Planning Transition of Care for Adolescents Affected by Congenital Heart Disease: The Irish National Pathway.

At some point in their life, adolescent patients with a congenital heart disease (CHD) transition from paediatric services to adult care facilities. The process is not without any risks, as it is often linked with a significantly progressive deterioration in adolescents' health and loss of follow-up. In fact, transition patients often encounter troubles in finding a care giver who is comfortable managing their condition, or in re-establishing trust with the new care provider.

Outcomes From Pediatric Ablation: A Review of 20 Years of National Data.

Objectives: This study set out to examine outcomes from pediatric supraventricular tachycardia ablations over a 20-year period. This study sought to examine success rates and repeat ablations over time and to evaluate whether modalities such as 3-dimensional (3D) mapping, contact force, and cryotherapy have improved outcomes.

Background: Ablation of supraventricular tachycardia in pediatric patients is commonly performed in most congenital heart centers with excellent long-term results.

International Triadin Knockout Syndrome Registry.

Background: Triadin knockout syndrome (TKOS) is a rare, inherited arrhythmia syndrome caused by recessive null mutations in TRDN-encoded cardiac triadin. Based previously on 5 triadin null patients, TKOS has been characterized by extensive T-wave inversions, transient QT prolongation, and severe disease expression of exercise-induced cardiac arrest in early childhood refractory to conventional therapy.

Methods: We have established the International Triadin Knockout Syndrome Registry to include patients who have genetically proven homozygous/compound heterozygous TRDN null mutations.

Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study.

Background: Although ventricular fibromas are rare, they are the second most common type of cardiac tumor in children. While histologically benign, they have a propensity to cause malignant arrhythmias, with cardiac arrest often being the first presentation.

Objective: The purpose of this study was to evaluate the arrhythmia risk and management strategies for pediatric ventricular fibromas.

A multicentre study of patients with Timothy syndrome.

Aims: Timothy syndrome (TS) is an extremely rare multisystem disorder characterized by marked QT prolongation, syndactyly, seizures, behavioural abnormalities, immunodeficiency, and hypoglycaemia. The aim of this study was to categorize the phenotypes and examine the outcomes of patients with TS.

Methods And Results: All patients diagnosed with TS in the United Kingdom over a 24-year period were reviewed.

Compound Heterozygous Triadin Mutation Causing Cardiac Arrest in Two Siblings.

We present the case of two siblings who both presented with an out-of-hospital cardiac arrest at 2 years of age. Both siblings underwent internal cardiac defibrillator implantation and both had recurrent episodes of ventricular fibrillation (VF). A compound heterozygous mutation in the triadin gene was discovered; one of these mutations has been described previously in the homozygous state, and the other one is unreported.

Extraction of SelectSecure leads compared to conventional pacing leads in patients with congenital heart disease and congenital atrioventricular block.

Background: SelectSecure™ pacing leads (Medtronic Inc) are increasingly being used in pediatric patients and adults with structural congenital heart disease. The 4Fr lead is ideal for patients who may require lifelong pacing and can be advantageous for patients with complex anatomy.

Objective: The purpose of this study was to compare the extraction of SelectSecure leads with conventional (stylette-driven) pacing leads in patients with structural congenital heart disease and congenital atrioventricular block.

Implantation of the new Medtronic LINQ™ loop recorder in an infant with ventricular tachycardia.

The Medtronic LINQ™ was inserted in an 11-month-old boy for close monitoring of rapid ventricular tachycardia. The device is much smaller than the conventional Medtronic loop recorder. The real advantage of the LINQ™ is that it automatically notifies Carelink if any ventricular tachycardia is seen, which is very advantageous for this particular type of patient.

Cumulative radiation exposure in pediatric patients with congenital heart disease.

Certain pediatric patients undergoing surgery for the most severe forms of congenital heart disease are exposed to high doses of ionizing radiation. The amount of cumulative radiation exposure from all modalities has not yet been evaluated. The purpose of our study was to evaluate the cumulative radiation exposure in a contemporary cohort of patients with congenital heart disease undergoing single-ventricle palliation.

Interrupted aortic arch with retrograde subclavian perfusion.

Type B interrupted aortic arch results in the left subclavian artery being perfused by a patent ductus arteriosus and antegrade flow is usually seen in the left subclavian artery. We describe a case of retrograde perfusion of the left subclavian artery through the circle of Willis in the presence of interrupted aortic arch type B and a restrictive patent ductus arteriosus. This is similar to subclavian steal syndrome when the perfusion pressure from the cerebral circuit is greater than that from the left subclavian artery.

Conduction abnormalities in pediatric patients with restrictive cardiomyopathy.

Background: Pediatric restrictive cardiomyopathy carries a poor prognosis secondary to a high risk of sudden death previously attributed to ventricular tachyarrhythmias. The extent of conduction abnormalities in this population and their relationship to life-threatening events has not been previously reported.

Methods And Results: A retrospective study of pediatric patients with restrictive cardiomyopathy diagnosed between April 1994 and May 2011 was performed.

Airway pressure release ventilation improves pulmonary blood flow in infants after cardiac surgery.

Objective: We hypothesized that spontaneous inspiratory effort transmitted to the pleural space during airway pressure release ventilation would result in increased lung perfusion after surgery for tetralogy of Fallot or following a cavopulmonary shunt as a consequence of transient decreases in intrapleural pressure.

Design: Prospective crossover cohort study.

Setting: A tertiary care cardiac pediatric intensive care unit.

Critical care outcomes in pulmonary atresia and intact ventricular septum undergoing single-ventricle palliation.

Objective: To examine early outcomes for pulmonary atresia with intact ventricular septum undergoing single-ventricle palliation and to determine risk factors for mortality.

Design: Retrospective observational study.

Setting: Tertiary paediatric critical care unit.

Kaposiform hemangioendothelioma presenting antenatally with a pericardial effusion.

We describe an infant presenting with fetal pericardial effusion requiring in utero pericardiocentesis. Important postnatal clinical features included recurrent pericardial effusion, progressive stridor, thrombocytopenia, anemia, and a mediastinal mass surrounding the heart and coronary arteries. Investigations and management consisted of repeat pericardiocentesis, platelet and red blood cell transfusions, laryngoscopy, creation of a pericardial window, and biopsy of the mediastinal mass.

Surgical repair of the mitral valve in children with dilated cardiomyopathy and mitral regurgitation.

Background: Significant mitral regurgitation is known to exacerbate left ventricular dysfunction in dilated cardiomyopathy. Although intervention on the regurgitant mitral valve is frequently described in adults, there is little pediatric data.

Methods: Five children (aged 3 months to 4 years) with dilated cardiomyopathy and mitral regurgitation underwent mitral valve repair between January 1999 and January 2007 at our institution.

Tbx5-dependent pathway regulating diastolic function in congenital heart disease.

At the end of every heartbeat, cardiac myocytes must relax to allow filling of the heart. Impaired relaxation is a significant factor in heart failure, but all pathways regulating the cardiac relaxation apparatus are not known. Haploinsufficiency of the T-box transcription factor Tbx5 in mouse and man causes congenital heart defects (CHDs) as part of Holt-Oram syndrome (HOS).

Occlusion of a Sano shunt using the amplatzer duct occluder.

The presence of a residual systemic-to-pulmonary shunt following a bidirectional Glenn operation may be necessary in patients with an elevated pulmonary vascular resistance. It is often possible to perform percutaneous occlusion of these shunts once the resistance has fallen. We report on the use of an Amplatzer Ductal Occluder Device to occlude a residual Sano shunt in an infant with a variant of hypoplastic left heart syndrome.

Radiofrequency perforation of the right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect.

Background: Radiofrequency perforation (RF) of the right ventricular outflow tract (RVOT), while an effective management strategy in children with an intact ventricular septum, has not been fully detailed in those presenting with a ventricular septal defect.

Objective: To determine whether transcatheter perforation of the atretic pulmonary valve is an acceptable management strategy prior to surgical repair.

Results: Valve perforation was attempted in eight children seen between May 2000 and March 2006, five being infants between 1 and 9 days of age.

Percutaneous closure of postoperative ventricular septal defects with the Amplatzer device.

The objective of this study was to look at the procedure, the results, and the follow-up of patients who underwent percutaneous closure of a residual ventricular septal defect (VSD) following a surgical closure using the Amplatzer VSD device. Four patients had an original diagnosis of tetralogy of Fallot, two patients had a patch leak following a surgical repair of a VSD, and three patients had a VSD not repaired at the time of surgery. All patients fulfilled the currently accepted surgical criterion for reoperation (Qp/Qs>1.