Novel Compound Heterozygous Variants in Trans-2,3-Enoyl-Coenzyme A Reductase-Like Gene Associated With Catecholaminergic Polymorphic Ventricular Tachycardia.

A 10-year-old female patient experienced syncope while swimming, and electrocardiography revealed polymorphic ventricular tachycardia, leading to a diagnosis of catecholaminergic polymorphic ventricular tachycardia. No pathogenic variant was identified in . Additional comprehensive genetic testing revealed novel compound heterozygous variants in trans-2,3-enoyl-coenzyme A reductase-like gene, which caused a recessive form of catecholaminergic polymorphic ventricular tachycardia.

Successful Ablation of a Biatrial Tachycardia from the Superior Vena Cava after a Senning Operation for Complete Transposition of the Great Arteries.

The Senning operation used to be widely performed for an intracardiac repair in a complete transposition of the great arteries. During the long-term follow-up, supraventricular tachycardia (SVT) is often observed because of the complex suture lines. The typical mechanism of a Senning-related SVT is cavo-tricuspid isthmus-dependent atrial flutter.

Efficacy of SubcutAneous implantable cardioVErter-defibrillators in ≤18 year-old CHILDREN: SAVE-CHILDREN registry.

Background: In adult patients, subcutaneous implantable cardioverter defibrillators (S-ICDs) have been reported to be non-inferior to transvenous ICDs with respect to the incidence of device-related complications and inappropriate shocks. Only a few reports have investigated the efficacy of S-ICDs in the pediatric field. This study aimed to investigate the utility and safety of S-ICDs in patients ≤18 years old.

A novel mechanism of sudden infant death syndrome during atrioventricular reentrant tachycardia: a case report.

Background: Although orthodromic atrioventricular reentrant tachycardia (AVRT) using retrograde conduction through an accessory pathway (AP) is a common manifestation of Wolff-Parkinson-White (WPW) syndrome, a rare yet critical consequence is sudden cardiac arrest in a few patients. This fatal event used to be reported as a result of rapid atrioventricular conduction of atrial fibrillation via an AP.

Case Summary: A 3-month-old infant with WPW syndrome had AVRT accompanied by global cardiac ischaemia, apparently caused by a rapid ventricular rate itself that degenerated into ventricular fibrillation during the AVRT.

Efficacy of a phosphodiesterase type 5 inhibitor on the pulmonary artery index and postoperative hemodynamics after a Fontan operation.

There have been no reports regarding the influence of a change in the pulmonary artery index before or after a Fontan operation. Moreover, the factors that might affect the change in the pulmonary artery index are unclear. This was a retrospective study, which included 94 patients who underwent a Fontan operation.

Efficacy of a Subcutaneous Implantable Cardioverter Defibrillator in a Child with Early Repolarization Syndrome.

An early repolarization (ER) pattern or J waves are considered to be a benign finding observed in the healthy population, however, it has been pointed out that the ER pattern seen in the inferolateral leads could be an independent risk factor for fatal arrhythmias. We present a pediatric case in which early repolarization syndrome (ERS) was suspected due to the presence of ER or J waves in the inferior leads, which eventually disappeared after the administration of pilsicainide. During the follow-up period, several fatal ventricular arrhythmias were recorded after implantation of a subcutaneous implantable cardiac defibrillator (S-ICD).

The Impact of the Pulmonary Artery Index and Aortopulmonary Collateral Artery Coil Embolization on Intractable Pleural Effusions After a Fontan Surgery.

An intractable pleural effusion is a common comorbidity of a Fontan operation, occasionally leading to undesirable outcomes. The preventive effect of aortopulmonary collateral (APC) coil embolization against a pleural effusion before a Fontan operation is still controversial.This is a retrospective single-center study; among 227 Fontan cases, 57 cases with complete MRI data were analyzed at first.

Ultrahigh density atrio-ventricular dual-chamber mapping as a next generation tool for ablation of accessory pathways.

Introduction: Detailed three-dimensional (3D) mapping has been useful for effective radiofrequency catheter ablation. The Rhythmia system can create atrio-ventricular dual-chamber mapping, which reveals the atrial and ventricular potentials all at once in the same map. The aim of this study was to investigate the utility of mapping the atrium and ventricle simultaneously with a high-density 3D mapping system for the ablation of accessory pathways (AP).

Elimination of arrhythmogenesis after subtotal resection of congenital cardiac fibroma: a case report.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery.

Elimination of arrhythmogenesis after subtotal resection of congenital cardiac fibroma: a case report.

Subtotal tumour resection is used to treat infants with congenital cardiac fibroma and medication-resistant ventricular arrhythmias; however, complete elimination of arrhythmogenic substrates has been unclear. A 4-month-old male infant with congenital cardiac fibroma and ventricular fibrillation underwent subtotal tumour resection and implantable cardioverter-defibrillator implantation. Five years later, angiography revealed impending compression of the left coronary artery.

Growing potential of small aortic valve with aortic coarctation or interrupted aortic arch after bilateral pulmonary artery banding.

Objectives: Bilateral pulmonary artery banding (bPAB) is utilized for some patients with a ventricular septal defect (VSD) and aortic coarctation (CoA) or interrupted aortic arch (IAA). We evaluated aortic valve (AoV) diameter and patient outcomes following bPAB.

Methods: Between August 2010 and September 2015, 10 consecutive patients with VSD and patent ductus arteriosus-dependent CoA or IAA underwent bPAB because of an AoV diameter of approximately <50% of the normal value (n = 6), severe subaortic stenosis and poor patient condition (n = 1, respectively), or low birthweight (n = 2).

Truncus arteriosus with interrupted aortic arch in very low birth weight infants.

A neonate, born after 29 weeks and 2 days gestation (weight 1015 g), was diagnosed with truncus arteriosus and interrupted aortic arch. On postnatal day 30 (1378 g), we performed bilateral pulmonary artery banding. On postnatal day 107 (3024 g), we carried out aortic arch repair and a Rastelli-type procedure.

QT Dynamics During Exercise in Asymptomatic Children with Long QT Syndrome Type 3.

Sympathetic provocative testing is commonly used to detect the abnormal QT dynamics in long QT syndrome (LQTS) patients, particularly LQTS type 1 and type 2. However, little is known about LQTS type 3 (LQT3). We investigated QT dynamics during exercise testing in LQTS patients, particularly LQT3.

Total anomalous pulmonary venous connection with ventricular septal defects.

The association of total anomalous pulmonary venous connection with ventricular septal defect and aortic coarctation is rare. A premature neonate weighing 1350 g was diagnosed with infracardiac total anomalous pulmonary venous connection, aortic coarctation, and a perimembranous ventricular septal defect. On postnatal day 23, we performed emergency extended aortic arch anastomosis under total circulatory arrest, pulmonary venous connection repair using a primary sutureless technique, and patch closure of the atrial septal and perimembranous ventricular septal defects.

QT dynamics evaluated on fully automated QT measurement in children.

Background: The association between QT interval and heart rate (HR), QT dynamics, has been reported to vary greatly between individuals. We investigated QT dynamics using fully automated QT interval measurement with a commercially available multifunctional electrocardiogram (ECG) recorder.

Methods: The study group included 17 otherwise healthy subjects (mean age, 12.

Phrenic nerve protection via packing of gauze into the pericardial space during ablation of cristal atrial tachycardia in a child.

The success of catheter ablation of focal atrial tachycardia is limited by possible collateral damage to the phrenic nerve. Protection of the phrenic nerve is required. Here we present a case of a 9-year-old girl having a history of an unsuccessful catheter ablation of a focal atrial tachycardia near the crista terminalis (because of proximity of the phrenic nerve) who underwent a successful ablation by means of a novel technique for phrenic nerve protection: packing of gauze into the pericardial space.