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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 RYR2. 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.

Abstract Average beat interval (BI) and beat interval variability (BIV) are primarily determined by mutual entrainment between the autonomic-nervous system (ANS) and intrinsic mechanisms that govern sinoatrial node (SAN) cell function. While basal heart rate is not affected by age in humans, age-dependent reductions in intrinsic heart rate have been documented even in so-called healthy individuals. The relative contributions of the ANS and intrinsic mechanisms to age-dependent deterioration of SAN function in humans are not clear. We recorded ECG on patients (n = 16

Duct-dependent systemic circulation is accompanied by a right-to-left ductal shunt, at least during systole. Although observations of paradoxical continuous left-to-right shunts in duct-dependent systemic circulation have been reported, the mechanism remains unclear. We report a continuous left-to-right ductal shunt throughout the cardiac cycle during the initial recovery phase from circulatory collapse and right ventricular (RV) dysfunction due to ductal closure in an infant with hypoplastic left heart and severe aortic coarctation. Further recovery improved his RV function and changed the ductal flow from continuous left-to-right to bidirectional, which is usually seen in duct-dependent systemic circulation. Marked RV dysfunction may contribute to the continuous left-to-right ductal shunt. A continuous left-to-right ductal shunt should not be used to rule out duct-dependent systemic circulation.

Abstract The characteristic shape of the T-wave in congenital long-QT syndrome type 3 (LQTS3) is considered a late-onset T-wave. We analyzed the difference in the shapes of the T-waves of V5 in the electrocardiograms (ECGs) of LQTS3 cases and normal subjects using generalized Procrustes analysis. The J and Q points of LQTS3 cases are shifted to the upper left compared to those of normal subjects. SdFmax is the point on the ECG where the second derivative is first maximized. SdFmax is the point where the change in the slope of the ascending limb of the T-wave is maximized. SdFmax in LQTS3 cases is shifted to the lower right compared to normal subjects. The interval from J to SdFmax in LQTS3 cases is expanded compared with that of normal subjects. From principal component analysis of the Procrustes mean shape of the T-wave landmarks, the second principal component shows a shift of SdFmax to the lower right. These results can quantitatively explain why the T-wave of LQTS3 cases looks like a late-onset T-wave. After being fitted to a multivariate logistic regression model, LQTS3 cases and normal subjects can be distinguished by the second independent component.

Abstract Aims This study aimed to assess the value of the hepatocyte growth factor (HGF) as an independent predictor of a Fontan circulation failure. Methods and results This retrospective case–control study included 34 consecutive patients (19 men and 15 women) who underwent a post‐operative cardiac catheterization after a Fontan operation at the Saitama Medical University International Medical Center between April 2017 and December 2019. We divided the patients into two groups according to the HGF level: HGF 0.405 ng/mL could detect the need for a catheter intervention with 75.0% sensitivity and 83.3% specificity. A multivariable regression analysis showed that an elevated HGF was an independent predictor of an elevated CVP (β‐coefficient 21.2, SE 5.5, P = 0.0005) and decreased SvO2 (β‐coefficient −92.9, SE 12.4, P

Abstract Desmin‐related myopathy (DRM) is a rare heritable cardiac and skeletal muscle disease caused by mutations in the desmin gene (DES). DRM is generally characterized by skeletal muscle weakness, conduction disturbance, and dilated cardiomyopathy. However, the clinical cardiac phenotypes of DRM are not yet fully understood. Herein, we report the first case of DRM with the de novo missense DES mutation, R454W, that is characterized by left ventricular non‐compaction cardiomyopathy, progressive cardiac conduction defect, spontaneous coronary artery dissection, and no skeletal muscle weakness. Our case findings suggest that clinicians should genetically test patients who have cardiomyopathy, progressive cardiac conduction defect, and coronary artery dissection, even if the patient has neither family history of DRM nor skeletal muscle symptoms.

Abstract Background The ablation index (AI) is reported to be useful for a durable pulmonary vein isolation (PVI). However, there have been no studies investigating the relationship between the power, contact force (CF), AI, and steam pops. Methods Using an in vitro model, ablation energy was delivered until a steam pop occurred and the time to the steam pop and AI when the steam pop occurred were measured. The experiment was performed with a combination of various powers (20, 30, 40, and 50 W) and contact forces (CFs) (10, 30, and 50 g) 20 times for each setting. The analysis consisted of two protocols. The first protocol was a comparison between the ablation power and several parameters under the same CF (10, 30, and 50 g). The second protocol was a comparison between the CF and several parameters under the same power (20, 30, 40, and 50 W). The correlation between the lesion formation and ablation parameters was evaluated. Results The AI value when steam pops occurred varied depending on the ablation settings. All AI median values were

Abstract We report a case of an 18‐year‐old male with a postsurgical lateral tunnel (LT) total cavopulmonary connection (TCPC) and supraventricular tachycardia (SVT). Patients after an LT TCPC have complicated suture lines and a considerable area of damaged myocardium in the LT, which could become a complex arrhythmogenic substrate of tachycardias. Detailed three‐dimensional (3D) mapping of the LT and atrium is important for a successful ablation. In this patient, successful catheter ablation of two types of complex tachycardias was accomplished using an ultra‐high density 3D mapping system inside the LT.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by emotions or exercise in patients without organic heart disease and may be polymorphic or bidirectional in nature. The prognosis of CPVT is not good, and therefore prevention of sudden death is of utmost importance. Genetic variants of CPVT include RyR2, CASQ2, CALM2, TRD, and possibly KCNJ2 and ANK2 gene mutations. Hypotheses that suggest the causes of CPVT include weakened binding of FKBP12.6 and RyR2, a store overload-induced Ca2+ release (SOICR), unzipping of intramolecular domain interactions in RyR2, and molecular and functional abnormalities caused by mutations in the CASQ2 gene. The incidence of an RyR2 anomaly in CPVTs is about 35–79%, whereas anomalies in the CASQ2 gene account for 3–5% CPVTs. The ping-pong theory, suggesting that reciprocating delayed after depolarization induces bigeminy of the right and left bundle branches, may explain the pathogenesis of bidirectional ventricular tachycardia. Flecainide, carvedilol, left sympathetic nerve denervation, and catheter ablation of the PVC may serve as new therapeutic strategies for CPVT while gene-therapy may be applied to some types of CPVT in the future. Although, not all sudden cardiac deaths in CPVT patients are currently preventable, new medical and interventional therapies may improve CPVT prognosis.

Background: There is little information regarding appropriate therapies for coronary artery disease (CAD) patients with implantable devices – such as implantable cardioverter-defibrillators (ICDs), cardiac resynchronization therapy devices (CRT-Ds) and cardiac resynchronization therapy pacemaker (CRT-P) devices – in Japan. To address this lack of information, we have launched the Japan Implantable Devices in Coronary Artery Disease (JID-CAD) study. Methods: This study has been designed as a prospective, multicenter, non-randomized and observational investigation. All patients will be followed up every six months over a two-year period. The primary endpoint will be the administration of appropriate device therapy. Secondary endpoints include administration of inappropriate therapy, death, hospitalization, or cardiovascular events. Conclusion: In this report, we discuss the current clinical situation of appropriate therapy, and how it is influenced by catheter ablation and revascularization therapy in patients with CAD.

Background: Device therapy is an established therapy for preventing sudden cardiac death or managing refractory congestive heart failure in adults. However, it is performed less commonly in pediatric populations. This review aimed to examine the indications and problems associated with implantable cardioverter-defibrillator (ICD) and cardiac resynchronization therapy (CRT) device implantations in pediatric and congenital heart disease (CHD) patients. Results: In a multicenter study in Japan, the cardiac condition of CHD patients improved by 83% after CRT device implantation. The need for CRT devices is more common in children than in adults. After ICD implantation, 44% of the patients experienced appropriate shocks, and epicardial lead implantation was performed in one-third of the patients. Nonendocardial electrode placement is mandatory for ICD implantation in small infants and patients with certain CHDs. Although inappropriate ICD discharges due to sinus tachycardia or other supraventricular tachycardias are common in children, the indication for ICD implantation may be higher than that reported in children. Conclusions: Despite the limited experience, limitations of device implantations owing to the size of the devices, and necessity for nonendocardial electrode placement, device implantations are required in more pediatric and CHD patients than expected.

Background: Vector-projected 187-channel electrocardiograms (ECGs) were recorded in 45 patients with a Brugada-type ECG to evaluate spatial and transmural repolarization and dispersion of action potential duration in Brugada syndrome (BS). Methods: Corrected recovery time (RT-c, R wave peak to the first positive maximum derivative of the T wave with Bazett correction) and RT-c dispersion were calculated. The corrected T peak-end interval (T(p-e)-c, T wave peak to the end of the T wave with Bazett correction) and T(p-e)-c dispersion were calculated. Results: RT-c dispersion and T(p-e)-c interval were longer in patients with a type 1 ECG, but there was no significant difference in Tp-e dispersion between patients with a type 1 and those with a type 2/3 ECG. No significant correlation was noted between RT-c dispersion, T(p-e)-c dispersion, and symptoms. Late potentials (P=0.023) and a family history of sudden cardiac death (P=0.0017) were correlated with symptoms. Conclusions: Spatial dispersion of repolarization may constitute the electrocardiographic pattern of the Brugada type ECG and conduction disturbance in addition to repolarization abnormality may contribute to the development of malignant ventricular tachyarrhythmias.

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterized by episodic ventricular tachycardia induced by adrenergic stress. Although β-blockers are used as first-line therapy, their therapeutic effects are largely incomplete. Flecainide has recently been shown to modify the molecular defects in CPVT. The aim of this study was to investigate the effects of flecainide as an add-on to conventional therapy on exercise-induced ventricular arrhythmia and compare them with those of conventional therapy alone. Methods: The study included 5 CPVT patients with a mutation in RYR2. They experienced episodic arrhythmic events despite conventional β-blocker therapy and were therefore given flecainide in addition. The effects of the addition of flecainide therapy on ventricular arrhythmia during exercise testing were compared with those of conventional therapy alone. Results: Both β-blockers alone and with additional flecainide increased the maximal workload attained at the onset of ventricular arrhythmia; however, only flecainide increased the sinus rate at the onset of ventricular arrhythmias. Furthermore, flecainide increased the exercise capacity by preventing exercise-induced arrhythmias. During a follow-up period of 17±2 months, 1 patient experienced recurrent arrhythmic episodes that were associated with noncompliance. All patients reported improvements in their ability to perform the activities of daily living. Conclusion: Flecainide effectively reduced ventricular arrhythmias via a mechanism that differs from that of β-blockers in genotype-positive patients with CPVT. The specific effects of flecainide may be critical in the improvement noted in the patients' ability to perform daily activities.

Background: Brugada syndrome is a distinct form of idiopathic ventricular fibrillation. We retrospectively investigated the incidence and clinical implications of supraventricular tachyarrhythmia in patients with Brugada syndrome. Methods: We reviewed 69 consecutive cases of Brugada syndrome, 11 of which had a history of syncope. Results: Seven patients (10.1%) had clinically documented supraventricular tachyarrhythmia: 3 patients with atrioventricular reentrant tachycardia, 2 with atrioventricular nodal reentrant tachycardia, and 2 with paroxysmal atrial fibrillation. The prevalence of a spontaneous type 1 Brugada electrocardiogram and symptoms (history of syncope, presyncope, documented ventricular tachyarrhythmia, or aborted sudden cardiac death) were significantly higher, and QRS duration was significantly longer in patients without than in those with supraventricular tachyarrhythmia. The PR and AH intervals were also longer in patients without than in those with supraventricular tachyarrhythmia, but the differences were not statistically significant. Conclusion: The arrhythmogenic substrate in Brugada syndrome may not be restricted to the ventricles. Palpitations in patients with this syndrome should raise the question of supraventricular tachyarrhythmia. Conversely, in patients with supraventricular tachyarrhythmia and aborted sudden cardiac death or syncope not related to supraventricular tachyarrhythmia, Brugada syndrome should be considered a possible additional electrophysiologic abnormality.

INTRODUCTION:Human induced pluripotent stem cells (hiPSCs) offer a unique opportunity for disease modeling. However, it is not invariably successful to recapitulate the disease phenotype because of the immaturity of hiPSC-derived cardiomyocytes (hiPSC-CMs). The purpose of this study was to establish and analyze iPSC-based model of catecholaminergic polymorphic ventricular tachycardia (CPVT), which is characterized by adrenergically mediated lethal arrhythmias, more precisely using electrical pacing that could promote the development of new pharmacotherapies. METHOD AND RESULTS:We generated hiPSCs from a 37-year-old CPVT patient and differentiated them into cardiomyocytes. Under spontaneous beating conditions, no significant difference was found in the timing irregularity of spontaneous Ca2+ transients between control- and CPVT-hiPSC-CMs. Using Ca2+ imaging at 1 Hz electrical field stimulation, isoproterenol induced an abnormal diastolic Ca2+ increase more frequently in CPVT- than in control-hiPSC-CMs (control 12% vs. CPVT 43%, p

In this study, the prevalence and prognostic significance of the early repolarization (ER) pattern in the inferolateral leads in patients with Brugada syndrome were investigated. Clinical, genetic, and electrophysiological data were collected and analyzed from 69 individuals with either a spontaneous or drug-induced Brugada type 1 electrocardiogram (ECG) pattern. An ER pattern was defined as J-point elevation at least 0.1 mV from the baseline in at least 2 inferior or lateral leads. The presence of late potentials and inducibility of ventricular fibrillation (VF) by programmed stimulation were compared between patients with and without a J wave. Follow-up data, including outcome events, were obtained for all patients. An ER pattern was observed in the inferolateral leads in 6 patients with a spontaneous Brugada type 1 ECG pattern and in 1 patient with a drug-induced Brugada type 1 ECG pattern. There was no significant intergroup difference in symptoms, family history of sudden cardiac death, prevalence of late potentials, or inducibility of VF. No patient with the ER pattern developed a cardiac event during the mean follow-up period of 73.6±38.1 months. The ER pattern in the inferolateral leads is not uncommon in Brugada syndrome; however, the presence of a J wave does not appear to be associated with subsequent arrhythmic events in patients with Brugada syndrome.