than 7 published algorithms (average: 62.1%), with satisfactory positive predictive value (77.9%). Delta wave polarity in V1 among septal pathways and QRS axis among right anteroseptal pathway showed age-related trend; this could be the reason for the lower accuracy in localizing septal pathways in children. The inaccuracy of published algorithms in pediatric patients is due to the age-related trend in the electrocardiography of septal pathways. The new algorithm was superior for localizing septal pathways in pediatric patients.

Noninvasive Localization of Accessory Pathways in Wolff–Parkinson–White Syndrome by Two-Dimensional Speckle Tracking Echocardiography

Background: Two-Dimensional Speckle Tracking Echocardiography (2D-STE) is a non-Doppler echocardiographic modality that allow quantification of myocardial deformity and its timing in 2D grey scale images, and was proved to be beneficial in assessment of dyssynchrony (STAR trial). Objective: To investigate the capability of 2D-STE for localizing Accessory Pathways (APs) in patients with Wolf-Parkinson-White (WPW) Syndrome. Patients and Methods: The prospective study included 15 patients with manifest AP indicated for invasive endocardial mapping and ablation. All patients were assessed by twelve-lead ECG, 2D-STE for the earliest activated site and invasive Electrophysiology study (EPs). Results: Out of the 15 patients with left-sided APs, 2D-STE was able to accurately localize the site of AP in 10 patents (66.6%), while electrocardiogram (ECG) was able to localize the site of AP in 12 patients (80%). Conclusion: 2D-STE has the ability to approximate the location of AP without the pitfalls of Doppler methods. However, ECG still more accurate and reproducible tool.

Infraclavicular Brachial Plexus Block and Sugammadex Use in a Paediatric Patient with Wolff-Parkinson--White Syndrome.

BackgroundWolff–Parkinson–White (WPW) syndrome is a rare disease that can cause various patterns of tachyarrhythmias. The main goal of anaesthesiologists for the perioperative anaesthetic management of WPW patients must be to avoid sympathetic stimulation and prevent tachyarrhythmias.CaseA 9-year-old male patient with a diagnosis of WPW syndrome, who underwent emergency surgery for supracondylar humerus fracture, is presented. General anaesthesia in combination with infraclavicular brachial plexus (ICBP) block was performed successfully without any complications. For the reversal of neuromuscular block, sugammadex was uneventfully used. Intraoperative sympathetic discharge due to surgical intervention was prevented with ICBP block. The ICBP block has also provided good postoperative analgesia.ConclusionsSugammadex is a good alternative for the reversal of neuromuscular blocks to avoid the undesirable effects of cholinergic drugs. Peripheral regional blocks that require relatively large doses of local anaesthetics can be safely performed with utmost attention in paediatric WPW patients.

Incessant accelerated idioventricular rhythm mimicking preexcitation

ECG of patients with Wolf Parkinson White (WPW) syndrome may simulate other entities such as myocardial infarction, ventricular premature complexes, ventricular bigeminy, accelerated idioventricular rhythm, intermittent bundle branch block or electrical alternans. On the other hand, the opposite can also occur where these other conditions may simulate WPW. We present the case of a young patient referred for WPW ablation showing an incessant accelerated idioventricular rhythm mimicking preexcitation.

Novel Assessment of Accessory Pathway Function in Patients with Wolff-Parkinson-White Syndrome.

Surrogates for the shortest pre-excited R-R interval in atrial fibrillation (SPERRI) such as the accessory pathway effective refractory period (APERP) and shortest pre-excited paced cycle length (SPPCL) are flawed assessments of accessory pathway function in patients with WPW. Multi-extrastimulus pacing may have the theoretical advantage of more accurately mimicking the clinical reality of atrial fibrillation and thus may serve to better assess accessory pathway function. This cross-sectional study included 25 consecutive patients, aged≤18years, undergoing electrophysiology study for WPW. The longest S1S2, S2S3, S3S4 coupling intervals at which the antegrade AP refractoriness occurred, SPERRI, and SPPCL were recorded. Induction of atrial fibrillation was attempted in all patients and induced in 8 (32%, 4 SPERRIbaseline (265ms±61ms), 4 SPERRIIsuprel (258ms±41ms)). At baseline, the lower value of the S3ERP or S4ERP (274ms±52ms) was lower than the SPPCL (296ms±54ms, p<0.0001) and APERP (296ms±41ms, p<0.0001). More patients had S3ERP or S4ERP≤250ms (12/25, 48%) compared to those with APERP≤250ms (2/25 8%), p=0.0016), SPPCL 5/24, 20%), p=0.008 or either (6/25, 24%), p=0.0143). With Isuprel, the lower value of the S3ERP or S4ERP (221ms±36ms) trended to be lower than the APERP (252ms±36ms, p=0.0001) and the SPPCL (266ms±57ms, p=0.001). With Isuprel, there was no statistical difference in the proportion of patients with S3ERP or S4ERP<250ms (12/16, 75%) compared to those with APERP≤250ms ((9/16, 56%), p=0.08), SPPCL≤250ms ((9/16, 56%), p=0.08), or either ((10/16, 63%), p=0.16). Multi-extrastimulus pacing protocols demonstrate that accessory pathways are less refractory than as defined by single extrastimulus pacing and straight decremental pacing.

Pre-excitation cardiac problems in children: recognition and treatment.

The prevalence of ventricular pre-excitation is 0.07-0.2% in the pediatric population. Kent bundle is the most common atrioventricular accessory pathway and Mahaim fiber is relatively rare. Approximately, 30-60% of children with ventricular pre-excitation have onset of atrioventricular reentrant tachycardia. Persistent atrioventricular reentrant tachycardia can lead to tachycardiomyopathy. The anterograde conduction of right accessory pathway might lead to ventricular systolic dyssynchrony which might result in cardiac dysfunction even in patients with no tachycardia onset. This type of dilated cardiomyopathy was named as accessory pathway-induced dilated cardiomyopathy. Antiarrhythmic drugs can be used to acutely terminate tachycardia or taken orally to decrease tachycardia recurrence in the long term. However, antiarrhythmic drugs that can be chosen for children are quite limited. Sotalol has become a new choice. With the maturation of radiofrequency catheter ablation technique, progress in three-dimensional electro-anatomic mapping, use of cryoablation, and accumulation of experience in children with small age and weight, catheter ablation has become the first choice for children with pre-excitation syndrome.Conclusion: For ventricular pre-excitation co-exists with dilated cardiomyopathy, differential diagnosis of tachycardiomyopathy or accessory pathway-induced dilated cardiomyopathy should be considered. Catheter ablation (radiofrequency and cryoablation) is a relatively safe and effective treatment option and has become the first choice to treat children with ventricular pre-excitation. What is Known: • Persistent atrioventricular reentrant tachycardia in children can lead to tachycardiomyopathy; • Antiarrhythmic drugs that can be chosen for children are quite limited. What is New: • The anterograde conduction of right accessory pathway (not related to supraventricular tachycardia) might lead to accessory pathway-induced dilated cardiomyopathy. • Catheter ablation (including radiofrequency and cryoablation) has become the first choice for children with pre-excitation syndrome.

Correlation Between Arrhythmia and the Prognosis in Children With EFE/LVNC/DCM.

Aim: To explore the correlation between different phenotypes of arrhythmia and the prognosis in children with EFE/LVNC/DCM.Methods: A total of 167 children with cardiomyopathy diagnosed and treated in Shengjing Hospital between January 2010 and May 2019 were evaluated. After patient screening, 31 patients with endomyocardial fibroelastosis (EFE), left ventricular non-compaction, or dilated cardiomyopathy with significant arrhythmias were selected. In addition, 42 children with primary EFE were selected to evaluate the prognosis with or without arrhythmia. Follow-up was undertaken 0, 1, 3, 6, 9, and 12 months after treatment.Results: We revealed the outcomes for five types of cardiomyopathy: EFE patients with Wolff–Parkinson–White syndrome B and supraventricular tachycardia, intraventricular block and complete left bundle branch block recovered slower than EFE patients with atrial flutter and atrial fibrillation, even slower than EFE with ventricular tachycardia. The average recovering time for LVEF and LVED in EFE patients without arrythmia was 10 months after diagnosis, while 76.9% (3/13 cases) of those with significant arrythmia hadn't recovered until 24 months after diagnosis. Three of patients died at 6, 7, and 6 and half years after diagnosis.Conclusion: The long-term prognosis in children with cardiomyopathy is associated with the type of arrhythmia and time of intervention. The prognosis of EFE patients with arrhythmia is worse than EFE patients without arrhythmia. Patients with Wolff–Parkinson–White syndrome B, especially a significantly widen QRS complex, carry a poor prognosis if radiofrequency ablation is not undertaken. CLBBB patients have similar poor prognosis if proper pacemaker is not implanted timely.

Clinical characteristics of challenging catheter ablation procedures in patients with WPW syndrome: A 10 year single-center experience

BackgroundCatheter 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. MethodsFour 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. ResultsFour 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 (p<0.001), 10.14 (p<0.001), and 6.88 (p<0.001), 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. ConclusionsSymptomatic 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.

P951Autonomic nervous function and excitability of the accessory pathway in pediatric patients with Wolff-Parkinson-White ECG

Abstract Abrupt loss of preexcitation identifies low-risk Wolff-Parkinson-White (WPW). We hypothesize that the autonomous nervous system (ANS) modulates the conduction of the accessory pathway (AP). PURPOSE To compare heart rate variability (HRV) in children with (WPWi) and without (WPWp) intermittent preexcitation. METHODS The study conforms to the Declaration of Helsinki. 18 WPW children and controls performed ECG Holter for HRV analysis. Intermittent preexcitation was defined as abrupt loss of WPW pattern. Kolmogorov-Smirnov test confirmed normal distribution of data. T-test and Fisher"s exact test were used for continuous and categorical variables. Multivariate regression excluded the effect of potential confounders (figure 1 shows correlation between HRV and HR). RESULTS WPWi (11/18, 61.1%) have higher HRV as compared with WPWp. Despite WPWp patients are younger, with higher HR, corrected multivariate analysis confirmed significant differences (table 1), also between WPW patients and controls. CONCLUSIONS WPW patients have lower HRV. WPWp may have worst autonomic control to the excitability of AP. Table 1 WPW (n = 18)A WPWi (n = 11)B WPWp (n = 7)C Controls (n = 18)D P value A vs. D P value B vs. C P value B vs. D P valueC vs. D Age 7.6 ± 4 10.1 ± 4.2 4.1 ± 2.9 7.8 ± 4.7 0.9 &amp;lt;0.01 0.1 0.03 24 hours mean HR 92.8 ± 15.9 81.1 ± 11.2 104.9 ± 12.6 90.3 ± 16.5 0.7 &amp;lt;0.01 0.03 0.07 pNN50 20 ± 11.4 30.5 ± 11.8 14.7 ± 9.9 24.3 ± 13.4 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 RMSSD 53 ± 19.1 68.8 ± 21.4 44.6 ± 14.7 59.4 ± 22.2 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.01 ASDNN 64.9 ± 21.1 78.4 ± 20.7 48.8 ± 11.8 66.9 ± 22.9 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 SDANN 108.2 ± 39.8 127.9 ± 36 77.5 ± 16.9 108.3 ± 38.8 &amp;lt;0.001 &amp;lt;0.01 &amp;lt;0.001 &amp;lt;0.01 VLF 7.4 ± 0.8 7.8 ± 0.4 6.9 ± 0.5 7.5 ± 0.6 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 LF 6.8 ± 0.8 7.3 ± 0.4 6.4 ± 0.4 6.9 ± 0.6 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 HF 6.6 ± 0.8 7.1 ± 0.7 6.2 ± 0.6 6.7 ± 0.8 &amp;lt;0.001 &amp;lt;0.01 &amp;lt;0.001 &amp;lt;0.01 LF/HF 1.4 ± 0.8 1.4 ± 0.7 1.4 ± 0.6 1.4 ± 0.6 0.2 0.3 0.05 0.7 VarIndex% 4.8 ± 1.4 5.8 ± 1.2 4.4 ± 1.4 5.3 ± 1.4 &amp;lt;0.001 &amp;lt;0.01 &amp;lt;0.01 &amp;lt;0.01 Total Power 8.2 ± 0.7 8.6 ± 0.4 7.8 ± 0.4 8.3 ± 0.6 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 &amp;lt;0.001 Frequent SVB 2 (11%) 1 (9.1%) 1 (14%) 0 0.5 1.0 0.4 0.3 SVT 1 (5.6%) 1 (9.1%) 0 0 n.a. n.a. n.a. n.a. P values from multivariate analysis and t-test for age and HR. HR: heart rate; WPWi: intermittent preexcitation; WPWp: persistent preexcitation; VarIndex%: variability index (%); SVB: supraventricular beats; SVT: supra ventricular tachycardia. Values are expressed as mean ± SD for age, HR and time domain variables (ms). Variables in the frequency domain are expressed as ln. P &amp;lt;0.05: significant. Abstract Figure 1

Increased preexcitation on electrocardiography improves accuracy of algorithms for accessory pathway localization in Wolff–Parkinson–White syndrome

Several electrocardiographic (ECG) algorithms have been developed for predicting accessory pathway (AP) location in Wolff-Parkinson-White syndrome. However, their accuracy may be related to the manifested degree of preexcitation on ECG. Our goal was to assess the effect of the degree of preexcitation on the accuracy of 4 traditional AP localization algorithms and to compare them with the algorithm specifically designed for ECGs with maximal preexcitation (Pambrun) Methods: The study included 300 patients who underwent successful ablation of an overt atrioventricular AP. Resting and maximally preexcited ECGs obtained during incremental atrial pacing were assessed using 4 traditional AP localization algorithms: Xie, d'Avila, Iturralde, and Taguchi. Maximally preexcited ECGs were additionally assessed with the Pambrun algorithm. We compared the precision of the algorithms to predict accurate or anatomically adjacent AP location. Theoverall accuracy of traditional AP localization algorithms using resting ECG ranged between 26% and 53.7% and improved to a range of 47.3% to 69.7% when adjacent locations were accepted. When used with maximal preexcitation, all algorithms had significantly higher accuracy, with a mean improvement of 14.3 and 15.6 percentage points for precise and adjacent sites, respectively. The Pambrun algorithm for maximally preexcited ECGs had the highest precision for both accurate and adjacent locations of the APs (89.7% and 97%, respectively). Greater preexcitation on ECG improved the accuracy of the traditional AP localization algorithms. The algorithm designed to use maximally preexcited ECGs has the best accuracy. Maximally preexcited ECG recordings should preferably be used in clinical practice to facilitate the ablation procedure.

Wolff parkinson white syndrome in third trimester of pregnancy: A case report

Arrhythmias are the one of the most common forms of cardiac disease during pregnancy. Supraventricular tachycardia is the most common type of sustained arrhythmia presenting in pregnancy. During pregnancy, this can be difficult to diagnose as common symptoms like palpitation, shortness of breath and syncope. We presented our cases of supraventricular tachycardia in pregnancy during antenatal period. Risk factors for the development of supraventricular tachycardia and use of antiarrhythmic drugs management, during pregnancy and at Caesarean section are discussed. We recommend the use of adenosine as first line therapy.

SUCCESSFUL PERFORMANCE OF CARDIAC ELECTROPHYSIOLOGICAL PROCEDURES VIA A CONGENITAL INFERIOR VENA CAVA ANOMALY- REPORT OF 2 CASES

Background: Femoral vein access is the preferred approach for advancing multiple catheters via the inferior vena cava (IVC) to the heart during routine cardiac electrophysiology study and catheter ablation. Compared to acquired venous abnormalities, congenital inferior vena cava anomalies are encountered rather infrequently in adult electrophysiology procedures and their presence may pose technical procedural challenges. Presentation: We describe 2 cases in which we were able to successfully perform cardiac electrophysiological procedures in the presence of a complex congenital venous anomaly, the left sided IVC. First case was 30 year old gentleman presented with history of recurrent episodes of supraventricular tachycardia terminated with AV nodal blocking agents. Second case was 21 year old boy with WPW syndrome and recurrent supra-ventricular tachycardia. Diagnosis and Management: We managed to pass the EP catheters in both cases with a bit difficulty and angulation, while given I/V heparin to reduce the risk of thrombosis and confirmed the position of catheters via subclavian venous placement of a guidewire. In first case, typical AV node reentry tachycardia was induced which was mapped and ablated in the slow pathway region. In second case, right posterior accessory pathway was ablated at 6 0’clock. CT angiography of the abdominal veins was performed which confirmed the finding of left sided IVC. Follow-up and Outcomes: Abnormalities of the IVC are relatively uncommon. But it is an important condition that may be encountered by electrophysiologist. Catheter ablation of the slow AV nodal pathway and right posterior accessory pathway was safely and successfully performed with this unusual venous anomaly. Keywords: SVT, LEFT IVC, RFA

DISORDERS OF CARDIAC RHYTHM IN PATIENTS WITH PRE-EXCITATION SYNDROME OF VENTRICLES AND THEIR PHARMACOLOGICAL CORRECTION

The aim of the research was to analyze the possibility of using different antiarrhythmic agents in patients with pre-excitation syndrome and disorders of cardiac rhythm. Most typical disorders of cardiac rhythm in patients with pre-excitation syndrome are orthodromic reciprocating supraventricular tachycardia, antidromic supraventricular tachycardia, atrial fibrillation and atrial fibrillation. During attack of tachycardia in patients with syndrome of pre-excitation, different clinical symptoms can be observed. They can range from mild palpitation to syncope. This tachycardia can be even reason of sudden cardiac death. Main its mechanism is macroreentrant circuit involving the AV-node, the additional pathway, the atria, the ventricles. To arrest the attacks of atrioventricular reciprocating tachycardia in patients with WPW syndrome and with narrow complexes QRS, calcium channel blocker verapamil has efficacy in 95% of patients. However, for treatment this arrhythmia with wide complexes QRS verapamil is contraindicated. Besides, it should be taken in account that treatment with this type of cardiac arrhythmia cardiac glycosides is also forbidden. Verapamil and cardiac glycosides are contraindicated for termination of arrhythmia in patients with WPW syndrome and such disorders of cardiac rhythm as atrial fibrillation (flutter). For therapy of atrial fibrillation (flutter) in patients with WPW syndrome antiarrhythmic agents of agents of І A subclass (quinidine, procainamide, disopyramide, propafenone) and ІІІ class (amiodarone, sotalol) can be useful. For interruption of paroxysmal tachycardia in patients with pre-excitation syndrome antiarrhythmical preparations of plant origin (gilurytmal and allapinin) have high efficacy. Termination of paroxysmal tachycardia in patients with premature excitation of ventricles can be achieved after administration of antiarrhythmic agents of ІС subclass, in particular after using of propafenone and encainide. However, treatment with the help of these agents quite often leads to appearance of arrhythmogenic action. In pre-excitation syndrome and cardiac arrhythmias, it is impossible to use drugs, which cause the acceleration of conductivity of nerve impulses in additional pathways (cardiac glycosides, β-blocker agents, for example propranolol). In patients with paroxysmal atrioventricular reciprocating (circular) tachycardia digitalis and calcium channel blockers should be avoided. Such agents as digoxin and verapamil in this arrhythmia can turn out to be dangerous in WPW syndrome, since they raise the conductivity through additional conductive pathways.

Atrial Fibrillation Associated with Wolf-Parkinson-White Syndrome in Patients with Brugada Syndrome: A Review

The Brugada syndrome is an autosomal dominant rare form of cardiac arrhythmia and has been associated with high risk of sudden cardiac death predominantly in younger male patients. It is associated with polymorphic ventricular arrhythmia/ventricular fibrillation, Supraventricular arrhythmia mainly atrial fibrillations and Wolf- Parkinson white syndrome. Patients can be presented with symptom like syncope, palpitation, sudden cardiac death and asymptomatically. Several pathogenic genes have been identified as associated with the disease but SCN5A is the most prevalent one. Several genetic mutations of different subunits of sodium, calcium and potassium channel have been involved. The management of Brugada syndrome and Wolf-Parkinson-White syndrome in patients with atrial fibrillation should be generally includes implantable cardioverter defibrillator and Radiofrequency catheter ablations. This brief review focuses on recent clinical diagnosis, genetic basis and advances in pharmacological treatment of Brugada syndromes with atrial fibrillation and wolf-Parkinson white syndrome.

Accessory Pathway Ablation Located Just Below The Bundle of His: A Challenging Case

We presented a case of Wolff-Parkinson-White (WPW) Syndrome. A 69-year-old man was admitted for evaluation of frequent episodes of palpitation. The diagnosis of WPW syndrome was established based on the 12-lead surface electrocardiogram (ECG) and electrophysiology (EP) study. We conducted the radiofrequency ablation (RFA) to the accessory pathway (AP). The challenging point of this case was the accessory pathway located just below to the bundle of His, which is related to the risk of complete atrioventricular (AV) block. In summary, we conclude that anatomical consideration, EP study, and the ablation strategy were important to improve the safety and success rate of RFA procedure.

Wolff-Parkinson-White syndrome: De novo variants and evidence for mutational burden in genes associated with atrial fibrillation.

Wolff-Parkinson-White (WPW) syndrome is a relatively common arrhythmia affecting ~1-3/1,000 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. 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. 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 ≤ 0.005) with CADD score ≥ 25 in genes linked to AF in cases compared to controls (P = .0023). Our findings show an increased burden of rare deleterious variants in genes linked to AF in WPW syndrome, suggesting that genetic factors that determine the development of accessory pathways may be linked to an increased susceptibility of atrial muscle to AF in a subset of patients.

Prevalence and Outcomes of Patients with Wolff-Parkinson-White in Danon Disease: Results of a Retrospective Analysis

Danon Disease is a rare X-linked myopathy caused by defects in the lysosome-associated membrane 2 (LAMP2) gene. It is strongly associated with hypertrophic cardiomyopathy as well as Wolff-Parkinson-White (WPW) syndrome, but the prevalence of WPW in Danon is unclear. This study examined the prevalence of WPW and associated outcomes in a cohort of patients with Danon Disease. Records were reviewed for 41 patients with confirmed Danon Disease enrolled in a previously described retrospective registry, 31 of which had at least one electrocardiogram (EKG) on file. EKGs were assessed for the presence of WPW, as well as left and right ventricular hypertrophy (LVH, RVH), or both. WPW was defined as a PR interval ≤ 120 milliseconds, QRS interval ≥120 milliseconds, and delta wave. Charts were reviewed for internal cardiac defibrillator (ICD) placement, heart transplantation, and death. These outcomes were evaluated between patients with and without WPW on EKG using chi-square analysis, with p <0.05 noted to be significant. EKG criteria for LVH, RVH or both was met in 10 of the 11 WPW patients, compared to 9 of the 20 without WPW (p=0.012). ICD devices were present in 9 of the 11 WPW patients, 3 of which were placed for secondary prevention, compared to 9 of the 20 patients without WPW (p=0.119). Six of the 11 WPW patients eventually underwent heart transplant, compared to 4 of the 20 without WPW (p=0.058). Only one death was noted in this cohort, and was determined to not be cardiac related. In a cohort of patients with Danon Disease, approximately one-third had WPW syndrome on EKG. Those with WPW on EKG had higher rates of EKG criteria for LVH, RVH or both and were more likely to require advanced therapies such as ICD placement and cardiac transplant. Larger studies involving WPW in Danon Disease should seek to better characterize these outcomes.