Wolff-Parkinson-White Research Articles

Anaesthetic challenges in a patient with Wolff-Parkinson-White (WPW) syndrome for orchidectomy.

Wolff-Parkinson-White (WPW) syndrome is an extremely rare congenital cardiac conduction disorder. It is due to an aberrant pathway between the atrium and ventricle. This manuscript entails a man with an underlying WPW who was posted for an elective orchidectomy. We discussed the important perioperative precautions to prevent the precipitation of acute cardiac events.

Essentials of Paroxysmal Supraventricular Tachycardia for the Pediatrician.

Paroxysmal supraventricular tachycardia (SVT) is the most common clinical arrhythmia in young patients. With an estimated prevalence of roughly 1 in 500 children, a pediatrician may knowingly or unknowingly see several patients who are affected each year. SVT symptoms can sometimes be vague or conflated with common pediatric complaints. Different forms of SVT are predominant at different ages, sometimes complicating timely recognition and referral. Differing pathophysiology and age distribution impact risk of complications such as heart failure, or rarely, sudden death. Treatment choices continue to evolve as new medications, monitoring devices, and ablation technologies continue to mature. This review focuses on the most common types of paroxysmal SVT: atrioventricular nodal reentrant tachycardia, atrioventricular reciprocating tachycardia, and Wolff-Parkinson White syndrome. Atrial arrhythmia mechanisms are also briefly outlined. It is meant to provide practical guidelines for the diagnosis and management of patients with SVT from infancy through adolescence. [Pediatr Ann. 2021;50(3):e113-e120.].

Wolff-Parkinson-White pattern on alternate beats: What is the mechanism?

The Holter electrocardiogram from an asymptomatic man shows intermittent preexcitation. At the lowest rates all the QRS complexes display a WPW pattern. As the rate increases, preexcitation fails to occur and all the QRS complexes become persistently narrow. With a further increase in sinus rate the WPW occurs on alternate beats and this alternation is maintained for a while. A careful analysis of the accessory pathway conduction in relation to sinus-cycle length and morphology of the prior beat strongly supports the supernormal conduction through the Kent bundle associated with linking as the key mechanism underlying the preexcitation on alternate beats.

Ebstein anomaly, left ventricular hypertrabeculation and ventricular septal defect associated with a myh7 mutation

Abstract Funding Acknowledgements Type of funding sources: None. Ebstein anomaly is an extremely rare anomaly of <1% among all congenital heart diseases. Pathologically, the septal and / or posterior leaflet of the tricuspid valve has abnormal locations towards the right ventricular apex. Ebstein anomaly is especially accompanied by atrial septal defect, patent ductus arteriosus, wolf parkinson white syndrome and pulmonary atresia. Defects located in the interventricular septum are called the ventricular septal defect (VSD). They can be single or multiple and congenital or acquired. Isolated VSDs are the most common congenital anomaly in childhood and constitute 20-30% of all congenital heart diseases. VSD can be a part of major congenital malformations ,such as, fallot tetralogy, transposition of the major arteries, double ventricular right ventricle. Left ventricular noncompaction (LVNC) is a relatively common genetic cardiomyopathy, characterized by prominent trabeculations with deep intertrabecular recesses in mainly the left ventricle. Although LVNC often occurs in an isolated entity, it may also be present in various types of congenital heart disease . A combination of Ebstein anomaly, hypertrabeculation and ventricular septal defect is a rare condition. Case Report A 49-year-old male patient presented to the emergency room with shortness of breath and swelling of the legs. The patient had diagnosed an Ebstein anomaly while the military examination in 1988. Already it ‘s known that he has gout disease and uses colchicine but no family history of any disease. On examination of the patient, bilateral ral in respiratory sounds and +++ / +++ pretibial edema in the lower extremity were detected. On his electrocardiogram, the sinus rhythm with first-degree atrioventricular block was observed. The findings on his echocardiographic examination are ejection fraction 30-35% with global left ventricular hypokinesia, Ebstein anomaly (Figure ), perimembranous type VSD, atrial septal aneurysm type 2 and left ventricular hypertrabeculation. His blood table was normal. Medical treatment of heart failure was started for the patient who was interneed to the service. After getting clinical relief, the patient was discharged under medical treatment. Genetic tests were studied while following up at the heart failure outpatient clinic. In the MYH7 gene, splice-acceptor-2 (PVS1) variation heterozygous was detected. This variant has not been seen in national data banks of genetics. Conclusion The MYH7 gene, localized on chromosome 14p12, is composed of 41 exons and encodes the b-myosin heavy chain, expressed in cardiac muscle. Mutations in the MYH7 gene have been identified in association with left ventricular hypertrabeculation and Ebstein anomaly. In conclusion, this is the first known report of Ebstein anomaly associated with the splice-acceptor-2 variation heterozygous of the MYH7 gene. Abstract Figure

Arrhythmias in Neonates and Infants at a Tertiary Care Center

BackgroundLimited data about arrhythmias in neonates and infants are coming out from the Middle East.ObjectivesTo evaluate different types of arrhythmias in neonates and infants at the American University of Beirut Medical Center (AUBMC), a tertiary care center in Lebanon, with the focus on the nature of arrhythmia, treatment modalities and relation to surgery.MethodsData were collected retrospectively from the hospital records. We included all neonates and infants presenting to AUBMC between 2013 and 2017. Collected data included: the type of arrhythmia, the treatment modality used and its success, the need for additional modes of treatment, the relationship to congenital heart diseases, and the cardiac surgeries performed.ResultsOf 16,346 subjects admitted to AUBMC between 2013 and 2017, 90 subjects developed arrhythmias that required medical intervention. The most frequent types of arrhythmias were supraventricular tachycardia (62.22%), junctional ectopic tachycardia (13.33%), complete heart block (7.78%), atrial flutter (5.56%), multifocal atrial tachycardia (3.33%), Wolf Parkinson White Syndrome (3.33%), non-sustained ventricular tachycardia (2.22%), sinus pause (1.11%), and premature ventricular contractions (1.11%). Bivariate analysis showed a significant difference between arrhythmias not related to cardiac surgery and arrhythmias related to cardiac surgery in terms of the type of arrhythmia developed, the presence of congenital heart defect, prematurity, and electrolyte disturbances (P-value <0.005). However, multivariate logistic regression showed no significant difference between the two groups after adjustment for the significant variables (P-value > 0.05).ConclusionThere is a significant difference between arrhythmias not related to cardiac surgery and arrhythmias related to cardiac surgery in neonates and infants at AUBMC. However, the difference disappears after adjusting for different variables.

Wolff — Parkinson — White syndrome in nurse practice

Wolff — Parkinson — White syndrome (WPWS) is still the leading cause of supraventricular tachycardia in the world. The frequency of WPWS ranges from 0.1 to 0.3 % per 1000 people. It is worth noting that in patients with congenital heart defects, it increases to 0.5 %. The article highlights the main criteria of Wolff — Parkinson — White syndrome, its morphological justification, and the features of modern topical diagnosis. Also, the review of the main methods of treatment and rehabilitation of patients is presented.

Wolf-Parkinson-White syndrome

We report the case of a 59-year-old patient with no cardiovascular risk factors and medical history. He consults in the emergency room for rapid onset of palpitations with precordial pain. Clinical examination was normal. The electrocardiogram shows a short PR interval <120 ms, an initial QRS foot base, enlargement of the QRS complexes with repolarization anomalies. Wolf Parkinson White syndrome is defined by the presence of a permeable accessory pathway (Kent bundle) leading to ventricular pre-excitation which by passes the normal conduction. The ventricular myocardium is therefore depolarized in advance by the accessory bundle, which results in a fusion between the first atrioventricular activation and that the one that passes through the normal pathway. The electric aspect combines a short PR interval <120 ms, an initial QRS foot base called the delta wave, an enlargement of the QRS complex with frequent repolarization anomalies. The pre-excitation of wolf parkinson white is usually asymptomatic. Tachycardia and/or arrhythmia may cause the following symptoms: palpitations, dizziness, faintness, syncope, chest pain, and sudden death. It had numerous etiologies: idiopathic, Epstein´s disease, coronary artery disease, hyperthyroidism, or high blood pressure. Episodes of paroxysmal supraventricular tachycardia due to Wolff Parkinson´s white syndrome can be stopped by any vagal maneuver which slows the heart rate. When these maneuvers are ineffective, drugs such as verapamil and adenosine are usually used to stop the arrhythmia. Arrhythmic drugs can then be taken indefinitely to prevent further episodes. Catheter ablation of the accessory bundle is successful in over 95% of cases.

Electocardiographic features in Ebstein's disease

Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease. The purpose of our work is to describe the electrocardiographic features observed in Ebstein's disease. We conducted a retrospective descriptive study that enrolled 26 patients followed for ED. The mean age of discovery of the ME was 103.51 ± 99 months [0–31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42.3%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42.3%). Eight patients (30.7%) had wide QRS ≥ 120 ms. Seven of these 8 patients (26.9%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84.6%), it was a complete block in 7 cases (26.9%). A preexcitation was found in 6 patients (23.1%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34.6%). It was a junctional tachycardia in 3 patients (11.5%), atrial flutter in 4 patients(15.4%) and atrial fibrillation in 2 patients (7.7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome. Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.

ПРОГНОСТИЧЕСКИЕ ФАКТОРЫ СИНДРОМА ВОЛЬФА-ПАРКИНСОНА-УАЙТА В ДЕТСКОМ ВОЗРАСТЕ

Objective: to determine the prognostic factors determining the severity of Wolff-Parkinson-White syndrome (WPW) in children. Methods: a retrospective analysis of the course of the disease was performed in 108 children with WPW syndrome aged 13.6 (12-16) years. Depending on the number of attacks of paroxysmal tachycardia (PT), all children were divided into 2 groups: group A consisted of 47 children (43.5%) with a history of no more than three attacks of PT and they were rare; group B included 61 children (56.5%) with frequent (more than once a month) attacks of PT, more than four in the history. We studied: the family history, the clinical picture of the disease, the nature of paroxysmal tachycardia, the effectiveness of treatment, and the results of instrumental studies. Using the method of mathematical modeling, the informative value of each feature was determined, and the diagnostic value was determined. Results: age and gender differences in the development of WPW syndrome in children were established. The presence of a family history of ECG signs of ventricular preexcitation is a reliable sign (p=0.02), contributing to more frequent PT attacks. More frequent development of PT attacks was noted in patients with mitral valve prolapse (p=0.03) and additional chords in the left ventricular cavity (p=0.001). In the group with frequent PT attacks, signs of sinus node dysfunction were detected more often (&lt;0.001). According to the echocardiographic study, children with frequent PT attacks were more likely to have disorders in the form of thickening of the interventricular septum in the diastole (p=0.010) and signs of diastolic dysfunction of the right ventricle (p = 0.010). Conclusion: The factors of prognostic value include: male gender, the presence of the expressed symptoms of the attack of PT and PT frequency more than once per month, low vagal efficiency on relieving the attack, a history of frequent attacks, a family history of ECG characteristics of premature ventricular excitation, the presence of signs of sinus node dysfunction during 24 hours without the attack and the signs of arrhythmogenic myocardial dysfunction by echocardiography.

Prophylactic Ablation in Asymptomatic Patients with Wolff-Parkinson-White Syndrome

Context: Radiofrequency ablation is a highly effective and safe therapy for the management of patients with symptomatic Wolff-Parkinson-White (WPW) syndrome. However, the management of asymptomatic patients with radiofrequency ablation is controversial. This review aimed to investigate whether prophylactic ablation could improve the long-term outcomes of asymptomatic patients with WPW. Methods: A systematic literature search was performed in databases such as PubMed, Google Scholar, and Cochrane Library for the relevant articles published during 2000 - 2020. Only original studies, cohorts, and randomized controlled trials (RCTs) performed on the WPW patients undergoing radiofrequency ablation were selected. Results: In total, six studies were reviewed, some of which suggested that the patients undergoing radiofrequency ablation had a high incidence of atrial fibrillation even after ablation. On the other hand, some of the findings indicated that ablation could reduce the mortality rate of WPW patients. In addition, two RCTs suggested that prophylactic ablation may reduce arrhythmic events in high-risk pediatric and adult patients, while one clinical trial showed no significant risk reduction for arrhythmias in the patients undergoing the prophylactic radiofrequency catheter ablation of the accessory pathways. Conclusions: Available evidence suggests that prophylactic ablation is effective in the prevention of arrhythmic events and sudden cardiac death in asymptomatic patients with WPW. However, the risk of asymptomatic patients becoming symptomatic or sudden cardiac death has been reported to be low. Therefore, only high-risk patients are recommended to undergo prophylactic ablation.

Clinical and epidemiological features of manifestations of Wolf – Parkinson – White syndrome in men of draft age with increased dysplastic stigmatization

Abstract. Clinical and epidemiological and electrocardiographic features of Wolf Parkinson White syndrome in men of military age were studied. The study was a retrospective study with a solid sample of patients. Of the 1,9056 men residents of St. Petersburg between the ages of 18 and 27, Wolf-Parkinson-White syndrome was identified in 107 (0,56%) people. The disease was asymptomatic in 38 (35,5%) patients. All identified individuals with Wolf Parkinson White syndrome, in addition to asthenic Constitution type, which was observed in 68,2% of cases, were assessed for signs of systemic connective tissue involvement. It was found that flat feet, scoliosis or kyphosis, myopia, as well as mitral valve prolapse and abnormally located chords in the left ventricle of the heart are detected in almost every second man of military age. Due to the inability to assess the presence of all signs of systemic connective tissue involvement, the isolation of specific variants of dysplastic phenotypes in patients with Wolf Parkinson White syndrome was not performed. It was found that in the presence of increased dysplastic stigmatization, the clinical course of Wolf Parkinson White syndrome is characterized by a lower number of asymptomatic cases (6,3 and 16,7%, respectively; p0,05), an increase in the frequency of paroxysmal tachyarrhythmias (81,5 and 63,1%, respectively; p0,05) and a high incidence of vegetative-vascular disorders (82,8 and 15,4%, respectively; p0,05).

The use of intravenous amiodarone in patients with atrial fibrillation and Wolff-Parkinson-White syndrome.

It was reported that intravenous amiodarone might induce ventricular fibrillation for acute treatment in patients with atrial fibrillation (AF) and Wolff-Parkinson-White (WPW) syndrome. No study was done to assess its application comprehensively in this population. This study was a retrospective analysis and undertaken by reviewing medical records and electronic databases to search for patients admitted with tachycardia resulting from WPW syndrome and AF, who have intravenously administrated amiodarone at the emergency department from January 2008 to June 2018. Thirty patients were involved in this study, of which 27 were males. The mean age of the patients was 47.8 ± 17.0 years. The mean systolic blood pressure and diastolic blood pressure were 111.9 ± 18.3mmHg and 76.1 ± 14.6mmHg, respectively. The mean heart rate was 171 (150-189) beats per minute. Half of the patients (53.3%) had no comorbidities, and only one had prior syncope. Nearly 17 patients (56.7%) started with a loading dose of 150mg. No ventricular acceleration or VF developed. The incidence of hypotension was 3.3% (1/30). Eighteen patients (60.0%) restored to sinus rhythm by amiodarone with the conversion time of 486.0 (229.0-1278.0) minutes. Intravenous amiodarone might be an alternative for acute treatment of AF and WPW syndrome in patients characterized by stable hemodynamics, relatively low admission heart rate, few comorbidities, elder age, and no prior syncope. The loading dosage of 150mg appeared to be preferred, and the maintenance period was better to less than 12hours. Monitoring and electrolyte correction were also necessary. It is essential to keep a defibrillator nearby during pharmacologic cardioversion.

Ventricular Preexcitation: An Anomalous Wave Interfering with the Ordered Ventricular Activation.

Ventricular preexcitation is a depolarization of the ventricles that occurs before the conventional sequence, and the electrocardiogram is the specific test for diagnosis. A Kent bundle is the paradigm of ventricular preexcitation, and it is associated with short PR, wide QRS and delta wave. This finding is not always very evident, as it can have different degrees of pre-eccitazione; therefore great diagnostic care must be taken in this field. If not properly identified, the pattern of ventricular preexcitation may lead to an incorrect diagnosis. The methodology of precision electrocardiology is able to confront all these aspects.

The prevalence of Wolff-Parkinson-White syndrome in newborns – results from a large general population study

Abstract Background The accessory electrical pathway in Wolff-Parkinson-White (WPW) syndrome predisposes to tachycardia and may increase risk of sudden cardiac death. Studies investigating the prevalence of this condition in newborns are few. Purpose To describe the prevalence of WPW syndrome and assess the localization of the accessory pathway and associated structural heart disease in newborns from a large general population study. Methods Electrocardiograms (ECG's) and echocardiograms of 17.489 newborns (aged 0–30 days) from a large, prospective general population study were included. WPW cases were identified through manual evaluation of outliers in measurements of PR-interval, QRS-duration, QTcB interval and QRS axis. Newborns suspected for WPW syndrome were offered a secondary echocardiogram and ECG recording. Localization of the accessory pathway was assessed based on a QRS polarity algorithm. The control group consisted of 5,000 randomly selected newborns with a normal echocardiogram. Results Among the 17,489 ECG's we manually analyzed 5,166 and found 15 newborns (80% boys) with WPW syndrome (secondary confirmatory ECG's will be available at ESC 2020) consistent with prevalence of WPW syndrome of 0.1%. The median values of the PR-interval, QRS-duration and QTcB in cases and controls were 80 vs 98 ms, 74 vs 56 ms, and 449 vs 420 ms, respectively (all p&amp;lt;0.0001). The accessory electrical pathway was left-sided in 13 (87%) of the newborns. One newborn had moderate mitral regurgitation while all other newborns had structurally normal hearts and no cases of Ebstein's anomaly. Conclusion The prevalence of WPW syndrome in our cohort was 0.1%. The syndrome was more frequent in boys, and the accessory pathways were mainly left-sided. All but one of the affected newborns had structurally normal hearts. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): This work was supported by the Danish Children Heart Foundation, Snedkermester Sophus Jacobsen and wife Astrid Jacobsen's foundation (Grant 19-R112-A5248-26048), the Research Council at Herlev-Gentofte Hospital and Toyota-Fonden, Denmark.

Bioenergetic and metabolic aberrations in induced pluripotent stem cell-derived cardiomyocytes generated from PRKAG2-mutated, WPW patient

Abstract Introduction Familial hypertrophic cardiomyopathy (HCM) is caused by over 400 mutations affecting mostly key sarcomere components, such as titin and myosin. However, HCM also results from mutations in non-structural genes such as PRKAG2 which is involved directly in a variety of bioenergetic and metabolic pathways. When the metabolic processes fail to work properly or effectively, structural and functional aberrations resulting in cardiac dysfunction can occur. Thus, mutations in the human PRKAG2 gene lead to HCM, autosomal dominant ventricular pre-excitation - Wolff-Parkinson-White syndrome (WPW), a progressive conduction system disease and vacuolar glycogen accumulation in cardiomyocytes. Purpose To investigate the hypothesis that intervening with the bioenergetic and metabolic consequences of the R302Q mutation in the PRKAG2 gene causing inherited cardiomyopathy, will attenuate the cardiac impairments. Methods We generated mutated and isogenic induced Pluripotent Stem Cell-derived cardiomyocytes (iPSC-CMs) from a WPW patient carrying the R302Q mutation. As additional control, we used healthy volunteers' iPSC-CMs. Bioenergetic (Oxygen Consumption Rate, OCR) and metabolic status were measured using the Seahorse Flux Analyzer and LC-MS, respectively. To decipher the molecular basis underlying the bioenergetic and metabolic deficits, RNA-seq analysis was performed. Results The OCR results demonstrated in PRKAG2-mutated compared to isogenic and healthy iPSC-CMs, a 2-fold increase in maximal respiration rate and a 3.75-fold increase in spare respiratory capacity, while basal OCR parameters were similar in all groups. Importantly, when treated with the AMPK activator metformin (2.5 [mM]), all the abovementioned OCR parameters were similar in the three groups. RNA-seq analysis demonstrated that of the 553 differently expressed genes (DEGs), and of the 99 DEGs mutually differentially expressed, compared to isogenic and healthy cells, the most relevant altered pathways were glycolysis, carbon metabolism, biosynthesis of amino acids, HIF-1 signaling and fructose and mannose metabolism. These findings are consistent with the LC-MS results demonstrating in PRKAG2-mutated versus isogenic and healthy iPSC-CMs, at least a 3-fold decrease in metabolites linked to the abovementioned pathways: butyryl-carnitine, creatine, docosahexaenoic acid, GMP, IMP, myristoyl-carnitine, palmitoyl-carnitine, succinyl-Cys, UMP, UTP, UDP-GlcNAc. Conclusion PRKAG2-mutated iPSC-CMs exhibit bioenergetic and metabolic aberrations, which contribute to the cardiac pathological aspects of WPW syndrome. Importantly, treatment with the AMPK activator metformin eliminated the bioenergetic abnormalities in the mutated cells, while isogenic and healthy control cells remained unaffected. Based on these novel findings, a new therapeutic modality in WPW patients may be considered. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): Israel Science Foundation (ISF)

Management of Young Athletes with Asymptomatic Preexcitation-A Review of the Literature.

Introduction: The management of young athletes with asymptomatic preexcitation remains a challenge, regardless of the progress we have made in understanding the basis of condition and developing catheter ablation procedures. The risk of sudden death, however small, yet definite, being the first symptom is determining our approach. The aim of the study was to establish the current state of knowledge regarding the management of young athletes diagnosed with asymptomatic preexcitation, by conducting a literature review. Material and methods: A comprehensive literature review was completed in accordance to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The search was limited to English language publications using the following search terms: “asymptomatic” or “incidental” and “pre-excitation” or “Wolff–Parkinson–White” or “delta wave” and “athlete” or “sport”. The search was supplemented by hand review of the bibliographies of previous relevant systematic reviews. Results: The search resulted in 85 of abstracts, and the manual search of the bibliographies resulted in 24 additional papers. After careful analysis 10 publications were included in the review. In all but one of the presented papers, the authors used non-invasive methods and then either trans-esophageal or invasive EPS as a way to risk stratify asymptomatic patients. Evidence of rapid conduction through the accessory pathway was considered high risk and prompted sport disqualification. In the analysed reports there were combined: 142 episodes of the life-threatening events (LTE)/sudden death (SCD), of which 56 were reported to occur at rest, 61 during activity and no data were available for 25. Conclusions: athletic activity may impose an increased risk of life-threatening arrhythmias in patients with asymptomatic preexcitation; hence, a separate approach could be considered, especially in patients willing to engage in high-intensity, endurance and competitive sports.

When the disease goes beyond the skin, changes in the skin can be a sign of systemic disease

Infantile hemangioma may be accompanied by malformations of internal organs and blood vessels. In 1996 PHACE syndrome was defined as a disease which is characterized by the association of segmental infantile hemangioma with localization in the head/neck region and the presence of malformations in the posterior cranial fossa, abnormalities of arterial blood vessels including coarctation of the aorta, heart defects, as well as malformations of the eyes and central nervous system. This article presents a clinical case of a child who was diagnosed this syndrome at the age of 1.5 months based on the presence of segmental hemangioma, as well as large and small criteria specific for this disease. In addition to the main symptoms, the child had an accompanying pathology: Wolff–Parkinson–White syndrome. Therapy with non-selective b-blockers and polychemotherapy allowed stopping already developed and prevent possible complications associated with this syndrome. Parents gave their consent to use information about the child, including fotos, in the article.

Effect of pulmonary vein isolation on atrial fibrillation recurrence after accessory pathway ablation in patients with Wolff-Parkinson-White syndrome.

BackgroundAlthough 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.HypothesisWhether adding pulmonary vein isolation (PVI) after successful AP ablation effectively prevents AF recurrence in patients with WPW syndrome is unknown.MethodsWe 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).ResultsDuring 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).ConclusionsPVI 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.

437 - COGNITIVE, PATHOLOGICAL, GENETIC AND NEURO-RADIOLOGICAL CORRELATES OF CEREBRAL AMYLOID ANGIOPATHY

Background:Cerebral Amyloid Angiopathy related inflammatory process (CAA-ri), a rare condition caused by an inflammatory reaction occurring within essential cerebral blood vessels against beta-amyloid deposits, leads to subclinical cognitive decline. Often misdiagnosed as dementia, this process can be treated through aggressive immunosuppression, thereby reversing much of the cognitive impairment.Case Report:We report a 69 year old female who came to the clinic for a second opinion and had received a previous diagnosis of Alzheimer’s Dementia (AD) from an outside hospital two years prior. She presented with her husband who provided some key aspects of the history. The husband reported two years of worsening of memory, while the patient denied her symptoms. Per husband and patient, she was able to perform activities of daily living (ADLs), including bathing, dressing and toileting, but had difficulties with many instrumental ADLs (IADLs). The clinical course was somewhat fluctuating with progressive cognitive symptoms and significant word-finding difficulties. Patient had been started on Donepezil 5 mg daily by her primary provider.Results:On examination, the patient did exhibit significant word-finding difficulties and scored 12/30 on the Montreal Cognitive Assessment (MoCA), indicating moderate cognitive impairment. The Patient was as well confused and disoriented to time and place. Neurological examination was otherwise unremarkable. Magnetic Resonance Imaging (MRI) studies were ordered and showed patchy and diffuse T2/FLAIR hyper intensities and particularly concentrated in the posterior cerebral artery and inferior division of the middle cerebral artery. These findings were consistent with cerebral amyloid angiopathy related inflammation (CAA-ri). Besides susceptibility weighted image (SWI) was showing multiple widely distributed microhemorrhages typical for CAA.To address the acute inflammatory reaction the patient was hospitalized and received high dose, 3 day course of intravenous steroids, followed by an oral steroid taper. The treatment had to be monitored due to an unrelated hypertensive emergency and WPW syndrome (both newly diagnosed and treated emergently) thus the Patient was hospitalized for a 3 days and discharged on oral steroids tapper in improved condition.Additionally, imaging showed that the patient’s hippocampal volumes were within normal range so this particular imaging biomarker didn’t support the diagnosis of AD. CSF biomarkers analysis didn’t support the diagnosis of AD either since had p-Tau levels were found to be within normal limits. Patient was found to be homozygous for the APOE e4 gene. Follow-up evaluation (including a repeat MRI study) was performed 2 months later showed clinical recovery and near complete resolution of diffuse hyperintensities, suggesting inflammation had resolved. Both the patient and the husband reported significant improvement in orientation and other aspects of cognition including working memory. The Patient scored 26/30 on MoCA.Discussion:Cerebral amyloid angiopathy (CAA) has been commonly associated with brain hemorrhages in the elderly, but the inflammatory subtype CAA-I occur much less frequently and may be often misdiagnosed as a cancerous process (Ronsin et al. 2016). In a recent systematic review by Caldas A et al. 2015, of the 155 patients with documented CAA-I, almost half displayed some form of cognitive impairment and 86% received corticosteroids. Nearly half of the cases improved following treatment.Conclusion:We present a case of a patient previously diagnosed with AD, upon further investigation, likely CAA-I, treated aggressively with intravenous steroids to good effect. Although rare, CAA-I is a reversible disorder that may be masked by a dementia or/and delirious process and should be considered in patients showing relatively rapid and fluctuating cognitive decline.

Признаки дисплазии соединительной ткани и ген эндотелиальной синтазы азота 3 типа (NOS3) при синдроме Вольфа–Паркинсона–Уайта

Признаки дисплазии соединительной ткани и ген эндотелиальной синтазы азота 3 типа (NOS3) при синдроме Вольфа–Паркинсона–Уайта