Two-Year Outcomes of Primary Prophylactic Use of Defibrillators for Ischemic and Non-Ischemic Cardiomyopathy - Propensity Score-Matched Analysis From the Nippon Storm Study.

Background: In 2016, the DANISH study reported negative results regarding the efficacy of implantable cardioverter-defibrillators (ICDs) in patients with non-ischemic cardiomyopathy (NICM) and reduced left ventricular ejection fraction (LVEF). In this study we determined the efficacy of using ICDs for primary prophylaxis in patients with NICM.

Methods and results: We selected 1,274 patients with underlying cardiac disease who were enrolled in the Nippon Storm Study.

Effectiveness of Implantable Cardioverter Defibrillator in Patients With Non-Ischemic Heart Failure With Systolic Dysfunction - Subanalysis of the Nippon Storm Study.

Background: The prospective observational Nippon Storm Study aggregated clinical data from Japanese patients receiving implantable cardioverter-defibrillator (ICD) therapy. This study investigated the usefulness of prophylactic ICD therapy in patients with non-ischemic heart failure (NIHF) enrolled in the study.

Methods and results: We analyzed 540 NIHF patients with systolic dysfunction (left ventricular ejection fraction <50%).

High platelet reactivity is a predictor of left ventricular remodelling in patients with acute myocardial infarction.

Aims: Acute myocardial infarction (AMI) is associated with left ventricular remodelling (LVR), which leads to progressive heart failure. Platelets play a pivotal role in promoting systemic and cardiac inflammatory responses during the complex process of myocardial wound healing or repair following AMI. This study aimed to investigate the impact of platelet reactivity immediately after primary percutaneous coronary intervention (PCI) on LVR in AMI patients with ST-segment (STEMI) and non-ST-segment elevation (NSTEMI).

Electrical Storm as an Independent Mortality Risk in Patients with Preserved or Moderately Reduced Left Ventricular Function.

Electrical storm (ES), defined by 3 or more occurrences of ventricular arrhythmias within 24 hours, has been shown to be associated with an increased risk of mortality; however, detailed information remains lacking. We aimed to examine the incidence and determinants of ES and its impact on mortality in patients enrolled in the nationwide implantable cardioverter-defibrillator (ICD) registry.We studied 1,256 patients (age 65 ± 12 years) who had structural heart disease with an ICD.

Anti-tachycardia pacing for non-fast and fast ventricular tachycardias in individual Japanese patients: From Nippon-storm study.

Background: Anti-tachycardia pacing (ATP) delivered from an implantable device is a useful tool to terminate ventricular tachycardia (VT). But its real-world efficacy for those patients having multiple VTs with varying VT rates has not been fully studied.

Methods: Using the Nippon-storm study database, efficacy of patient-by-patient basis ATP programing for Japanese patients having both non-fast (120-187 bpm) and fast VT (≥188 bpm) was assessed.

Comparison of 2-year outcomes between primary and secondary prophylactic use of defibrillators in patients with coronary artery disease: A prospective propensity score-matched analysis from the Nippon Storm Study.

Background: The Nippon Storm Study was a prospective observational study designed to gather clinical data on implantable cardioverter-defibrillator (ICD) therapy in Japanese patients.

Objective: The purpose of this subanalysis was to compare the incidence of ICD therapy in patients with left ventricular dysfunction owing to coronary artery disease (CAD) for primary and secondary prophylaxis of sudden cardiac death.

Methods: We analyzed data of 493 patients with CAD and ICDs (men, 87%; age, 68 ± 10 years; left ventricular ejection fraction, 36% ± 13%; primary prophylaxis, 36%).

Risk factors for the first and second inappropriate implantable cardioverter-defibrillator therapy.

Introduction: Various risk factors for the first inappropriate implantable cardioverter-defibrillator (ICD) therapy event have been reported, including a history of atrial fibrillation/atrial flutter (AF/AFL), younger age, and multiple zones. Nonetheless, which factors are concordant with real-world data has not been clarified, and risk factors for the second inappropriate ICD therapy event have not been well examined. This study aimed to clarify the risk factors for the first and second inappropriate ICD therapy events.

Impact of atrial fibrillation/flutter on outcomes of patients with implantable cardioverter defibrillators: A sub-analysis of the Nippon Storm study.

Background: Implantable cardioverter-defibrillator and cardiac resynchronization therapy using a defibrillator (ICD/CRT-D) are established means of reducing mortality due to ventricular arrhythmia. Although atrial fibrillation/flutter (AF) is the most common cardiac arrhythmia in patients with heart disease, the impact of AF on the prognosis of patients with ICD/CRT-D remains controversial.

Methods And Results: We analyzed data from the Nippon Storm Study, a prospective observational study of 1570 patients that was conducted at 48 Japanese ICD centers.

Efficacy of antitachycardia pacing for electrical storms in patients with implantable defibrillators.

Introduction: Although antitachycardia pacing (ATP) is effective in terminating ventricular tachyarrhythmias in patients with implantable cardioverter defibrillators (ICDs), the efficacy of ATP during an electrical storm (ES) and the positive impact on all-cause mortality have not been fully elucidated.

Methods And Results: From 2010 to 2012, 1570 patients who underwent ICD implantation in 48 ICD centers in Japan were enrolled in the study and prospectively followed up. Patients with long QT syndrome, Brugada syndrome, and idiopathic ventricular fibrillation were excluded.

Comparison of second appropriate defibrillator therapy occurrence in patients implanted for primary prevention and secondary prevention - Sub-analysis of the Nippon Storm Study.

Background: Patients with implantable cardioverter defibrillator (ICD) use for primary prevention (primary prevention patients) of sudden cardiac death have lower incidence of appropriate ICD therapy (app-Tx) compared with those with ICD use for secondary prevention (secondary prevention patients). However, detail analysis of a second app-Tx after a first app-Tx is still lacking.

Objective: This study aimed to compare the incidence of a second app-Tx in primary vs secondary prevention patients.

Systematic Evaluation of Variant Using ACMG/AMP Guidelines and Risk Stratification in Long QT Syndrome Type 1.

- Mutation/variant-site specific risk stratification in long-QT syndrome type 1 (LQT1) has been well investigated, but it is still challenging to adapt current enormous genomic information to clinical aspects caused by each mutation/variant. We assessed a novel variant-specific risk stratification in LQT1 patients. - We classified a pathogenicity of 141 variants among 927 LQT1 patients (536 probands) based on the American College of Medical Genetics and Genomics (ACMG) and Association for Molecular Pathology (AMP) guidelines and evaluated whether the ACMG/AMP-based classification was associated with arrhythmic risk in LQT1 patients.

Patient-by-patient basis anti-tachycardia pacing for fast ventricular tachycardia with structural heart diseases.

Background: Anti-tachycardia pacing (ATP) delivered from an implantable device is an important tool to terminate ventricular tachycardia (VT). But its real-world efficacy for fast VT has not been fully studied.

Methods: Using the database of Nippon-storm study, effect of patient-by-patient basis ATP programming for fast VT (≥188 bpm) was assessed for the patients with structural heart diseases.

Successful Nonsurgical Treatment of Acute Peritonitis Caused by a Large Thrombosis of the Superior Mesenteric Vein in a 25-year-old Woman.

Superior mesenteric venous thrombosis (SMVT), which results from various etiologies, including coagulation disorders, can be diagnosed early using advanced imaging technology. However, few reports have described the nonsurgical treatment of acute peritonitis caused by SMVT. We encountered a young woman whose history included abdominal pain and daily oral contraceptives and who presented with acute peritonitis caused by SMVT.

Association of Genetic and Clinical Aspects of Congenital Long QT Syndrome With Life-Threatening Arrhythmias in Japanese Patients.

Importance: Long QT syndrome (LQTS) is caused by several ion channel genes, yet risk of arrhythmic events is not determined solely by the responsible gene pathogenic variants. Female sex after adolescence is associated with a higher risk of arrhythmic events in individuals with congenital LQTS, but the association between sex and genotype-based risk of LQTS is still unclear.

Objective: To examine the association between sex and location of the LQTS-related pathogenic variant as it pertains to the risk of life-threatening arrhythmias.

Association between changes in platelet reactivity during elective percutaneous coronary intervention and periprocedural myocardial infarction: A pilot study.

Background: High platelet reactivity before percutaneous coronary intervention (PCI) reportedly increases the risk of PCI-related myocardial infarction (PMI) following elective PCI. We conducted a pilot study to evaluate changes in platelet reactivity during PCI and their association with the incidence of PMI.

Methods: In total, 133 consecutive patients undergoing elective PCI after pretreatment with dual antiplatelet therapy for at least 7 days were prospectively enrolled.

Significant impact of electrical storm on mortality in patients with structural heart disease and an implantable cardiac defibrillator.

Background: Electrical storm (E-Storm), defined as multiple episodes of ventricular arrhythmias within a short period of time, is an important clinical problem in patients with an implantable cardiac defibrillator (ICD) including cardiac resynchronization therapy devices capable of defibrillation. The detailed clinical aspects of E-Storm in large populations especially for non-ischemic dilated cardiomyopathy (DCM), however, remain unclear.

Objective: This study was performed to elucidate the detailed clinical aspects of E-Storm, such as its predictors and prevalence among patients with structural heart disease including DCM.

Impact of premature activation of the right ventricle with programmed stimulation in Brugada syndrome.

Background: In Brugada syndrome (BrS), it has been reported that delayed activation in the RV is related to the development of type-1 ECG, which is more critical than type-2. On the other hand, the coexistence of complete right bundle-branch block (CRBBB), which also causes delayed activation in the RV, sometimes makes typical BrS ECG misleading. We hypothesized that premature stimulation of the RV can unmask the influence of delayed activation in the RV and convert the morphology of ECG in BrS patients.

Genotype-Phenotype Correlation of Mutation for the Clinical and Electrocardiographic Characteristics of Probands With Brugada Syndrome: A Japanese Multicenter Registry.

Background: The genotype-phenotype correlation of mutations as a predictor of cardiac events in Brugada syndrome remains controversial. We aimed to establish a registry limited to probands, with a long follow-up period, so that the genotype-phenotype correlation of mutations in Brugada syndrome can be examined without patient selection bias.

Methods: This multicenter registry enrolled 415 probands (n=403; men, 97%; age, 46±14 years) diagnosed with Brugada syndrome whose gene was analyzed for mutations.