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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 -
Scientific Reports Sep 2023Cardiac rhythm regulated by micro-macroscopic structures of heart. Pacemaker abnormalities or disruptions in electrical conduction, lead to arrhythmic disorders may be...
Cardiac rhythm regulated by micro-macroscopic structures of heart. Pacemaker abnormalities or disruptions in electrical conduction, lead to arrhythmic disorders may be benign, typical, threatening, ultimately fatal, occurs in clinical practice, patients on digitalis, anaesthesia or acute myocardial infarction. Both traditional and genetic animal models are: In-vitro: Isolated ventricular Myocytes, Guinea pig papillary muscles, Patch-Clamp Experiments, Porcine Atrial Myocytes, Guinea pig ventricular myocytes, Guinea pig papillary muscle: action potential and refractory period, Langendorff technique, Arrhythmia by acetylcholine or potassium. Acquired arrhythmia disorders: Transverse Aortic Constriction, Myocardial Ischemia, Complete Heart Block and AV Node Ablation, Chronic Tachypacing, Inflammation, Metabolic and Drug-Induced Arrhythmia. In-Vivo: Chemically induced arrhythmia: Aconitine antagonism, Digoxin-induced arrhythmia, Strophanthin/ouabain-induced arrhythmia, Adrenaline-induced arrhythmia, and Calcium-induced arrhythmia. Electrically induced arrhythmia: Ventricular fibrillation electrical threshold, Arrhythmia through programmed electrical stimulation, sudden coronary death in dogs, Exercise ventricular fibrillation. Genetic Arrhythmia: Channelopathies, Calcium Release Deficiency Syndrome, Long QT Syndrome, Short QT Syndrome, Brugada Syndrome. Genetic with Structural Heart Disease: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia, Dilated Cardiomyopathy, Hypertrophic Cardiomyopathy, Atrial Fibrillation, Sick Sinus Syndrome, Atrioventricular Block, Preexcitation Syndrome. Arrhythmia in Pluripotent Stem Cell Cardiomyocytes. Conclusion: Both traditional and genetic, experimental models of cardiac arrhythmias' characteristics and significance help in development of new antiarrhythmic drugs.
Topics: Humans; Animals; Guinea Pigs; Dogs; Anti-Arrhythmia Agents; Ventricular Fibrillation; Calcium; Atrial Fibrillation; Papillary Muscles; Models, Animal
PubMed: 37775650
DOI: 10.1038/s41598-023-41942-4 -
Journal of Cardiovascular Medicine... Sep 2023Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular... (Review)
Review
Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular tachycardia (PSVT). These arrhythmias are commonly observed in pediatric age. PSVT may occur at any age, from fetus to adulthood, and its symptoms range from none to syncope or heart failure. VP too can range from no symptoms to sudden cardiac death. Therefore, these arrhythmias frequently need risk stratification, electrophysiologic study, drug or ablation treatment. In this review of the literature, recommendations are given for diagnosis and treatment of fetal and pediatric age (≤12 years) WPW, VP, PSVT, and criteria for sport participation.
Topics: Humans; Child; Infant, Newborn; Infant; Child, Preschool; Wolff-Parkinson-White Syndrome; Accessory Atrioventricular Bundle; Electrocardiography; Tachycardia, Paroxysmal; Tachycardia, Ventricular; Fetus
PubMed: 37409656
DOI: 10.2459/JCM.0000000000001484 -
Cureus Oct 2023Wolff-Parkinson-White (WPW) syndrome is a congenital cardiac preexcitation syndrome that arises from abnormal cardiac electrical conduction through an accessory pathway... (Review)
Review
Wolff-Parkinson-White (WPW) syndrome is a congenital cardiac preexcitation syndrome that arises from abnormal cardiac electrical conduction through an accessory pathway and results in symptomatic and life-threatening arrhythmias. The aim of this study is to analyze the patterns of gender representation among first-author publications concerning "Wolf-Parkinson-White syndrome" within the PubMed-indexed publications from "January 1, 1973, to December 31, 2022," based on country and year. On May 9, 2023, bibliometric analysis was performed. The phrase "(Wolf-Parkinson-White Syndrome)" was looked up in PubMed. It covered articles released between January 1, 1973, and December 31, 2022. Articles accepted in the year 2022 and published in Pubmed in 2023 were included in the study. A total of 138 articles were considered and included in our analysis. Among these articles, 29 (21.01%) were authored by females, while 109 (78.99%) were authored by males. To conclude, this research study reveals a rising trend of females in lead authorship roles within the field of cardiac arrhythmia research. However, it remains evident that there is a significant gender gap, with male researchers still outnumbering their female counterparts.
PubMed: 38022330
DOI: 10.7759/cureus.47208 -
Europace : European Pacing,... Jul 2023Wolff-Parkinson-White (WPW) syndrome is a conduction disorder characterized by an accessory electrical pathway between the atria and ventricles, which may predispose to... (Clinical Trial)
Clinical Trial
AIMS
Wolff-Parkinson-White (WPW) syndrome is a conduction disorder characterized by an accessory electrical pathway between the atria and ventricles, which may predispose to supraventricular tachycardia (SVT) and sudden cardiac death. It can be seen as an isolated finding or associated with structural heart disease. Our aims were to determine the prevalence of a WPW pattern in a large and unselected cohort of neonates and to describe the electro- and echocardiographic characteristics as well as the natural history during early childhood.
METHODS AND RESULTS
Electrocardiograms and echocardiograms of neonates (aged 0-30 days) from a large, prospective, population-based cohort study were included. Neonates with a WPW pattern were identified and matched 1:4 to controls. Localization of the accessory pathway was assessed by different algorithms. Among 17 489 neonates, we identified 17 (76% boys) with a WPW pattern consistent with a prevalence of 0.1%. One neonate had moderate mitral regurgitation while other echocardiographic parameters were similar between cases and controls (all P > 0.05). The accessory pathways were primarily predicted to be left-sided. At follow-up (available in 14/17 children; mean age 3.2 years) the pre-excitation pattern persisted in only four of the children and none of the children had experienced any episodes of SVT.
CONCLUSION
The prevalence of a WPW pattern in our cohort of unselected neonates was 0.1%. The WPW pattern was more frequent in boys and generally not associated with structural heart disease, and the accessory pathways were primarily left-sided. At follow-up, the WPW pattern had disappeared in most of the children suggesting either an intermittent nature or that normalization occurs.
CLINICAL TRIAL REGISTRATION
Copenhagen Baby Heart, NCT02753348.
Topics: Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Accessory Atrioventricular Bundle; Cohort Studies; Electrocardiography; Heart Diseases; Prospective Studies; Tachycardia, Supraventricular; Wolff-Parkinson-White Syndrome
PubMed: 37465966
DOI: 10.1093/europace/euad165 -
BMC Cardiovascular Disorders Aug 2023Danon disease (DD) is an exceptionally uncommon X-linked dominant lysosomal glycogen storage disorder characterized by pronounced ventricular hypertrophy and cardiac...
BACKGROUND
Danon disease (DD) is an exceptionally uncommon X-linked dominant lysosomal glycogen storage disorder characterized by pronounced ventricular hypertrophy and cardiac insufficiency. The timely identification of cardiac impairment in individuals with DD holds significant clinical importance.
CASE PRESENTATION
We present a case of Danon Disease in a three-generation pedigree from Anhui Province, China. Clinical features and laboratory data were collected and analyzed for a 16-year-old male proband (III-1) and two affected female family members (II-2 and II-3). The proband exhibited Wolf-Parkinson-White syndrome, hypertrophic cardiomyopathy, abnormal cognitive function, and muscle weakness. Gene sequencing confirmed a mutation (c.963G > A) in the LAMP-2 gene.
CONCLUSION
Patients with DD may present both dilated and hypertrophic cardiomyopathy. Comprehensive myocardial tissue characterization by MRI plays a key role in the diagnosis of the disease.
Topics: Male; Female; Humans; Glycogen Storage Disease Type IIb; Cardiomyopathy, Hypertrophic; Mutation; Wolff-Parkinson-White Syndrome; Magnetic Resonance Imaging
PubMed: 37568080
DOI: 10.1186/s12872-023-03356-y -
Europace : European Pacing,... Aug 2023This review article reflects how publications in EP Europace have contributed to advancing the science of management of arrhythmic disease in children and adult patients... (Review)
Review
This review article reflects how publications in EP Europace have contributed to advancing the science of management of arrhythmic disease in children and adult patients with congenital heart disease within the last 25 years. A special focus is directed to congenital atrioventricular (AV) block, the use of pacemakers, cardiac resynchronization therapy devices, and implantable cardioverter defibrillators in the young with and without congenital heart disease, Wolff-Parkinson-White syndrome, mapping and ablation technology, and understanding of cardiac genomics to untangle arrhythmic sudden death in the young.
Topics: Adult; Humans; Child; Heart Defects, Congenital; Heart; Wolff-Parkinson-White Syndrome; Cardiac Resynchronization Therapy Devices; Death, Sudden
PubMed: 37622573
DOI: 10.1093/europace/euad155