Transaortic cryoablation of uncommon atrioventricular nodal reentrant tachycardia after Senning and Rastelli operations.

A 36-years-old woman with a congenital corrected transposition of the great arteries, who underwent Senning and Rastelli operations, was admitted with a symptomatic supraventricular tachycardia. During an electrophysiological study, uncommon atrioventricular (AV) nodal reentrant tachycardia was induced. The coronary veins and coronary sinus did not connect to the systemic venous atrium.

Familial atrial rapid fibrillation associated with double mutations of SCN5A and KCNQ1.

Familial atrial fibrillation is inherited and sporadically occurs in the paediatric population. Generally, fibrillated wavelets are reported at a frequency of approximately 6 Hz. Herein, we report a familial case presenting rapidly fibrillated wavelets at frequencies of approximately 12 to 30 Hz associated with KCNQ1 and SCN5A mutations.

A novel procedure of puncture to access the pulmonary venous atrium in complex congenital heart disease with surgical correction.

Background: In complex congenital heart disease, supraventricular arrhythmia develops long term after surgical repair. The arrhythmia could have pharmacological tolerance and sometimes be fatal. We report our experiences with puncturing tough or calcified surgical repair material to access the pulmonary venous atrium for the purpose of the management of arrhythmia in complex congenital heart disease with surgical correction.

Transvenous shock-only implantable cardioverter defibrillator after an atrio-pulmonary Fontan surgery.

A 42-year-old woman with tricuspid atresia who underwent a Fontan surgery (atrio-pulmonary connection) was admitted to our hospital due to symptomatic ventricular tachycardia (VT). A defibrillation lead was implanted in a distal site of a coronary vein since there was no usual entry to the ventricle. Ventricular pacing was impossible due to the high threshold, however, good sensing was obtained.

Point ablation for macro-reentrant ventricular tachycardia associated with Ebstein's anomaly and pulmonary atresia: a case report.

We report a case with Ebstein's anomaly and pulmonary atresia with sustained monomorphic ventricular tachycardia in a patient without a ventriculotomy history. In the low voltage area between the atrialised right ventricle and hypoplastic right ventricle, there was a ventricular tachycardia substrate and slow conduction. The tachycardia circuit was eliminated by a point catheter ablation at the area with diastolic fractionated potentials.

Impact of preoperative electrophysiological intervention on occurrence of peri/postoperative supraventricular tachycardia following Fontan surgery.

Background: Little is known about the effects of preoperative electrophysiological study (EPS) and catheter ablation (CA) in Fontan surgery candidates with supraventricular tachycardia (SVT).

Objective: The purpose of this study was to investigate the clinical impact of EPS-guided intervention in Fontan surgery candidates with preceding SVT events.

Methods: A total of 109 consecutive patients with a history of SVT before Fontan surgery were divided into 3 groups: 44 in whom EPS with CA was attempted (CA group); 21 in whom EPS without CA was attempted (EPS group); and 44 in whom EPS was not performed (N group).

Electrophysiological features and radiofrequency catheter ablation of accessory pathways associated with atrioventricular discordance.

Introduction: Although a high prevalence of the presence of an accessory pathway (AP) associated with atrioventricular (AV) discordance has been reported, a case series of its characteristics and the results of catheter ablation (CA) have not been sufficiently documented.

Methods And Results: We retrospectively examined 11 consecutive patients with atrioventricular discordance who underwent CA for atrioventricular reciprocating tachycardia (AVRT) via an AP and planned cardiac surgery after CA. Orthodromic AVRTs were induced in 10 patients via AP, but no antidromic/duodromic AVRT was induced in any of the cases.

Acute and Long-Term Outcomes of Transvenous Cardiac Pacing Device Implantation in Patients With Congenital Heart Disease.

Little is known about the acute/long-term outcomes of implantation of cardiac implantable electronic devices (CIED) using a transvenous approach for patients with congenital heart disease (CHD). We retrospectively investigated the acute/long-term results and complications associated with transvenous CIED implantation in 140 patients with CHD. We implanted 77 pacemakers, 51 implantable cardioverter defibrillators (ICD), and 12 cardiac resynchronization therapy (CRT) devices.

Different responses to exercise between Andersen-Tawil syndrome and catecholaminergic polymorphic ventricular tachycardia.

Aims: Andersen-Tawil Syndrome (ATS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are both inherited arrhythmic disorders characterized by bidirectional ventricular tachycardia (VT). The aim of this study was to evaluate the diagnostic value of exercise stress tests for differentiating between ATS and CPVT.

Methods And Results: We included 26 ATS patients with KCNJ2 mutations from 22 families and 25 CPVT patients with RyR2 mutations from 22 families.

Safety and Efficacy of Radiofrequency Catheter Ablation for Tachyarrhythmia in Children Weighing Less Than 10 kg.

An increasing number of children are undergoing radiofrequency catheter ablation (RFCA) for tachyarrhythmia. However, infants and toddlers undergoing RFCA are often resistant to medication or need to eliminate arrhythmia substrate, and the risks of RFCA complications are still high in infants and toddlers. From April 2008 and December 2016, 285 children who underwent radiofrequency catheter ablation (RFCA) were stratified according to body weight (group A, less than 10 kg, n = 22; group B, over 10 kg, n = 263) and the clinical features of RFCA were retrospectively reviewed in these groups.

Long-Term Efficacy of Implantable Cardioverter Defibrillator in Repaired Tetralogy of Fallot - Role of Anti-tachycardia Pacing.

Background: Tetralogy of Fallot (TOF) is one of the common congenital heart diseases (CHD) in implantable cardioverter defibrillator (ICD) recipients, but few studies have reported the long-term outcomes of and the anti-tachycardia pacing (ATP) efficacy in repaired TOF.Methods and Results:Twenty-one repaired TOF patients with an ICD implanted between April 2003 and March 2015 were investigated retrospectively. ICD therapy and clinical outcome were analyzed.

Successful catheter ablation of ventricular tachycardia in a patient with congenitally corrected transposition of great arteries after double switch operation.

Ventricular tachycardia (VT) may cause sudden death late after repair of congenital heart disease. Radiofrequency catheter ablation (CA) of VT can be effective but may be hampered by hypertrophied myocardium or prosthetic material. A 33-year-old man with congenitally corrected transposition of the great arteries (ccTGA) had undergone a double switch operation (DSO) with the combined Mustard and Rastelli procedures when he was 10 years old.

Ventricular Tachycardia Rotating a Scar of a Total Right Ventricular Exclusion.

We describe a 15-year-old postoperative girl who underwent surgical 3-dimensional mapping and ablation of hemodynamically unstable ventricular tachycardia (VT) with an on-pump beating heart surgical technique. She had previously received a tricuspid valve closure, entire right ventricular free wall resection, and finally Fontan operation with an extracardiac conduit to treat a severe Ebstein's anomaly. Activation mapping revealed a VT rotating around a large right ventricular free wall incisional scar with a narrow conduction channel between the scar and a tricuspid annulus (TA).

Incidence, Clinical Course, and Risk Factors of Amiodarone-Induced Thyroid Dysfunction in Japanese Adults With Congenital Heart Disease.

Background: Although amiodarone (AMD)-induced thyroid dysfunction (AITD) is an important complication of AMD therapy, little is known about AITD in adult Japanese patients with congenital heart disease (CHD).

Methods And Results: We retrospectively studied 131 adult patients with CHD who were on low-dose AMD (median, 150 mg/day). The median patient age was 28 years, and the median follow-up was 44 months.

Electrophysiologic studies and radiofrequency catheter ablation of ectopic atrial tachycardia in children.

Ectopic atrial tachycardia (EAT) often resists medical therapy, making radiofrequency catheter ablation (RFCA) the preferred treatment. This study reviewed the records of 35 patients who underwent electrophysiologic studies (EPS) and 39 RFCA procedures for EAT during a 10-year period. Of the 35 patients, 10 (28%) presented with decreased ventricular function and tachycardia-induced cardiomyopathy (TIC).

Successful catheter ablation of reentrant junctional tachycardia in a patient with asplenia syndrome before total cavo-pulmonary connection.

Asplenia syndrome is commonly associated with complex structural cardiac malformations, and junctional tachycardia (JT), which may compromise hemodynamic status, has been reported in association with asplenia syndrome.(1) We report successful radiofrequency catheter ablation of reentrant JT in a patient with asplenia syndrome.

Spontaneous closure of ventricular septal defects followed up from <3 months of age.

Background: This study evaluates the incidence and timing of spontaneous closure (SC) of ventricular septal defect (VSD) using Doppler color flow mapping.

Methods: A total of 225 infants (mean age 30 days) were diagnosed with uncomplicated VSD: 31 (14%) subpulmonary VSD, 159 (70%) perimembranous, and 35 (16%) muscular. The patients were divided into two groups according to the presence or absence of congestive heart failure (CHF).

Catheter ablation for a right anteroseptal accessory pathway in a patient with an endocardial cushion defect.

We report a radiofrequency catheter ablation for atrioventricular reentrant tachycardia in a patient with a right anteroseptal accessory pathway complicating an endocardial cushion defect. His bundle potential was recorded 20 mm posterior to the accessory pathway. In the presence of associated congenital heart disease, it is very important to understand the anatomy of the conduction system prior to radiofrequency catheter ablation.