Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy.

Background: Up to one-half of childhood sarcomeric hypertrophic cardiomyopathy (HCM) presents before the age of 12 years, but this patient group has not been systematically characterized.

Objectives: The aim of this study was to describe the clinical presentation and natural history of patients presenting with nonsyndromic HCM before the age of 12 years.

Methods: Data from the International Paediatric Hypertrophic Cardiomyopathy Consortium on 639 children diagnosed with HCM younger than 12 years were collected and compared with those from 568 children diagnosed between 12 and 16 years.

Anomalous coronary artery in Tetralogy of Fallot-Feasibility of right ventricular outflow tract stenting as initial palliation.

This study addresses the outcome of right ventricle outflow tract (RVOT) stenting in Tetralogy of Fallot (ToF) with anomalous coronaries crossing the RVOT. RVOT stenting in ToF patients has emerged as an alternative to Blalock Taussig shunting. This is a single center study of patients who underwent RVOT stenting for symptomatic ToF at Birmingham Children's Hospital between 2005 and 2020.

Relationship Between Maximal Left Ventricular Wall Thickness and Sudden Cardiac Death in Childhood Onset Hypertrophic Cardiomyopathy.

Background: Maximal left ventricular wall thickness (MLVWT) is a risk factor for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM). In adults, the severity of left ventricular hypertrophy has a nonlinear relationship with SCD, but it is not known whether the same complex relationship is seen in childhood. The aim of this study was to describe the relationship between left ventricular hypertrophy and SCD risk in a large international pediatric HCM cohort.

Supravalvular pulmonary stenosis: A risk factor for reintervention in Noonan syndrome with pulmonary valve stenosis.

Objectives: To assess the short- and long-term outcomes of balloon pulmonary valvuloplasty (BPV) in children with Noonan syndrome (NS).

Background: Pulmonary stenosis (PS) is the most common congenital heart lesion in NS. BPV is the accepted first line treatment in PS.

Friedreich's ataxia-associated childhood hypertrophic cardiomyopathy: a national cohort study.

Objective: Hypertrophic cardiomyopathy (HCM) is an important predictor of long-term outcomes in Friedreich's ataxia (FA), but the clinical spectrum and survival in childhood is poorly described. This study aimed to describe the clinical characteristics of children with FA-HCM.

Design And Setting: Retrospective, longitudinal cohort study of children with FA-HCM from the UK.

Clinical presentation and long-term outcomes of infantile hypertrophic cardiomyopathy: a European multicentre study.

Aims: Children presenting with hypertrophic cardiomyopathy (HCM) in infancy are reported to have a poor prognosis, but this heterogeneous group has not been systematically characterized. This study aimed to describe the aetiology, phenotype, and outcomes of infantile HCM in a well-characterized multicentre European cohort.

Methods And Results: Of 301 children diagnosed with infantile HCM between 1987 and 2019 presenting to 17 European centres [male n = 187 (62.

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.

Stenting of the right ventricular outflow tract as an initial intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries.

Objectives: To assess the role of right ventricular outflow tract stenting as the primary intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries.

Background: The management of a subset of infants with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries requires a staged approach including rehabilitation of diminutive native pulmonary arteries, conventionally using an aortopulmonary shunt. We share our experience of pulmonary artery rehabilitation with right ventricular outflow tract stenting.

Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study.

Aims: Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort.

Methods And Results: Data from 90 patients undergoing ICD insertion at a median age 13 (±3.

Development of a Novel Risk Prediction Model for Sudden Cardiac Death in Childhood Hypertrophic Cardiomyopathy (HCM Risk-Kids).

Importance: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.

Objective: To develop and validate an SCD risk prediction model that provides individualized risk estimates.

Design, Setting, And Participants: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM.

A validation study of the European Society of Cardiology guidelines for risk stratification of sudden cardiac death in childhood hypertrophic cardiomyopathy.

Aims: Sudden cardiac death (SCD) is the most common cause of death in children with hypertrophic cardiomyopathy (HCM). The European Society of Cardiology (ESC) recommends consideration of an implantable cardioverter-defibrillator (ICD) if two or more clinical risk factors (RFs) are present, but this approach to risk stratification has not been formally validated.

Methods And Results: Four hundred and eleven paediatric HCM patients were assessed for four clinical RFs in accordance with current ESC recommendations: severe left ventricular hypertrophy, unexplained syncope, non-sustained ventricular tachycardia, and family history of SCD.

Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom.

Aims: Understanding the spectrum of disease, symptom burden and natural history are essential for the management of children with hypertrophic cardiomyopathy (HCM). The effect of changing screening practices over time has not previously been studied. This study describes the clinical characteristics and outcomes of childhood HCM over four decades in a well-characterized United Kingdom cohort.

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.

Stenting and overdilating small Gore-Tex vascular grafts in complex congenital heart disease.

Background: Gore-Tex grafts are integral in the management of congenital heart disease. Issues of graft stenosis or somatic outgrowth may precipitate high-risk early surgery, and catheter intervention is a relatively under-reported management option.

Objectives: To assess efficacy, safety, and outcomes of stenting and overdilating small Gore-Tex vascular grafts with the aim of optimizing surgical timing.

Stenting of the Right Ventricular Outflow Tract Promotes Better Pulmonary Arterial Growth Compared With Modified Blalock-Taussig Shunt Palliation in Tetralogy of Fallot-Type Lesions.

Objectives: This study sought to compare pulmonary arterial (PA) growth during palliation after right ventricular outflow tract (RVOT) stenting versus modified Blalock-Taussig shunt (mBTS) in patients coming forward for complete repair of tetralogy of Fallot-type lesions.

Background: RVOT stenting is a recent alternative to mBTS in the initial palliation of selected patients with Fallot-type lesions.

Methods: This was a retrospective, single-center study of nonrandomized, consecutive palliated Fallot patients over a 10-year period.

Right ventricular outflow tract stent versus BT shunt palliation in Tetralogy of Fallot.

Objective: This study sets out to compare morbidity, mortality and reintervention rates after stenting of the right ventricular outflow tract (RVOT) versus modified Blalock-Taussig shunt (mBTS) for palliation in patients with tetralogy of Fallot (ToF)-type lesions.

Methods: Retrospective case review study evaluating 101 patients (64 males) with ToF lesions who underwent palliation with either mBTS (n=41) or RVOT stent (n=60) to augment pulmonary blood flow over a 10-year period. Procedure-related morbidity, mortality and reintervention rates were assessed and compared.

Outcome after transcatheter occlusion of patent ductus arteriosus in infants less than 6 kg: A national study from United Kingdom and Ireland.

Objectives: This study aimed to report our national experience with transcatheter patent ductus arteriosus (PDA) occlusion in infants weighing <6 kg.

Background: The technique of transcatheter PDA closure has evolved in the past two decades and is increasingly used in smaller patients but data on safety and efficacy are limited.

Methods: Patients weighing < 6 kg in whom transcatheter PDA occlusion was attempted in 13 tertiary paediatric cardiology units in the United Kingdom and Ireland were retrospectively analyzed to review the outcome and complications.

Transcatheter Retrieval of Cardiovascular Foreign Bodies in Children: A 15-Year Single Centre Experience.

There has been a rapid increase in the practice of interventional catheter treatment of congenital heart disease. Catheter retrieval of embolized cardiac devices and other foreign bodies is essential, yet no large studies have been reported in the paediatric population. Retrospective 15-year review of all children who underwent transcatheter foreign body retrieval in a tertiary cardiac centre from January 1997 to September 2012.

Rare cause of central cyanosis: right pulmonary artery to left atrial fistula.

A fistula between the right pulmonary artery and the left atrium is a very rare cause of central cyanosis. A clinical diagnosis is often made by first excluding other common causes and then performing diagnostic catheterization. Surgical closure offers a definitive cure for this rare congenital anomaly.

Successful emergency cardiac pacing and permanent pacemaker insertion in a preterm 29-week gestation hydropic baby with congenital complete heart block.

A preterm 29-week gestation baby was delivered because of gross foetal hydrops secondary to congenital complete heart block. Despite a poor prognosis, she survived stabilisation and received emergency epicardial pacing followed by permanent pacemaker insertion on day 13, weighing 1.2 kg.

Initial experience with the cook formula balloon expandable stent in congenital heart disease.

Introduction: Balloon expandable stents are an integral part in the catheter treatment of congenital heart disease. In the growing child, stents require dilatation to greater diameters over time. The Cook Formula stent is a recent 316 stainless steel open-cell design licensed for peripheral vascular work.

Stenting of the right ventricular outflow tract.

Objective: To assess the indication, technical aspects, and outcome of stenting of the right ventricular outflow tract (RVOT) in the management of symptomatic patients with severely limited pulmonary blood flow.

Methods: Retrospective case note and procedure review of patients undergoing stenting of the RVOT over an 8 year period.

Patients: Between 2005 and 2012, 52 selected patients underwent percutaneous stent implantation into a very narrow RVOT to improve pulmonary blood flow.

Catheter interventions in the staged management of hypoplastic left heart syndrome.

Aim: To analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome.

Methods: This study is a retrospective case note review of 527 patients undergoing staged Norwood/Fontan palliation at a single centre between 1993 and 2010. Indications and type of catheter interventions were reviewed over a median follow-up period of 7.