Evaluation of a modified paediatric early warning score for children with congenital heart disease.

Background: Paediatric early warning score systems are used for early detection of clinical deterioration of patients in paediatric wards. Several paediatric early warning scores have been developed, but most of them are not suitable for children with cyanotic CHD who are adapted to lower arterial oxygen saturation.

Aim: The present study compared the original paediatric early warning system of the Royal College of Physicians of Ireland with a modification for children with cyanotic CHD.

Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits.

Objectives: An unobstructed extracardiac conduit (ECC) is essential for optimal Fontan haemodynamics. We aimed to evaluate the feasibility and results of percutaneous transcatheter stenting of the ECC in paediatric patients with a significant Fontan pathway obstruction.

Methods: Our institutional database was searched to identify all Fontan patients who had a stent placed in their ECC.

Percutaneous tricuspid valve replacement in childhood.

Percutaneous replacement of the tricuspid valve with a bovine jugular venous valve (melody valve) was successfully undertaken in a 9-year-old boy. The patient had a previous history of bacterial endocarditis of the native tricuspid valve in infancy. Initially, a pericardial patch valve was created, followed by surgical replacement of the valve using a biological tissue valve at 4 years of age.

Low serum 25-hydroxyvitamin D levels and secondary hyperparathyroidism in Fontan patients.

Background: Limited data exist on the vitamin D status in Fontan patients. We determined the prevalence and potential risk factors of vitamin D deficiency in this patient subset. Methods and results Data were collected from 27 Fontan patients (55.

Stenting of Native Right Ventricular Outflow Tract Obstructions in Symptomatic Infants.

Objective: To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants.

Methods: Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011.

Results: Median age and weight at the time of first stent implantation were 8 weeks and 3.

Stent therapy for acute and chronic obstructions in extracardiac Fontan conduits.

We describe transcatheter therapy for early onset occlusion or stenoses of extracardiac conduits in three children who had undergone Fontan completion. Successful stent implantation was associated with complete resolution of symptoms.

Coarctation of the aorta and vein of Galen malformation - treatment considerations in a severely compromised patient.

A vein of Galen malformation - a rare cause of cardiac insufficiency in neonates - is sometimes associated with coarctation of the aorta, two diseases requiring urgent therapy in the neonatal period. We report on a term neonate in whom we first palliated the coarctation by stent implantation, providing time to treat the vein of Galen malformation by endovascular embolisation. Following this, the coarctation was surgically repaired and the stent was explanted.

Percutaneous radiofrequency septal reduction for hypertrophic obstructive cardiomyopathy in children.

Objectives: The aim of this study was to assess the efficacy of radiofrequency catheter ablation (RFCA) in the treatment of hypertrophic obstructive cardiomyopathy in children.

Background: Hypertrophic obstructive cardiomyopathy is an uncommon cause of left ventricular outflow tract obstruction in children. In symptomatic patients, open heart surgical myectomy has hitherto been the only therapeutic option.

Safety and results of cryoablation in patients <5 years old and/or <15 kilograms.

Current recommendations discourage elective radiofrequency ablation in patients <5 years old and/or weighing <15 kg, primarily because of the greater complication rate. To describe the current use, complications, and immediate outcomes of cryoablation in this patient population, a multicenter retrospective review of all patients <5 years old and/or weighing <15 kg who were treated with cryoablation for arrhythmia was performed. Eleven centers contributed data for 68 procedures on 61 patients.

Ablation strategies in a patient with Belhassen tachycardia.

Unlabelled: The verapamil-sensitive Belhassen tachycardia is a ventricular reentrant tachycardia, involving left-sided Purkinje fibers and abnormal Purkinje or myocardial tissue. Ablation is feasible, targeting a diastolic fascicular potential in the apical left ventricle.

Case Report: We report on a 13-year-old girl with left posterior fascicular ventricular tachycardia.

Extended applications of the Amplatzer vascular plug IV in infants.

Objective: A variety of devices are available for transcatheter closure of unwanted shunts. We describe our experience with the use of the Amplatzer vascular plug IV in a consecutive series of infants.

Methods: A total of eight consecutive infants - all born preterm at gestational ages ranging from 24 to 35 weeks - undergoing transcatheter closure of unwanted shunts - persistently patent arterial duct in five patients, an aorta to right atrium fistula in one, multiple aortopulmonary collateral vessels in one, and an azygos vein to left atrium connection in one - are described.

Device closure of ventricular septal defects by hybrid procedures: a multicenter retrospective study.

Objective: This study reports on the feasibility, efficacy, and outcome of hybrid procedures to close ventricular septal defects (VSD), reflecting the experience of 11 centers in Germany, Austria, and Switzerland.

Background: Beating heart closure of VSD has attracted interest in small infants, complex anomalies and postinfarction scenarios where patients are at high risk during surgery. Perventricular or intraoperative device placement allows access to the lesions where percutaneous delivery is limited.

Reopening acutely occluded cavopulmonary connections in infants and children.

Little is known about the outcome of acute thrombotic occlusion of segments of the cavopulmonary connections (CPC) in infants and children with univentricular hearts. Early recognition and aggressive therapy may result in successful salvage of some of these patients. Five consecutive patients (age range 4-8 months) presenting with acute occlusion of a CPC segment underwent emergency cardiac catheterization.

Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this association.

Background: Ventricular preexcitation may be associated with dilated cardiomyopathy, even in the absence of recurrent and incessant tachycardia.

Methods: This report describes the clinical and electrophysiologic characteristics of 10 consecutive children (6 males), with median age of 8 years (range, 1-17 years), who presented with dilated cardiomyopathy and overt ventricular preexcitation on the 12-lead electrocardiogram. Incessant tachycardia as the cause of dilated cardiomyopathy could be excluded.

Atrioventricular block following implantation of a stent in the oval fossa.

A neonate weighing 2.8 kilograms underwent the staged hybrid procedure for palliation of hypoplastic left heart syndrome. Within 6 hours following placement of a stent to retain patency of the interatrial communication, the infant developed second degree atrioventricular block, which resolved within 24 hours.

Perioperative placement of stents for relief of proximal pulmonary arterial stenoses in infants.

Introduction: Stenoses in the pulmonary arterial system can have a significant negative impact on the early postoperative course in infants. Early recognition and aggressive management are mandatory.

Patients And Methods: We describe our experience with 8 infants, with ages ranging from 3 to 9 months, weighing from 4.

Documented narrow QRS tachycardia not inducible during electrophysiology study: should we modify the AV node or not.

Ablation therapy is the widely accepted definitive therapy for atrioventricular reentry tachycardia. Noninducibility of the tachycardia is the targeted end point of the procedure. We report on 21 patients with documented narrow QRS tachycardia, in whom the clinical tachycardia could not be induced during the electrophysiologic study.

Superior vena cava stenting and transvenous pacemaker implantation (stent and pace) after the Mustard operation.

The Mustard operation for transposition of the great arteries is associated with good long-term survival. Typical complications at follow-up include progressive loss of sinus node function requiring permanent pacemaker implantation, and systemic venous pathway obstruction often precluding a transvenous approach to pacing. We report on 7 patients (median age 14.

Wireless Holter transmission in suspected dysrhythmias.

Background: In patients with a history of possible dysrhythmias, documentation of an episode is mandatory before any form of treatment is given. Holter recordings with wireless telemetry offer the possibility of continuously recording electrocardiograms for days or even weeks with instantaneous analysis of the data in the physician's office.

Methods: Thirty-seven patients (20 male; median age, 8.

Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study.

Objectives: Our objective was to evaluate the short-term safety and efficacy of cardiac resynchronization therapy (CRT) in children.

Background: Cardiac resynchronization therapy has been beneficial for adult patients with poor left ventricular function and intraventricular conduction delay. The efficacy of this therapy in the young and in those with congenital heart disease (CHD) has not yet been established.