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Heart Rhythm Mar 2022Altered ventricular depolarization due to manifest accessory pathway conduction (ie, Wolff-Parkinson-White syndrome) leads to repolarization abnormalities that persist...
BACKGROUND
Altered ventricular depolarization due to manifest accessory pathway conduction (ie, Wolff-Parkinson-White syndrome) leads to repolarization abnormalities that persist after pathway ablation. The term T-wave memory (TWM) has been applied to these changes, as the postablation T-wave vector "remembers" the pre-excited QRS vector. In adults, these abnormalities can be misinterpreted as ischemia leading to unnecessary interventions. To date, no comprehensive studies have evaluated this phenomenon in the pediatric population.
OBJECTIVE
The purpose of this study was to define TWM in the pediatric population, identify preablation risk factors, and delineate the timeline of recovery.
METHODS
Pre- and postablation electrocardiograms (ECGs) in patients ≤25 years were analyzed over a 5-year period. Frontal plane QTc interval, T-wave axis, QRST angle, and T-wave inversions were used to identify patients with TWM. Univariate analysis was performed to determine the association of preablation ECG features with the outcome of TWM.
RESULTS
TWM 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.
CONCLUSION
TWM is less common in children compared to adults, and normalization occurred within 3 months postablation. The most predictive features for the development of TWM include a leftward pre-excited QRS axis and posteroseptal pathway location.
Topics: Accessory Atrioventricular Bundle; Adult; Catheter Ablation; Child; Electrocardiography; Heart Conduction System; Humans; Pre-Excitation Syndromes; Wolff-Parkinson-White Syndrome
PubMed: 34767987
DOI: 10.1016/j.hrthm.2021.11.007 -
Journal of Medical Genetics Mar 2009Wolff-Parkinson-White syndrome (WPW) is a bypass re-entrant tachycardia that results from an abnormal connection between the atria and ventricles. Mutations in PRKAG2...
BACKGROUND
Wolff-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 RESULTS
Using 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.
CONCLUSIONS
Our 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.
Topics: Adult; Alagille Syndrome; Animals; Bone Morphogenetic Protein 2; Calcium-Binding Proteins; Cognition Disorders; Comparative Genomic Hybridization; Electrocardiography; Facies; Female; Gene Dosage; Humans; Infant; Intercellular Signaling Peptides and Proteins; Jagged-1 Protein; Male; Membrane Proteins; Mice; Mice, Transgenic; Oligonucleotide Array Sequence Analysis; Sequence Deletion; Serrate-Jagged Proteins; Wolff-Parkinson-White Syndrome
PubMed: 18812404
DOI: 10.1136/jmg.2008.061002 -
The Journal of Biological Chemistry Nov 2016Mutations of the AMP-activated kinase gamma 2 subunit (AMPKγ2), N488I (AMPKγ2) and R531G (AMPKγ2), are associated with Wolff-Parkinson-White (WPW) syndrome, a cardiac...
Mutations of the AMP-activated kinase gamma 2 subunit (AMPKγ2), N488I (AMPKγ2) and R531G (AMPKγ2), are associated with Wolff-Parkinson-White (WPW) syndrome, a cardiac disorder characterized by ventricular pre-excitation in humans. Cardiac-specific transgenic overexpression of human AMPKγ2 or AMPKγ2 leads to constitutive AMPK activation and the WPW phenotype in mice. However, overexpression of these mutant proteins also caused profound, non-physiological increase in cardiac glycogen, which might abnormally alter the true phenotype. To investigate whether physiological levels of AMPKγ2 or AMPKγ2 mutation cause WPW syndrome and metabolic changes in other organs, we generated two knock-in mouse lines on the C57BL/6N background harboring mutations of human AMPKγ2 and AMPKγ2, respectively. Similar to the reported phenotypes of mice overexpressing AMPKγ2 or AMPKγ2 in the heart, both lines developed WPW syndrome and cardiac hypertrophy; however, these effects were independent of cardiac glycogen accumulation. Compared with AMPKγ2 mice, AMPKγ2 and AMPKγ2 mice exhibited reduced body weight, fat mass, and liver steatosis when fed with a high fat diet (HFD). Surprisingly, AMPKγ2 but not AMPKγ2 mice fed with an HFD exhibited severe kidney injury characterized by glycogen accumulation, inflammation, apoptosis, cyst formation, and impaired renal function. These results demonstrate that expression of AMPKγ2 and AMPKγ2 mutations at physiological levels can induce beneficial metabolic effects but that this is accompanied by WPW syndrome. Our data also reveal an unexpected effect of AMPKγ2 in the kidney, linking lifelong constitutive activation of AMPK to a potential risk for kidney dysfunction in the context of an HFD.
Topics: AMP-Activated Protein Kinases; Animals; Apoptosis; Disease Models, Animal; Gene Knock-In Techniques; Inflammation; Kidney; Male; Mice, Inbred C57BL; Mutation; Renal Insufficiency; Wolff-Parkinson-White Syndrome
PubMed: 27621313
DOI: 10.1074/jbc.M116.738591 -
Anatolian Journal of Cardiology Sep 2018The use of fluoroscopy in pediatric catheter ablation has decreased because of mapping systems. In this study, we present the efficiency and reliability of the...
OBJECTIVE
The use of fluoroscopy in pediatric catheter ablation has decreased because of mapping systems. In this study, we present the efficiency and reliability of the electroanatomic mapping system in nonfluoroscopic pediatric catheter ablation.
METHODS
The medical records of patients aged <18 years who underwent ablation between November 2016 and April 2018 were evaluated. Fluoroscopy was not used in cases involving ablation of right sided-arrhythmia foci. Fluoroscopy was used only for trans-septal puncture or retroaortic approach/coronary angiography.
RESULTS
A total of 76 patients underwent catheter ablation for 78 supraventricular and ventricular tachyarrhythmia substrates under the guidance of EnSite Velocity system. Fluoroscopy was used in only 14 (18.4%) of these substrates. The mean fluoroscopy duration in these 14 procedures was 5.4±3.15 min. No complications were noted, except a temporary right bundle branch block in one patient and pericardial effusion in another following cryoablation. The acute success rate in achieving complete elimination of arrhythmia substrates was 97.4% (76/78). The recurrence rate was 5.1% (4/78) at follow-up.
CONCLUSION
Fluoroscopy can be completely eliminated in most pediatric catheter ablation procedures with the use of mapping systems by achieving high acute success rates and acceptable low complication rates.
Topics: Adolescent; Body Surface Potential Mapping; Catheter Ablation; Child; Cryosurgery; Electrophysiologic Techniques, Cardiac; Female; Fluoroscopy; Humans; Male; Pre-Excitation Syndromes; Radiation Exposure; Tachycardia, Supraventricular; Tachycardia, Ventricular; Treatment Outcome
PubMed: 30152797
DOI: 10.14744/AnatolJCardiol.2018.72687 -
Arquivos Brasileiros de Cardiologia Jun 2019
Topics: Adolescent; Craniofacial Abnormalities; Female; Humans; Wolff-Parkinson-White Syndrome
PubMed: 31188966
DOI: 10.5935/abc.20190033 -
Europace : European Pacing,... Feb 2023
Topics: Humans; Microelectrodes; Heart Conduction System; Atrioventricular Node; Bundle of His; Catheter Ablation; Electrocardiography; Wolff-Parkinson-White Syndrome
PubMed: 36634051
DOI: 10.1093/europace/euac271 -
Clinical Cardiology Jun 1993Metastatic tumor involvement of the heart as a basis for the genesis of various cardiac arrhythmias is reviewed. The morphologic basis of pre-excitation syndromes and... (Review)
Review
Metastatic tumor involvement of the heart as a basis for the genesis of various cardiac arrhythmias is reviewed. The morphologic basis of pre-excitation syndromes and right ventricular dysplasia are also reviewed.
Topics: Arrhythmias, Cardiac; Atrioventricular Node; Heart Conduction System; Heart Neoplasms; Humans; Pre-Excitation Syndromes
PubMed: 8358886
DOI: 10.1002/clc.4960160611 -
Journal of the American College of... Nov 1990
Topics: Adolescent; Anti-Arrhythmia Agents; Child; Child, Preschool; Humans; Infant; Recurrence; Tachycardia, Supraventricular; Wolff-Parkinson-White Syndrome
PubMed: 2229770
DOI: 10.1016/0735-1097(90)90556-5 -
Journal of the American College of... Oct 1990
Topics: Electrocardiography; Heart Conduction System; Humans; Pre-Excitation Syndromes; Wolff-Parkinson-White Syndrome
PubMed: 2278570
DOI: 10.1016/s0735-1097(10)80360-2 -
Herzschrittmachertherapie &... Mar 2024The history of surgical treatment of ventricular tachycardias (VT) is short, lasting from 1978 until 1993. "Indirect procedures" with infarct scar resection were... (Review)
Review
[History of surgical treatment of cardiac arrhythmias in Germany : Surgical treatment of ventricular tachycardia and supraventricular tachycardia, especially pre-excitation syndromes (WPW)].
The history of surgical treatment of ventricular tachycardias (VT) is short, lasting from 1978 until 1993. "Indirect procedures" with infarct scar resection were performed without electrophysiologic studies, whereas "direct procedures" consisted of either complete endocardial incisions ("encircling endocardial ventriculotomy") or large endocardial resections ("endocardial peel-off" technique) after precise epicardial and endocardial mapping procedures. In Germany, the first to report on intra-operative electrophysiologic mapping for VT treatment were Ostermeyer, Breithardt and Seipel in 1979. In 1981, the Hannover group (Frank, Klein) published their first results of surgical treatment of VT. In 1984, Ostermeyer et al. demonstrated that a partial endocardial incision resulted in more beneficial results with less myocardial damage (8% versus 46%) than applying a complete encircling incision. In 1987, the Düsseldorf group reported treatment results of 93 patients. After 5 years, 77% had no VT recurrence, while total mortality after 1 year was 11% and after 5 years 30%. In 1992, the Hannover group reported results of 147 patients after endocardial resection for VT. Total mortality after 3 years was 27%; recurrence of VT events occurred in 18% of the surviving cohort.The history of surgical procedures for supraventricular tachycardia (SVT), in particular Wolff-Parkinson-White (WPW) syndrome, is even shorter than that of surgery for VT. As early as 1969, Sealy, Gallagher and Cox reported the first cases of surgical intervention for WPW syndrome via endocardial access in cardioplegic arrest. In 1984, Guiraudon and Klein reported on a new procedure with epicardial access to the accessory bundle without cardioplegia in laterally localised conduction pathways. In Germany, too, the groups in Düsseldorf (Ostermeyer, Seipel, Breithardt, Borggrefe) from 1980 and the Hannover group (Frank, Klein and Kallfelz) from 1981 performed surgical procedures for WPW syndrome. In 1987, Borggrefe reported on 18 patients with WPW syndrome and atrial fibrillation who had undergone surgery. After 2 years, 14 of 18 patients had no recurrences of tachycardia; in 1989, Frank, Klein and Kallfelz (Hannover) reported on 10 children (2-14 years) operated on using the cryoablation technique. Between 1984 and 1992, a total of 120 patients with SVT, mostly WPW syndrome, were operated on in Hannover; after 42 months, 12 patients had a recurrence of SVT. Two patients died during the reoperation.
Topics: Child; Humans; Wolff-Parkinson-White Syndrome; Tachycardia, Supraventricular; Tachycardia, Ventricular; Pre-Excitation Syndromes; Atrial Fibrillation
PubMed: 38416160
DOI: 10.1007/s00399-024-01012-2