Prominent atrial fibrosis and its correlation with atrial tachyarrhythmia and dilated atrium long after classic Fontan surgery.

Background: The progression of atrial fibrosis long after Fontan surgery is unclear. This study aimed to evaluate the degree of atrial fibrosis long after the classic Fontan procedure and to investigate the factors associated with atrial fibrosis.

Methods: We obtained atrial free wall specimens resected at Fontan conversion from 43 patients (Fontan group) and studied the degree of atrial fibrosis, along with its association with atrial tachycardia/fibrillation (AT/AF) and other clinical parameters, compared with those of the control group without heart disease (n=6).

Induced Pluripotent Stem Cell-Derived Cardiomyocytes with R1623Q Mutation Associated with Severe Long QT Syndrome in Fetuses and Neonates Recapitulates Pathophysiological Phenotypes.

The R1623Q mutation is one of the most common genetic variants associated with severe congenital long QT syndrome 3 (LQT3) in fetal and neonatal patients. To investigate the properties of the R1623Q mutation, we established an induced pluripotent stem cell (iPSC) cardiomyocyte (CM) model from a patient with LQTS harboring a heterozygous R1623Q mutation. The properties and pharmacological responses of iPSC-CMs were characterized using a multi-electrode array system.

Statement for electrophysiological procedures under the COVID-19 pandemic from the Japanese heart rhythm society task force.

COVID-19 is a global catastrophe with markedly reduced health and economy of human civilization. Heart rhythm disorder has also been impacted by this disease. This statement is the universal criteria for EP procedures in the new era, which we will face during COVID-19 pandemic.

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.

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.

Successful staged tricuspid valve replacement following cardiac resynchronization therapy in a congenitally corrected transposition of the great arteries.

Simple tricuspid valve surgery for complex heart disease with systemic right ventricular dysfunction is a high-risk procedure; however, staged tricuspid valve surgery following cardiac resynchronization therapy seems to be a beneficial method to expect reverse systemic ventricular remodeling.

Prevalence and risk factors of sleep apnea in adult patients with congenital heart disease.

Background: Although sleep apnea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with congenital heart disease are limited.

Methods: In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia.

Prevalence and risk factors of sleep apnoea in adult patients with CHD.

Background: Although sleep apnoea is an important disorder associated with cardiac events, data regarding its prevalence and risk factors in adult patients with CHD are limited.

Methods: In this study, patients underwent a sleep study in the hospital. Indications for admission were classified as heart failure, diagnostic catheterisation, interventional catheterisation, or arrhythmia.

Thoracic endovascular aortic repair of a late aortic aneurysm after patch repair of an interrupted aortic arch.

We reported a successful case of thoracic endovascular aortic repair of a late aortic aneurysm after patch repair of an interrupted aortic arch and favourable outcome 4 years after the procedure.

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.

Prognostic value of multiple biomarkers for cardiovascular mortality in adult congenital heart disease: comparisons of single-/two-ventricle physiology, and systemic morphologically right/left ventricles.

Although there are many biomarkers for heart failure, limited data are available regarding their prognostic value in adult congenital heart disease (ACHD). We investigated the potential of various biomarkers to predict ACHD mortality in a single-center, retrospective cohort study. Blood levels of neurohormones [angiotensin II, endothelin-1 (ET-1), norepinephrine (NE), aldosterone, and plasma renin activity]; inflammatory biomarkers [high-sensitivity C-reactive protein (hs-CRP), high-sensitivity tumor necrosis factor, soluble TNF receptor type I and II (sTNF-RI and sTNF-RII), and interleukin-6 (IL-6)]; and brain natriuretic peptide (BNP) were measured in 103 ACHD patients (median age 28 years).

Blood coagulation abnormalities and the usefulness of D-dimer level for detecting intracardiac thrombosis in adult Fontan patients.

Background: Coagulation abnormality is associated with a high incidence of intracardiac thrombus (ICT) and systemic thromboembolism in Fontan patients. The biomarker for detecting ICT is currently unknown.

Methods: We retrospectively investigated the underlying coagulation abnormality and useful biomarkers to screen for ICT in adult Fontan patients.

High prevalence of cardiovascular risk factors in children and adolescents with Williams-Beuren syndrome.

Background: A high incidence of cardiovascular (CV) risk factors has been reported in adults with Williams-Beuren syndrome (WS). However, the prevalence of these factors in children and adolescents with WS is unknown. Therefore, the purpose of this study was to evaluate the prevalence of CV risk factors in these patients.

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.

Relationships among red cell distribution width, anemia, and interleukin-6 in adult congenital heart disease.

Background: Red cell distribution width (RDW) is known to be associated with anemia and mortality in cardiovascular diseases, while anemia itself is related to increased mortality. RDW may also be related to cytokine activation. We investigated the potential of RDW to predict anemia-adjusted mortality in patients with adult congenital heart disease (ACHD) and we evaluated the relationships among RDW, anemia, and interleukin-6 (IL-6).

Cardiac strangulation from epicardial pacemaker leads: diagnosis, treatment, and prevention.

Cardiac strangulation from epicardial leads is a rare but potentially lethal complication associated with epicardial pacemaker implantation in growing children. Early diagnosis and quick lead removal are required to rescue patients suffering from cardiac strangulation. In April 2013, the Japanese Association for Thoracic Surgery, Japan Society of Arrhythmia and Japan Cardiovascular Surgery Society published information about cardiac strangulation from epicardial leads in children on the home page of each organization's website to increase awareness of this condition Here, we review ten previously reported cases and discuss cardiac strangulation from the point of view of the pediatric cardiologist.

Successful Fontan conversion combined with cardiac resynchronization therapy for a case of failing Fontan circulation with ventricular dysfunction.

Although Fontan conversion combined with cardiac resynchronization therapy appears to be an effective surgical solution for the management of failing Fontan circulation with refractory atrial arrhythmia and cardiac dysfunction due to dyssynchronous ventricular wall motion, limited data are available on the mid- to long-term results of this treatment. We report our successful experience with Fontan conversion combined with cardiac resynchronization therapy in a male patient with failing Fontan circulation who showed favourable outcomes 5 years after the operation.

Pacing device therapy in infants and children: a review.

The number of pediatric pacemakers implanted is still relatively small. Children requiring pacing therapy have characteristics that are distinct from those of adults, including physical size, somatic growth, and cardiac anomalies. Considering these features, long-term follow-up of pediatric pacemaker implantation is necessary.