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Ugeskrift For Laeger Apr 2023A 63-year-old mand with permanent atrial fibrillation through five years presents to the emergency room with dyspnea and an ECG showing pre-excited afib. The ECG was...
A 63-year-old mand with permanent atrial fibrillation through five years presents to the emergency room with dyspnea and an ECG showing pre-excited afib. The ECG was initially perceived as afib with bundle branch block and treated with digoxin. After that, treatment with amiodaron was given; also without success. After DC-conversion, multiple times and relapse, the patient was transferred to a highly specialised hospital, and an accessory pathway was performed ablated. This is a case report of a patient who had permanent atrial fibrillation and whose initial presentation of Wolff-Parkinson White syndrome was pre-excited atrial fibrillation.
Topics: Humans; Middle Aged; Atrial Fibrillation; Electrocardiography; Wolff-Parkinson-White Syndrome; Heart Block
PubMed: 37114581
DOI: No ID Found -
Europace : European Pacing,... Feb 2023Accessory pathway (AP) ablation is a standard procedure for the treatment of Wolff-Parkinson-White syndrome (WPW). Twelve-lead electrocardiogram (ECG)-based delta wave...
AIMS
Accessory pathway (AP) ablation is a standard procedure for the treatment of Wolff-Parkinson-White syndrome (WPW). Twelve-lead electrocardiogram (ECG)-based delta wave analysis is essential for predicting ablation sites. Previous algorithms have shown to be complex, time-consuming, and unprecise. We aimed to retrospectively develop and prospectively validate a new, simple ECG-based algorithm considering the patients' heart axis allowing for exact localization of APs in patients undergoing ablation for WPW.
METHODS AND RESULTS
Our multicentre study included 211 patients undergoing ablation of a single manifest AP due to WPW between 2013 and 2021. The algorithm was developed retrospectively and validated prospectively by comparing its efficacy to two established ones (Pambrun and Arruda). All patients (32 ± 19 years old, 47% female) underwent successful pathway ablation. Prediction of AP-localization was correct in 197 patients (93%) (sensitivity 92%, specificity 99%, PPV 96%, and NPV 99%). Our algorithm was particularly useful in correctly localizing antero-septal/-lateral (sensitivity and specificity 100%) and posteroseptal (sensitivity 98%, specificity 92%) AP in proximity to the tricuspid valve. The accuracy of EASY-WPW was superior compared to the Pambrun (93% vs. 84%, P = 0.003*) and the Arruda algorithm (94% vs. 75%, P < 0.001*). A subgroup analysis of children (n = 58, 12 ± 4 years old, 55% female) revealed superiority to the Arruda algorithm (P < 0.001*). The reproducibility of our algorithm was excellent (ϰ>0.8; P < 0.001*).
CONCLUSION
The novel EASY-WPW algorithm provides reliable and accurate pre-interventional ablation site determination in WPW patients. Only two steps are necessary to locate left-sided AP, and three steps to determine right-sided AP.
Topics: Humans; Adult; Child; Female; Adolescent; Young Adult; Middle Aged; Male; Wolff-Parkinson-White Syndrome; Retrospective Studies; Reproducibility of Results; Catheter Ablation; Accessory Atrioventricular Bundle; Electrocardiography; Algorithms
PubMed: 36504238
DOI: 10.1093/europace/euac216 -
Ugeskrift For Laeger Feb 2023In this case report, a previously asymptomatic 11-year-old boy presented with sudden palpitations and syncope. He eventually went into cardiac arrest and was...
In this case report, a previously asymptomatic 11-year-old boy presented with sudden palpitations and syncope. He eventually went into cardiac arrest and was successfully resuscitated. The ECG showed pre-excited atrial fibrillation degenerating into pulseless ventricular tachycardia. The patient was found to have Wolff-Parkinson-White syndrome (WPW) with an accessory pathway between right atrium and ventricle which was successfully ablated. Sudden cardiac death (SCD) is rare in WPW, however, early diagnosis is essential for eliminating the risk of SCD.
Topics: Male; Humans; Child; Wolff-Parkinson-White Syndrome; Heart Arrest; Death, Sudden, Cardiac; Atrial Fibrillation; Syncope; Electrocardiography
PubMed: 36892318
DOI: No ID Found -
International Journal of Molecular... Oct 2016Hypertrophic cardiomyopathy (HCM) is mainly associated with myosin, heavy chain 7 (MYH7) and myosin binding protein C, cardiac (MYBPC3) mutations. In order to...
Hypertrophic cardiomyopathy (HCM) is mainly associated with myosin, heavy chain 7 (MYH7) and myosin binding protein C, cardiac (MYBPC3) mutations. In order to better explain the clinical and genetic heterogeneity in HCM patients, in this study, we implemented a target-next generation sequencing (NGS) assay. An Ion AmpliSeq™ Custom Panel for the enrichment of 19 genes, of which 9 of these did not encode thick/intermediate and thin myofilament (TTm) proteins and, among them, 3 responsible of HCM phenocopy, was created. Ninety-two DNA samples were analyzed by the Ion Personal Genome Machine: 73 DNA samples (training set), previously genotyped in some of the genes by Sanger sequencing, were used to optimize the NGS strategy, whereas 19 DNA samples (discovery set) allowed the evaluation of NGS performance. In the training set, we identified 72 out of 73 expected mutations and 15 additional mutations: the molecular diagnosis was achieved in one patient with a previously wild-type status and the pre-excitation syndrome was explained in another. In the discovery set, we identified 20 mutations, 5 of which were in genes encoding non-TTm proteins, increasing the diagnostic yield by approximately 20%: a single mutation in genes encoding non-TTm proteins was identified in 2 out of 3 borderline HCM patients, whereas co-occuring mutations in genes encoding TTm and galactosidase alpha (GLA) altered proteins were characterized in a male with HCM and multiorgan dysfunction. Our combined targeted NGS-Sanger sequencing-based strategy allowed the molecular diagnosis of HCM with greater efficiency than using the conventional (Sanger) sequencing alone. Mutant alleles encoding non-TTm proteins may aid in the complete understanding of the genetic and phenotypic heterogeneity of HCM: co-occuring mutations of genes encoding TTm and non-TTm proteins could explain the wide variability of the HCM phenotype, whereas mutations in genes encoding only the non-TTm proteins are identifiable in patients with a milder HCM status.
Topics: Adolescent; Adult; Aged; Arrhythmias, Cardiac; Cardiomyopathy, Hypertrophic; Child; Child, Preschool; Exons; High-Throughput Nucleotide Sequencing; Humans; Middle Aged; Mutation; Myofibrils; Phenotype; Syndrome; Young Adult
PubMed: 27600940
DOI: 10.3892/ijmm.2016.2732 -
Circulation. Arrhythmia and... Nov 2023The cause of hypertrophic cardiomyopathy (HCM) in the young is highly varied. Ventricular preexcitation (preexcitation) is well recognized, yet little is known about the...
BACKGROUND
The cause of hypertrophic cardiomyopathy (HCM) in the young is highly varied. Ventricular preexcitation (preexcitation) is well recognized, yet little is known about the specificity for any cause and the characteristics of the responsible accessory pathways (AP).
METHODS
Retrospective cohort study of patients <21 years of age with HCM/preexcitation from 2000 to 2022. The cause of HCM was defined as isolated HCM, storage disorder, metabolic disease, or genetic syndrome. Atrioventricular AP (true AP) were distinguished from fasciculoventricular fibers (FVF) using standard invasive electrophysiology study criteria. AP were defined as high risk if any of the following were <250 ms: shortest preexcited RR interval in atrial fibrillation, shortest paced preexcited cycle length, or anterograde AP effective refractory period.
RESULTS
We identified 345 patients with HCM and 28 (8%) had preexcitation (isolated HCM, 10/220; storage disorder, 8/17; metabolic disease, 5/19; and genetic syndrome, 5/89). Six (21%) patients had clinical atrial fibrillation (1 with shortest preexcited RR interval <250 ms). Twenty-two patients underwent electrophysiology study which identified 23 true AP and 16 FVF. Preexcitation was exclusively FVF mediated in 8 (36%) patients. Five (23%) patients had AP with high-risk conduction properties (including ≥1 patient in each etiologic group). Multiple AP were seen in 8 (36%) and AP plus FVF in 10 (45%) patients. Ablation was acutely successful in 13 of 14 patients with recurrence in 3. One procedure was complicated by complete heart block after ablation of a high-risk midseptal AP. There were significant differences in QRS amplitude and delta wave amplitude between groups. There were no surface ECG features that differentiated AP from FVF.
CONCLUSIONS
Young patients with HCM and preexcitation have a high likelihood of underlying storage disease or metabolic disease. Nonisolated HCM should be suspected in young patients with large QRS and delta wave amplitudes. Surface ECG is not adequate to discriminate preexcitation from a benign FVF from that secondary to potentially life-threatening AP.
Topics: Humans; Retrospective Studies; Atrial Fibrillation; Electrocardiography; Pre-Excitation Syndromes; Accessory Atrioventricular Bundle; Cardiomyopathy, Hypertrophic; Metabolic Diseases; Wolff-Parkinson-White Syndrome
PubMed: 37877314
DOI: 10.1161/CIRCEP.123.012191 -
Ugeskrift For Laeger Jan 2024Paediatric patients with ventricular pre-excitation/asymptomatic WPW syndrome have a higher risk of atrial fibrillation degenerating into ventricular fibrillation and... (Review)
Review
Paediatric patients with ventricular pre-excitation/asymptomatic WPW syndrome have a higher risk of atrial fibrillation degenerating into ventricular fibrillation and sudden cardiac death (SCD). In more than half of these patients this can be the first symptom presenting. Hence, it is important to conduct a risk stratification for SCD in asymptomatic patients with pre-excitation/delta wave in the ECGs. In this review, invasive risk stratification by electrophysiologic testing and ablation is recommended when possible. Catheter ablation is reported to have a high rate of success and low risk of complications.
Topics: Child; Humans; Atrial Fibrillation; Catheter Ablation; Death, Sudden, Cardiac; Electrocardiography; Risk; Ventricular Fibrillation; Wolff-Parkinson-White Syndrome
PubMed: 38305265
DOI: 10.61409/V08230550 -
Cleveland Clinic Journal of Medicine Jun 2015The differential diagnosis of ST-segment elevation includes four major processes: ST-segment elevation myocardial infarction (STEMI); early repolarization; pericarditis;... (Review)
Review
The differential diagnosis of ST-segment elevation includes four major processes: ST-segment elevation myocardial infarction (STEMI); early repolarization; pericarditis; and ST elevation secondary to an abnormality of the QRS complex (left bundle branch block, left ventricular hypertrophy, or preexcitation). Other processes that may be associated with ST elevation include hyperkalemia, pulmonary embolism, and Brugada syndrome. The clinical setting and specific electrocardiographic criteria often allow identification of the cause. This article reviews ST-T and QRS configurations specific to each diagnosis.
Topics: Diagnosis, Differential; Electrocardiography; Heart Diseases; Humans; Reproducibility of Results
PubMed: 26086496
DOI: 10.3949/ccjm.82a.14026 -
Cardiac Electrophysiology Clinics Mar 2015Noninvasive electrocardiographic imaging (ECGI) has been used in pediatric and congenital heart patients to better understand their electrophysiologic substrates. In... (Review)
Review
Noninvasive electrocardiographic imaging (ECGI) has been used in pediatric and congenital heart patients to better understand their electrophysiologic substrates. In this article we focus on the 4 subjects related to pediatric ECGI: (1) ECGI in patients with congenital heart disease and Wolff–Parkinson–White syndrome, (2) ECGI in patients with hypertrophic cardiomyopathy and preexcitation, (3) ECGI in pediatric patients with Wolff–Parkinson–White syndrome, and (4) ECGI for pediatric cardiac resynchronization therapy.
Topics: Adolescent; Adult; Cardiac Resynchronization Therapy; Child; Electrocardiography; Electrophysiologic Techniques, Cardiac; Female; Heart Defects, Congenital; Humans; Imaging, Three-Dimensional; Male; Young Adult
PubMed: 25722754
DOI: 10.1016/j.ccep.2014.11.006