Atrial fibrillation associated with Wolff-Parkinson-White syndrome in a patient with concomitant Brugada syndrome.
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ABSTRACT:
SUBMITTER: Shi S
PROVIDER: S-EPMC5420038 | biostudies-literature | 2017 Jan
REPOSITORIES: biostudies-literature
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Atrial fibrillation associated with Wolff-Parkinson-White syndrome in a patient with concomitant Brugada syndrome.
Shi Shaobo S Liu Tao T Barajas-Martinez Hector H Pfeiffer Ryan R Jiang Hong H Huang Congxin C Hu Dan D
HeartRhythm case reports 20170103 1
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Project description:BackgroundWolff-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.MethodsWe 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.ResultsA 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).ConclusionsOur 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.
| S-EPMC7275694 | biostudies-literature
Project description:Background: In recent years, Wolff-Parkinson-White (WPW) syndrome and Brugada electrocardiogram (ECG) patterns have been reported as coexistent in the same patient. In most cases, the two waveforms appeared separately. Here, we described combinations of different waveforms on one ECG, such as the Brugada pattern with delta waves and the Brugada pattern with paroxysmal supraventricular tachycardia (PSVT). Importantly, we recorded an alternate conversion of these combined ECG waveforms, which has not previously been reported in the literature. At the same time, we confirmed that the change in the waveform was related to fever by analyzing Holter data. Case: A 48-year-old male was admitted to our hospital due to palpitations and fever. The patient had a history of a cold 3 days ago. Laboratory examinations showed an elevated neutrophil percentage (85%) and troponin I level (0.86 ng/ml). A chest computed tomography (CT) scan showed inflammation in the right lung. The diagnosis of pneumonia and myocarditis was made. ECG indicated WPW syndrome and the Brugada pattern. We recorded the dynamic changes in this combination of delta waves and Brugada waves with a Holter monitor, and we found the changes would happen when the patient's body temperature rose. The doctors thought that the patient's pulmonary infection led to fever, which caused the changes in waveform. After treatment with antibacterial therapy and supportive care, his body temperature returned to normal. The various laboratory indicators also gradually returned to normal. The doctor recommended that the patient undergo further pre-excitation bypass radiofrequency ablation treatment, but the patient refused and was discharged. Conclusion: Delta waves and Brugada ECG patterns could appear on one ECG at the same time. There were dynamic changes of QRS complex, relating to fever.
| S-EPMC8566699 | biostudies-literature
Project description:Congenital right atrial aneurysms (RAA) have a wide range of clinical presentations and leads to various complications. Depending on the initial presentation and associated complications, a conservative or surgical approach may be considered. A patient suffering from a giant RAA associated with the Wolff-Parkinson-White syndrome, who underwent successful surgical treatment, is presented here.
| S-EPMC9014696 | biostudies-literature
Project description:While Drs Wolff, Parkinson, and White fully described the syndrome in 1930, prior case reports had described the essentials. Over the ensuing century this syndrome has captivated the interest of anatomists, clinical cardiologists, and cardiac surgeons. Stanley Kent described lateral muscular connections over the atrioventricular (AV) groove which he felt were the normal AV connections. The normal AV connections were, however, clearly described by His and Tawara. True right-sided AV connections were initially described by Wood et al., while Öhnell first described left free wall pathways. David Scherf is thought to be the first to describe our current understanding of the pathogenesis of the WPW syndrome in terms of a re-entrant circuit involving both the AV node-His axis as well as the accessory pathway. This hypothesis was not universally accepted, and many theories were applied to explain the clinical findings. The basics of our understanding were established by the brilliant work of Pick, Langendorf, and Katz who by using careful deductive analysis of ECGs were able to define the basic pathophysiological processes. Subsequently, Wellens and Durrer applied invasive electrical stimulation to the heart in order to confirm the pathophysiological processes. Sealy and his colleagues at Duke University Medical Center were the first to successfully surgically divide an accessory pathway and ushered in the modern era of therapy for these patients. Morady and Scheinman were the first to successfully ablate an accessory pathway (posteroseptal) using high-energy direct-current shocks. Subsequently Jackman, Kuck, Morady, and a number of groups proved the remarkable safety and efficiency of catheter ablation for pathways in all locations using radiofrequency energy. More recently, Gollob et al. first described the gene responsible for a familial form of WPW. The current ability to cure patients with WPW is due to the splendid contributions of individuals from diverse disciplines throughout the world.
| S-EPMC3678820 | biostudies-other
Project description:BackgroundPatients with an accessory pathway (AP) have an increased propensity to develop atrial fibrillation (AF), but the mechanism is unknown.ObjectiveThe purpose of this study was to identify crucial risk factors and to test the hypothesis that reflection and/or microreentry of atrial impulses propagating into the AP triggers AF.MethodsFive hundred thirty-four patients successfully treated with radiofrequency ablation of AP at two university hospitals were evaluated. Patients were separated into those with concealed vs those with manifest AP in terms of their propensity to develop AF. To investigate AF triggering mechanisms, linear and branched two-dimensional models of atrium-to-ventricle propagation across a heterogeneous 1 x 6 AP using human ionic kinetics were simulated.ResultsA history of AF was twice as common in patients with manifest AP vs concealed AP irrespective of AP location. AF was more likely to occur in older males and in patients with larger atria. There was no correlation between AF history and AP refractory measures. However, the electrophysiologic properties of APs seemed to fulfill the prerequisites for reflection and/or microreentry of atrially initiated impulses. In the linear AP model, repetitive atrial stimulation resulted in progressively larger delay of atrium-to-ventricle propagation across the passive segment. Eventually, sufficient time for repolarization of the atrial segment allowed for reflection of an impulse that activated the entire atrium and by wavefront-wavetail interaction with a new atrial stimulus AF reentry was initiated. Simulations using the branched model showed that microreentry at the ventricular insertion of the AP could also initiate AF via retrograde atrial activation as a result of unidirectional block at the AP-ventricle junction.ConclusionPropensity for AF in patients with an AP is strongly related to preexcitation, larger atria, male gender, and older age. Reflection and microreentry at the AP may be important for AF initiation in patients with manifest (preexcited) Wolff-Parkinson-White syndrome. Similar mechanisms also may trigger AF in patients without an AP.
| S-EPMC2597634 | biostudies-literature
Project description:BackgroundWolff-Parkinson-White syndrome (WPW) is a bypass re-entrant tachycardia that results from an abnormal connection between the atria and ventricles. Mutations in PRKAG2 have been described in patients with familial WPW syndrome and hypertrophic cardiomyopathy. Based on the role of bone morphogenetic protein (BMP) signalling in the development of annulus fibrosus in mice, it has been proposed that BMP signalling through the type 1a receptor and other downstream components may play a role in pre-excitation.Methods and resultsUsing the array comparative genomic hybridisation (CGH), we identified five individuals with non-recurrent deletions of 20p12.3. Four of these individuals had WPW syndrome with variable dysmorphisms and neurocognitive delay. With the exception of one maternally inherited deletion, all occurred de novo, and the smallest of these harboured a single gene, BMP2. In two individuals with additional features of Alagille syndrome, deletion of both JAG1 and BMP2 were identified. Deletion of this region has not been described as a copy number variant in the Database of Genomic Variants and has not been identified in 13 321 individuals from other cohort examined by array CGH in our laboratory.ConclusionsOur findings demonstrate a novel genomic disorder characterised by deletion of BMP2 with variable cognitive deficits and dysmorphic features and show that individuals bearing microdeletions in 20p12.3 often present with WPW syndrome.
| S-EPMC2680125 | biostudies-literature
Project description:Wolff-Parkinson-White syndrome (WPW) is rarely seen in association with atrioventricular septal defect. Although paroxysm's of palpitation due to supraventricular tachycardia can occur in these patients, rare, fatal, ventricular dysrhythmias can also occur. Herein, we report the case of a 20-year-old male patient with partial atrioventricular septal defect and WPW syndrome for intracardiac repair, developing intraoperative Torsades de pointes and postoperative cardiac arrest, adding to the difficulty in overall patient management.
| S-EPMC8617382 | biostudies-literature
Project description:BackgroundAtrial fibrillation in Wolff-Parkinson-White syndrome may result in life-threateningly rapid antegrade conduction over a bypass tract, manifested by an irregular broad-complex (pre-excited) tachycardia that can degenerate to ventricular fibrillation. The shortest pre-excited RR interval below 250 ms during atrial fibrillation (AF) predicts increased risk of sudden cardiac death.Case summaryWe report a case of a 43-year-old man with unremarkable cardiac history who presented due to sudden-onset feeling of palpitations and pre-syncope after strenuous lifting. Electrocardiography depicted fast pre-excited AF. The shortest pre-excited RR interval was estimated at 160 ms, indicating an accessory pathway (AP) with short antegrade refractory period at risk for mediating sudden cardiac death. Direct current cardioversion restored sinus rhythm unravelling delta waves. The patient was put on propafenone 450 mg/day having an uneventful clinical course. On Day 10 post-admission, electrophysiological study induced rapid AF but the shortest pre-excited RR interval was substantially increased to 264 ms. A left anterolateral AP was ablated. The patient remained symptom free until his latest follow-up in the 3rd-month post-ablation without manifest pre-excitation on the surface electrocardiogram.DiscussionTreatment options of pre-excited AF include anti-arrhythmic agents but mainly electrical cardioversion. Cardioversion can safely restore sinus rhythm, while use of anti-arrhythmics often requires intensive care unit monitoring due to the risk of QT prolongation. Catheter ablation is the mainstay of therapy for symptomatic patients. Our rare report highlights the direct impact of propafenone on prolonging the refractoriness of the AP, effectively and safely, and reappraises propafenone's worthiness as a protective measure following pre-excited AF episode until ablation.
| S-EPMC8665683 | biostudies-literature
Project description:BackgroundAltered 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.ObjectiveThe purpose of this study was to define TWM in the pediatric population, identify preablation risk factors, and delineate the timeline of recovery.MethodsPre- 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.ResultsTWM was present in 42% of pediatric patients, with resolution occurring within 3 months of ablation. Preablation QRS axis <0° 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.ConclusionTWM 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.
| S-EPMC9026902 | biostudies-literature
Project description:Several studies have shown an overlap between genes involved in the pathophysiological mechanisms of atrial fibrillation (AF) and Brugada Syndrome (BrS). We investigated whether three single-nucleotide polymorphisms (SNPs) (rs11708996; G>C located intronic to SCN5A, rs10428132; T>G located in SCN10A, and rs9388451; T>C located downstream to HEY2) at loci associated with BrS in a recent genome-wide association study (GWAS) also were associated with AF. A total of 657 patients diagnosed with AF and a control group comprising 741 individuals free of AF were included. The three SNPs were genotyped using TaqMan assays. The frequencies of risk alleles in the AF population and the control population were compared in two-by-two models. One variant, rs10428132 at SCN10A, was associated with a statistically significant decreased risk of AF (odds ratio (OR)=0.77, P=0.001). A meta-analysis was performed by enriching the control population with allele frequencies from controls in the recently published BrS GWAS (2230 alleles). In this meta-analysis, both rs10428132 at SCN10A (OR=0.73, P=5.7 × 10(-6)) and rs11708996 at SCN5A (OR=0.80, P=0.02) showed a statistically significant decreased risk of AF. When assessing the additive effect of the three loci, we found that the risk of AF decreased in a dose-responsive manner with increasing numbers of risk alleles (OR=0.50, P=0.001 for individuals carrying ≥4 risk alleles vs ≤1 allele). In conclusion, the prevalence of three risk alleles previously associated with BrS was lower in AF patients than in patients free of AF, suggesting a protective role of these loci in developing AF.
| S-EPMC4231412 | biostudies-literature