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European Review For Medical and... Sep 2023Our study aimed at analyzing the echocardiographic multi-indicator evaluation of the risk of Wolff-Parkinson-White syndrome (WPW) on the left ventricular function and...
Two-dimensional speckle tracking imaging to assess the hazards of left ventricular function and ventricular wall motion disorders in children with pre-excitation syndrome and the efficacy of radiofrequency ablation treatment.
OBJECTIVE
Our study aimed at analyzing the echocardiographic multi-indicator evaluation of the risk of Wolff-Parkinson-White syndrome (WPW) on the left ventricular function and ventricular wall motion disorders, as well as the effect of radiofrequency ablation treatment.
PATIENTS AND METHODS
The clinical data of 55 WPW patients treated with radiofrequency (RF) ablation at the Children's Hospital of Nanjing Medical University between January 2018 and December 2022 were retrospectively analyzed and included in the observation group, while other 50 healthy children were included in the control group during the same time. We analyzed the echocardiographic indices of the patients, assessed the effects of the disease on left ventricular myocardial function and ventricular wall motion disorders, and evaluated the effects of radiofrequency ablation treatment on the myocardium of the left ventricle. The echocardiographic parameters were analyzed to assess the effect of the disease on left ventricular myocardial function and ventricular wall dyskinesia.
RESULTS
Of the 55 patients with pre-excited syndrome, 20 had type A bypass and 35 had type B bypass. Ten patients had pre-excited dilated cardiomyopathy with significant enlargement of the left ventricular cavity, reduced left ventricular systolic function, and a significant impairment of ventricular wall motion; the other 5 patients had basal segmental septal motion incoordination. Compared to the control group, patients with left ventricular end-diastolic diameter (LVEDD) (42.9±5.0 mm vs. 39.2±3.0 mm), peak strain dispersion (PSD) (38.8±15.3 ms vs. 21.7±2.2 ms), maximum peak time difference (MPTD) (200.2±92.8 ms vs. 89.5±9.8 ms) and interventricular mechanical delay (IVMD) (36.2±13.7 ms vs. 21.2±2.1 ms) before RF ablation were increased. Left ventricular ejection fraction (LVEF) (57.1±9.1% vs. 65.9±2.6%), E/A (1.1±0.2 vs. 1.8±0.2) and global longitudinal strain (GLS) (-18.7±2.2% vs. -22.4±0.5%) decreased, with statistically significant differences (p<0.05). All 55 patients had a successful procedure, and all postoperative echocardiographic parameters were found to be improved, compared to the preoperative period. The results of the postoperative review after 3 months showed differences in E/A, PSD, MPTD, and IVMD compared to the healthy group, suggesting that left ventricular diastolic function and synchrony had not fully returned to normal.
CONCLUSIONS
Echocardiography can better evaluate myocardial motion and function in patients with Wolff-Parkinson-White syndrome and monitor the effect and progress of disease treatment, and has high clinical application value.
Topics: Humans; Child; Wolff-Parkinson-White Syndrome; Ventricular Function, Left; Stroke Volume; Heart Ventricles; Retrospective Studies; Pre-Excitation Syndromes; Echocardiography
PubMed: 37750616
DOI: 10.26355/eurrev_202309_33545 -
Genes Jul 2023Danon disease is a rare x-linked dominant multisystemic disorder with a clinical triad of severe cardiomyopathy, skeletal myopathy, and intellectual disability. It is... (Review)
Review
Danon disease is a rare x-linked dominant multisystemic disorder with a clinical triad of severe cardiomyopathy, skeletal myopathy, and intellectual disability. It is caused by defects in the lysosome-associated membrane protein-2 () gene. Numerous different mutations in the protein have been described. Danon disease is typically lethal by the mid-twenties in male patients due to cardiomyopathy and heart failure. Female patients usually present with milder and variable symptoms. This report describes a 42-year-old father and his 3-year-old daughter presenting with mild manifestations of the disease. The father has normal intellectual development and normal physical activity. At the age of 13, he was diagnosed with mild ventricular pre-excitation known as Wolf-Parkinson-White syndrome (WPWs), very mild and mostly asymptomatic cardiomyopathy and left ventricular hypertrophy, and at about the age of 25 presented with visual impairment due to cone-rod dystrophy. His daughter showed normal development and very mild asymptomatic electrocardiographic WPWs abnormalities with left mild ventricular hypertrophy. Genetic testing revealed an Xq24 microdeletion encompassing the entire gene. Relevant literature was reviewed as a reference for the etiology, diagnosis, treatment and case management.
Topics: Female; Male; Humans; Glycogen Storage Disease Type IIb; Gene Deletion; Genes, Regulator; Heart Failure; Cone-Rod Dystrophies; Lysosomal-Associated Membrane Protein 2
PubMed: 37628591
DOI: 10.3390/genes14081539 -
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 -
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,... 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 -
Singapore Medical Journal Jul 2023
Topics: Humans; Wolff-Parkinson-White Syndrome; Electrocardiography; Athletes
PubMed: 37459000
DOI: 10.4103/singaporemedj.SMJ-2021-151 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Jul 2023Wolff-Parkinson-White syndrome is rarely associated with a right atrial aneurysm. However, when such a condition occurs, it will be hard to manage since pre-excitation...
Wolff-Parkinson-White syndrome is rarely associated with a right atrial aneurysm. However, when such a condition occurs, it will be hard to manage since pre-excitation will be induced as long as the aneurysm persists. A 14-year-old female patient received emergency treatment for irregular wide QRS complex tachycardia in our center, and a pre-excitation pattern was then observed on the surface electrocardiogram. An initial electrophysiological study revealed a high-risk right posterior accessory pathway that was resistant to both radiofrequency and irrigated radiofrequency ablations. Subsequently, fluoroscopy showed that this was due to a right atrial aneurysm. Although successful ablation with irrigated radiofrequency was performed in the second procedure, the procedure was considered suboptimal due to the association of aneurysm. Accordingly, we initiated anti-thrombotic and anti-arrhythmic drug therapy. We decided to omit surgery and followed the case under medical treatment for 2 years without complications. Here, we report this rare coexistence and our treatment approach in detail.
Topics: Female; Humans; Adolescent; Wolff-Parkinson-White Syndrome; Atrial Fibrillation; Catheter Ablation; Heart Atria; Tachycardia; Heart Aneurysm; Electrocardiography
PubMed: 37450450
DOI: 10.5543/tkda.2023.60196 -
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 -
Journal of Arrhythmia Jun 2023Radiofrequency ablation (RFA) is the standard method of treatment for tachyarrhythmias in school children, and it leads to complete recovery in children without...
BACKGROUND
Radiofrequency ablation (RFA) is the standard method of treatment for tachyarrhythmias in school children, and it leads to complete recovery in children without structural heart disease. However, RFA in young children is limited by the risk of complications and unstudied remote effects of radiofrequency lesions.
OBJECTIVE
To present the experience of RFA of arrhythmias and the results of follow-up of younger children.
MATERIALS AND METHODS
RFA procedures ( = 255) were performed in 209 children with arrhythmias from 0 to 7 years old. The arrhythmias were presented with atrioventricular reentry tachycardia with Wolff-Parkinson-White (WPW) syndrome (56%), atrial ectopic tachycardia (21.5%), atrioventricular nodal reentry tachycardia (4.8%), and ventricular arrhythmia (17.2%).
RESULTS
The overall effectiveness of RFA, considering the repeated procedures performed due to the primary ineffectiveness and recurrencies, was 94.7%. There was no mortality associated with RFA in patients, including young patients. All cases of "major" complications are associated with RFA of the left-sided accessory pathway and tachycardia foci and are represented by the mitral valve damage in three patients (1.4%). Tachycardia and preexcitation recurred in 44 (21%) patients. There was a correlation between recurrences and parameters of RFA (odds ratio 0.894; 95% confidence interval: 0.804-0.994; = .039). Reducing the maximum power of effective applications in our study increased the risk of recurrence.
CONCLUSION
The use of the minimum effective parameters of RFA in children reduces the risk of complications, but increases arrhythmia recurrence rate.
PubMed: 37324759
DOI: 10.1002/joa3.12827