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Objective The aim of this study was to examine the accuracy in predicting pathway location in children with Wolff--Parkinson--White syndrome for each of seven published algorithms. Patients and interventions ECGs from 100 consecutive children with Wolff--Parkinson--White syndrome undergoing electrophysiological study were analysed by six investigators using seven published algorithms, six of which had been developed in adult patients. Main outcome measures Accuracy and concordance of predictions were adjusted for the number of pathway locations. Results Accessory pathways were left-sided in 49, septal in 20 and right-sided in 31 children. Overall accuracy of prediction was 30-49% for the exact location and 61-68% including adjacent locations. Concordance between investigators varied between 41% and 86%. No algorithm was better at predicting septal pathways (accuracy 5-35%, improving to 40-78% including adjacent locations), but one was significantly worse. Predictive accuracy was 24-53% for the exact location of right-sided pathways (50-71% including adjacent locations) and 32-55% for the exact location of left-sided pathways (58-73% including adjacent locations). Conclusions All algorithms were less accurate in our hands than in other authors' own assessment. None performed well in identifying midseptal or right anteroseptal accessory pathway locations. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Discusses ventricular tachycardias and examines how to determine whether a broad complex tachycardia is ventricular or supraventricular in origin. Ventricular tachycardias; Broad complex tachycardias of supraventricular origin; Wolff-Parkinson-White syndrome; Differentiating between ventricular and supraventricular origin.

Discusses junctional tachycardias, including atrioventricular nodal re-entrant tachycardia and atrioventricular re-entrant tachycardia. Medical information on the two types of tachycardia and their causes; Symptoms in patients with these disorders; Classification of Wolff-Parkinson-White syndrome.

For most types of supraventricular arrhythmias medical treatment with antiarrythmic drugs is not completely effective. In addition to poor or sporadic efficacy, such drugs can be associated with a number of bothersome and even fatal side effects, proarrhythmia, cost, and inconvenience. Over the past two decades, catheter ablation has evolved from a highly experimental technique to first line treatment for many cardiac arrhythrnias. During radiofrequency ablation, current flows into the tissue underlying the active electrode in alternating direction at high frequency.

Clinical and electrocardiographic findings for 30 patients with the pre-excitation syndrome are described together with details of treatment. Nineteen (63%) were younger than 2 years, 14 of whom were under 2 months. Sixteen infants and 7 children (77%) presented with paroxysmal supraventricular tachycardia, 14 (61%) of whom had the electrocardiographic pattern of type A Wolff-Parkinson-White (WPW) syndrome. During paroxysmal bouts the QRS complex was normal in 21 patients and wide in two. Six (20%) patients had congenital heart disease often associated with WPW syndrome type B. Seventeen patients were treated with either digoxin or verapamil intravenously to stop tachyarrhythmias. Verapamil was more effective due to the immediate response and lack of adverse effects. The tachyarrhythmias resolved in all the patients and in some of them the WPW pattern resolved later indicating maturation of the conduction tissue with loss of the accessory pathways. Verapamil provides a rapid and safe form of treatment for conversion of tachyarrhythmias since it has no effect on the accessory pathways. Oral amiodarone prevents recurrent tachyarrhythmias resistant to other treatment. [ABSTRACT FROM AUTHOR]

This report highlights the association between tuberous sclerosis and Wolff-Parkinson-White syndrome. Ten patients with concurrent diagnoses of Wolff-Parkinson-White syndrome and tuberous sclerosis were identified. Wolff-Parkinson-White syndrome presented early in life, nine cases being diagnosed in the first year. Eight of the 10 cases were male. In eight cases, the syndrome was associated with supraventricular tachycardias, and in nine with cardiac rhabdomyomata. One child died from cardiac failure secondary to obstruction of the left ventricular outflow tract by a rhabdomyoma. Five of nine survivors showed resolution of Wolff-Parkinson-White syndrome on follow up. The accessory pathway was localised in nine patients from surface electrocardiograms: six children had left sided pathways and three had right sided pathways. [ABSTRACT FROM AUTHOR]

Discusses the results of a study examining asymptomatic ventricular pre-excitation in children and adolescents. Wolff-Parkinson-White syndrome; Electrophysiology; Congenital heart disease; Accessory pathway location; Risk of sudden death.

Supraventricular tachycardia (SVT) is the most common symptomatic tachyarrhythmia in childhood. Treatment involves vagal manoeuvres, pharmacological agents or electrical cardioversion. We report a child with recurrent SVT secondary to Wolff-Parkinson-White syndrome who is consistently able to revert her SVT by doing a handstand. This case highlights a simple non-invasive technique for the treatment of SVT. [ABSTRACT FROM AUTHOR]

Examines the atrial fibrillation complication in Wolff-Parkinson-White syndrome treated with amiodarone. Transmission of impulses at the myocardial bridge bypassing the atrioventricular node; Increase in ventricular rate during atrial fibrillation; Efficacy of adrenoceptor blocking agents in medical treatment.

A female neonate with congenital complete heart block developed atrioventricular conduction through an accessory pathway. Despite sinus rhythm and an adequate heart rate she developed severe dilated cardiomyopathy and died at age 14 months. This case illustrates that underlying heart block can be present in individuals with asymptomatic Wolff-Parkinson-White syndrome and that the dilated cardiomyopathy that occasionally accompanies autoimmune congenital heart block is not primarily caused by bradycardia. [ABSTRACT FROM AUTHOR]

The article focuses on the risks and management of Wolff-Parkinson-White syndrome (WPW). WPW is an electrocardiographic abnormality and a common diagnostic and therapeutic issue among cardiac specialists. The condition is associated to the eccentric activation of the ventricular myocardium causing electrophysiological consequences like paroxysmal occurrence of atrioventricular reciprocating tachycardia (SVT).

The presence of Wolff-Parkinson-White (WPW) syndrome in patients presenting with syncope suggests that tachyarrhythmia may be the cause. However, symptoms require careful evaluation. Two young patients presented with syncope and were found to have WPW syndrome on their ECG.

Reports on key developments concerning pediatrics in Great Britain as of April 2003. Effects of infant feeding on adolescent serum concentrations of total- and low-density lipoprotein cholesterol; Ventricular pre-excitation pattern associated with Wolff-Parkinson-White syndrome; Other key issues of interest.

A 41 year old male with Wolff-Parkinson-White syndrome and symptomatic atrioventricular re-entrant tachycardia was admitted for electrophysiological study. An exceptionally large coronary sinus diverticulum was identified and found to contain the accessory atrioventricular pathway. Successful radiofrequency ablation to this posteroseptal accessory pathway was carried out. Coronary sinus aneurysms or diverticulae are rare congenital anomalies with only a few published images. Diverticulae associated with a posteroseptal accessory atrioventricular connection were first described in 1985.

Examines the pre-excitation and mitral valve prolapse. Incidence of both supraventricular and ventricular arrhythmias; Details of the patient with mitral valve prolapse; Causes of a prolapsing mitral valve.