The impact of viral respiratory infection on surgical outcome of cavopulmonary shunt.

Undetected respiratory infections may adversely affect the intrapulmonary resistance after Stage 2 or Stage 3 Fontan palliation. A few studies describe a higher risk for viral pneumonia during respiratory virus season, but none of them have focused on the effect of symptomatic viral pneumonia on in-hospital clinical course after bidirectional Glenn shunt. We analysed 77 patients who underwent bidirectional Glenn shunt surgery.

Stent Strut Dilation in Branch Pulmonary Artery Stenosis Following Stenting of Arterial Duct in Duct-dependent Pulmonary Circulation.

To assess the feasibility and outcome of stent strut dilation after arterial duct stenting with associated branch pulmonary artery (BPA) stenosis. Stenting of arterial duct in infants with duct-dependent pulmonary circulation is technically challenging. The presence of BPA stenosis is a relative contraindication for stent implantation.

Thyroid dysfunction in pediatric Fontan patients is associated with unfavorable hemodynamic status and severity of protein-losing enteropathy: A report from the Fontan care network.

Background: Thyroid dysfunction may have adverse effects on Fontan hemodynamics. Data on thyroid function in pediatric Fontan patients with or without protein-losing enteropathy (PLE) are limited.

Methods: This retrospective multicenter study included 67 Fontan patients (median age 10.

Patterns of Electrocardiographic Abnormalities in Children with Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM), a common cardiomyopathy in children, is an important cause of morbidity and mortality. Early recognition and appropriate management are important. An electrocardiogram (ECG) is often used as a screening tool in children to detect heart disease.

Indications for and outcomes of interstage catheter interventions following the Norwood procedure: A single-institution study.

Aims: The aim of this study was to document the incidence, types, and outcome of interstage catheter interventions following the Norwood surgical palliation.

Patients And Methods: A retrospective single-center study of all patients surviving the Norwood operation was performed. All data concerning interstage catheter interventions up to the completion of the superior cavopulmonary shunt were collected.

Initial Clinical Trial of a Novel Pulmonary Valved Conduit.

Valved allografts and xenografts for reconstruction of the right ventricular outflow tract (RVOT) lack durability and do not grow. We report the first clinical use of a completely bioabsorbable valved conduit (Xeltis pulmonary valve - XPV) in children. Twelve children (six male), median age five (two to twelve) years and median weight 17 (10 to 43) kg, underwent RVOT reconstruction with the XPV.

Interventional recanalisation of long segment aortic atresia.

We report the case of a long-segment aortic atresia as the cause for therapy resistant arterial hypertension in a young adult. Recanalization was achieved interventionally by wire-crossing and stent implantation with subsequent normalization of blood pressure.

Emerging clinical applications of strain imaging and three-dimensional echocardiography for the assessment of ventricular function in adult congenital heart disease.

Management of congenital heart disease (CHD) in adults (ACHD) remains an ongoing challenge due to the presence of residual hemodynamic lesions and development of ventricular dysfunction in a large number of patients. Echocardiographic imaging plays a central role in clinical decision-making and selection of patients who will benefit most from catheter interventions or cardiac surgery..

Subclinical myocardial disease in patients with primary hyperoxaluria and preserved left ventricular ejection fraction: a two-dimensional speckle-tracking imaging study.

Background: Primary hyperoxaluria (PH) is characterized by progressive chronic kidney disease (CKD) and systemic oxalate deposition. Myocardial dysfunction might be present early in the course of the disease. However, this hypothesis has not yet been tested in the PH population.

Impact of Obesity on Left Ventricular Thickness in Children with Hypertrophic Cardiomyopathy.

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM.

Successful biventricular cardiac resynchronization therapy in a failing Fontan patient: Implications of ventriculo-ventricular interdependency in single ventricle physiology.

We describe a Fontan patient with severe heart failure who was successfully treated with biventricular cardiac resynchronization therapy (CRT). Our case shows that strain imaging might play a crucial role in guiding placement of pacing leads and in characterizing the electromechanical substrate associated with a favorable CRT response. Furthermore, we demonstrate for the first time that ventriculo-ventricular interdependency seems an important mechanical concept, which can be utilized to augment cardiac performance in failing Fontan patients with a functional hypoplastic ventricle.

Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: An international multicenter study.

Background: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM).

Objective: The purpose of this study was to determine predictors of LAE in children with HCM.

Methods: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.

Implantation of a subcutaneous implantable cardioverter defibrillator with right parasternal electrode position in a patient with D-transposition of the great arteries and concomitant AAI pacemaker: a case report.

Background: Implantable cardioverter defibrillator (ICD) therapy is indicated in patients with structural heart disease who have had an aborted cardiac arrest (ACA). After atrial repair of d-transposition of the great arteries (d-TGA, Mustard repair) patients seem to be at a higher risk of failing intraoperative subcutaneous ICD (S-ICD) shock testing.

Case Summary: We report the case of a 45-year-old patient with congenital heart disease (CHD) who suffered a cardiac arrest from ventricular fibrillation and was subsequently implanted with a S-ICD.

Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients.

Objectives: The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA).

Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population.

Off-label use of duct occluder devices to close hemodynamically significant perimembranous ventricular septal defects: A multicenter experience.

Objective: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc.

Background: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique.

Predictors of electrocardiographic screening failure for the subcutaneous implantable cardioverter-defibrillator in children: A prospective multicenter study.

Background: Subcutaneous implantable cardioverter-defibrillator (SICD) shows promise for select patients at risk of sudden cardiac death. However, patients need to pass an electrocardiographic (ECG) screening (ECG-S) test before they can receive an SICD. Predictors of ECG-S failure in children are unclear.

The development of pacing induced ventricular dysfunction is influenced by the underlying structural heart defect in children with congenital heart disease.

Background: Right ventricular pacing can cause pacing-induced ventricular dysfunction (PIVD) correctable with biventricular pacing (BiVP). Factors associated with PIVD are poorly understood.

Methods: We reviewed children receiving epicardial dual-chamber pacemakers for complete heart block (CHB) after congenital heart disease (CHD) surgery.

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.

The electrocardiogram of the neonate and infant.

The ECG in children has a number of characteristic differences compared to the ECG of the adult. The transition of the ECG in neonates after birth represents dynamic changes of the circulatory system due to the postnatal adaptation, different physiologic properties of the fetal and neonatal myocardium, the location and orientation of the heart in the chest and influence of body mass during that period and later on in childhood. The complexity of the changes implies a broad variation of ECG changes during the first days and weeks of life, whose interpretation requires expert knowledge.

Postnatal Development of Right Ventricular Myofibrillar Biomechanics in Relation to the Sarcomeric Protein Phenotype in Pediatric Patients with Conotruncal Heart Defects.

Background: The postnatal development of myofibrillar mechanics, a major determinant of heart function, is unknown in pediatric patients with tetralogy of Fallot and related structural heart defects. We therefore determined the mechanical properties of myofibrils isolated from right ventricular tissue samples from such patients in relation to the developmental changes of the isoforms expression pattern of key sarcomere proteins involved in the contractile process.

Methods And Results: Tissue samples from the infundibulum obtained during surgery from 25 patients (age range 15 days to 11 years, median 7 months) were split into half for mechanical investigations and expression analysis of titin, myosin heavy and light chain 1, troponin-T, and troponin-I.

Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review.

Objective: A standardised diagnostic definition of protein-losing enteropathy (PLE) in Fontan patients serves both patient care and research. The present study determined whether a diagnostic definition of PLE was routinely used in published clinical Fontan studies, and to identify potentially relevant diagnostic criteria for composing a uniform PLE definition.

Methods: A systematic review was conducted in adherence to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations.

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.