Search

J waves may be observed during coronary angiography (CAG), but they have not been fully studied. We investigated the characteristics of J waves in 100 consecutive patients during CAG. The patients and their family members had no history of cardiac arrest. Approximately 60% of patients had ischemic heart disease, previous myocardial infarction, or angina pectoris, but at the time of this study, the right coronary artery was shown to be normal or patent after stenting. Electrocardiogram was serially recorded to monitor J waves and alteration of the QRS complex during CAG. In 12 patients (12%), J waves (0.249 ± 0.074 mV) newly appeared during right CAG, and in another 13 patients (13%), preexisting J waves increased from 0.155 ± 0.060 mV to 0.233 ± 0.133 mV during CAG. Left CAG induced no J waves or augmentation of J waves. Distinct alterations were observed in the QRS complex during CAG of both coronary arteries. Mechanistically, myocardial ischemia induced by contrast medium was considered to result in a local conduction delay, and when it occurred in the inferior wall, the site of the late activation of the ventricle, the conduction delay was manifested as J waves. In conclusion, J waves were confirmed to emerge or increase during angiography of the right but not the left coronary artery. Myocardial ischemia induced by contrast medium caused a local conduction delay that was manifested as J waves in the inferior wall, the site of the late activation of the ventricle.

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. The patients were classified into reduced ejection fraction (EF < 35%; 657 (52%) patients) and preserved or moderately reduced EF (EF >= 35%; 599 (48%) patients). ES occurred in 49 (7%) and 36 (6%) patients in the EF < 35% and EF >= 35% groups (log-rank P = 0.297) during the median follow-up of 2.3 years. ICD with resynchronization therapy was associated with a lower incidence of ES in patients with EF < 35%. Non-ischemic heart disease and diuretics were associated with ES in patients with EF >= 35%. During the follow-up, 10/49 (20%) patients with ES and 80/608 patients (13%) without ES died in patients with EF < 35%, while 7/36 (19%) patients with ES and 38/563 patients (7%) without ES died in those with EF >= 35%. We have created 4 Cox multivariate models. All models showed approximately 2-fold higher hazard ratios in patients with EF >= 35% compared to EF < 35%. Our study showed that the determinants of ES differed between EF < 35% and EF >= 35%. The impact of ES for mortality was numerically higher in EF >= 35% than in EF < 35%, although a significant interaction was not detected.

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. According to the useful criteria of ≥50% success termination by ATP, patients were divided into three subgroups; success ≥50% for both non‐fast and fast VT (both useful), ≥50% only for non‐fast VT (non‐fast VT useful), or ≥50% for neither non‐fast nor fast VT (neither useful). Results During a median follow‐up of 28 months, ATP terminated 184 of the 203 non‐fast VT episodes (91%) and 86 of the 113 fast VT episodes (76%) in all 41 patients. In the patient‐by‐patient analysis, efficacy of ATP was not different between non‐fast and fast VT in most of the patients (36/41 = 88%); 32 patients were in the both useful and four other patients in the neither useful. Neither ischemic nor non‐ischemic structural heart disease was associated with the ATP efficacy, whereas LVEF more than 37.0% and non‐prescribed amiodarone were characteristics of the patients classified into the both useful. Conclusions ATP well terminated both non‐fast and fast VT occurring in individual Japanese patients with various structural heart diseases in the real‐world device treatment and this finding further supports ATP programing for all device tachycardia detection zones in most patients with multiple VTs.

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 fraction50%). Propensity score matching was used to select patient subgroups for comparison; 126 patients were analyzed in each of the primary (PP) and secondary (SP) prophylaxis groups. The incidence of appropriate ICD therapy during follow-up in the PP and SP groups was 21.4% and 31.7%, respectively (P=0.044). The incidence of electrical storm (ES) was higher in SP than PP patients (P=0.024). Cox proportional hazard analysis revealed that increased serum creatinine in SP patients (hazard ratio [HR] 1.18; 95% confidence interval [CI] 1.02-1.33; P=0.013) and anemia in PP patients (HR 0.92; 95% CI 0.86-0.98; P=0.008) increased the likelihood of appropriate ICD therapy, whereas long-lasting atrial fibrillation in PP patients (HR, 0.64 [95% CI, 0.45-0.91], P=0.013) decreased that likelihood.In propensity score-matched Japanese NIHF patients, the incidence of appropriate ICD therapy and ES was significantly higher in SP than PP patients. Impaired renal function in SP patients and anemia in PP patients increased the likelihood of appropriate ICD therapy, whereas long-lasting atrial fibrillation reduced that likelihood in PP patients.

P-wave indices have been not fully studied in subtypes of ischemic stroke. We compared P-wave indices among embolic stroke, lacunar stroke and the control.P-wave duration, advanced interatrial block (aIAB) defined as P-wave duration ≥120 ms and biphasic (positive negative) morphology in inferior leads, and P-terminal force in lead V1 (PTFV1) were measured at the time of the first episode of cardioembolic stroke in 81 patients with paroxysmal atrial fibrillation (PAF), and in 64 patients with lacunar stroke, and compared with 100 control subjects. The latter two groups had no episode of PAF.The age of participants was 76 ± 11 years. Age, sex distribution, body mass index and CHADS2 score were comparable among three groups. Maximum P-wave duration, the longest across 12 leads, was significantly prolonged in cardioembolic and lacuna stroke compared to the control; 118 ± 12 ms and 118 ± 11 ms vs. 110 ± 11 ms, respectively (P 0.0001). P-wave duration ≥120 ms and aIAB were more prevalent in ischemic stroke groups than the control, and associated with a higher Odds ratio for stroke, more so in cardioembolic stroke. However, PTFV1 value and the prevalence of PTFV1 ≥ 4.0 ms·mV were significantly not different among the three groups. Abnormal P-wave duration and aIAB indicating the presence of atrial myopathy were present in cardioembolic and lacuna stroke.Atrial myopathy was present in cardioembolic and lacunar stroke, but it can't be the direct cause of small vessel occlusion in lacunar stroke. Roles of atrial myopathy in each subtype of ischemic stroke should be studied.

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. The prevalence of shocks during ESs and impact on the all-cause mortality were evaluated. During a median follow-up of 28 months, there were 127 ESs in 84 patients. Of those 127 ESs, 80 ESs (63%) in 37 patients were treated by only ATP and the remaining 47 ESs in 47 patients required at least one shock. The lower ventricular rate of the initial arrhythmia during ES (odds ratio [OR]: 1.02 per unit; 95% confidence interval [CI]: 1.00-1.04; p = .02) and narrower QRS complex (OR: 1.03 per unit; 95% CI: 1.01-1.06; p

Near-infrared spectroscopy with intravascular ultrasound (NIRS)-IVUS enables precise detection of lipid core burden. Intracoronary electrocardiography (ECG) can detect slight ischemia during percutaneous coronary intervention (PCI), indicating microvascular dysfunction (MD) by distal embolization, etc. Thus, this study aimed to investigate whether plaques with a low max-lipid core burden index (LCBI) at 4 mm (LCBI4mm) influence MD, using intracoronary ECG. We enrolled 40 consecutive patients who underwent PCI for stable angina pectoris (SAP) due to stenosis of the proximal segment of the left anterior descending artery in this study. Max-LCBI4mm was measured for each culprit lesion. Gray-scale IVUS data including plaque burden were measured. Intracoronary ECG was performed to measure the time from the initiation of ST-segment elevation from the isoelectric baseline after stent balloon inflation to the return of the ST-segment to the isoelectric baseline after the deflation of the stent balloon, which was defined as the severity of the MD. The patients were divided into two groups according to median max-LCBI4mm of 120 as follows: low- [n = 20] and high- [n = 20] LCBI groups. The overall mean Max-LCBI4mm was 120 ± 86. No differences in baseline characteristics, including prevalence of dyslipidemia, were found between both groups, as well as in the gray-scale IVUS parameters. The severity of the MD was greater in the high-LCBI group than in the low-LCBI group (16.6 ± 9.1 vs 4.7 ± 4.8 s, P

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%). All patients were followed up for at least 2 years. Propensity score matching was used to select patient subgroups for comparison: 133 patients with ICD for primary prophylaxis and 133 with ICD for secondary indications. Results There were no significant differences between primary and secondary prophylaxis groups with respect to the incidence of appropriate ICD therapy within 2 years (0.153 vs 0.239; hazard ratio, 1.565 [95% confidence interval (CI), 0.898–2.727]; P = .114). Two-year electrical storm risks were 3.3% and 9.6% with HR = 3.236 (95% CI, 1.058–9.896; P = .039) in patients with primary and secondary prophylaxis, respectively. Conclusion The incidence of ICD therapy received by patients with CAD for primary and secondary prophylaxis was not significantly different based on our propensity score–matched analysis. However, secondary-prophylaxis ICD therapy seems to be associated with a significantly higher risk for electrical storm than primary-prophylaxis ICD therapy., Graphical abstract

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. Fast VTs were divided into three groups depending on heart rate (HR); Group A was 188-209 bpm, and Group-B and Group-C were 210-239 bpm and ≥240 bpm, respectively. Results During a median follow-up of 28 months, 202 fast VT episodes (209 ± 19 bpm) were demonstrated in the 85 patients. ATP terminated 151 of the 202 episodes (74.8%) in total. The success rate of the ATP was not different among the three groups: 73.3% in Group A, 80.6% in Group B, and 66.7% in Group C. ATP success rate of >50% and >70% was 77.6% and 64.7% of the patients, respectively. Left ventricular ejection fraction (LVEF) was significantly higher in the patients with rather than without successful ATP therapy, and receiver operating characteristic (ROC) analysis revealed that LVEF of 23% was the optimal cut-off value. ATP was less effective in patients taking amiodarone, but etiology of the structural heart diseases, indication of the device implantation, and all Electrocardiogram (ECG) parameters were not useful predictors for successful ATP therapy. Conclusions ATP highly terminated fast VT with wide HR ranges in patients with structural heart diseases, and should be considered as the first-line therapy for fast VT except for patients with very low LVEF.

Background The effect of myocardial injury (MI) post-transcatheter aortic valve implantation (TAVI) on clinical outcomes is controversial. This study aimed to evaluate the effect of MI severity on clinical outcome and left ventricle function 30 days post-TAVI and determine MI post-TAVI predictors. Methods Overall, 138 consecutive patients who underwent successful transfemoral TAVI using SAPIEN3 and diagnosed using echocardiography and computed tomography were analyzed. High-sensitivity cardiac troponin T (TnT) was evaluated at baseline, immediately, and at 24, 48, and 72 h post-TAVI. Echocardiography findings and N-terminal pro-B-type natriuretic peptide (Nt-pro BNP) levels were evaluated 30 days post-TAVI. Results Mean age and STS score were 84.4 ± 3.5 years and 6.4 ± 3.2%, respectively. All cases showed severe aortic valve stenosis. Peri-procedural MI was observed in 48 of 100 patients (48.0%). Patients were grouped into MI (n = 48) and non-MI (n = 52), without significant difference in characteristics. Pre-balloon aortic valvuloplasty rate and total pacing time were significantly higher in MI vs non-MI. Total rapid pacing time (TRPT) was an independent predictor for MI (OR 1.06; 95% CI 1.01–1.16; p = 0.04). Echocardiography and Nt-pro BNP changes 30 days post-TAVI were similar between groups. Conclusion Peri-procedural MI, assessed by TnT changes, was observed in 48% of patients. The MI was not associated with overt cardiac dysfunction, and the recovery of left ventricular function and Nt-pro BNP level occurred similarly by 30 day post-TAVI between both groups. In multivariate analysis, TRPT was associated with MI after SAPIEN3 implantation. Trial registration number UMIN000036669.

Ambulatory blood pressure monitoring (ABPM) is used for the evaluation of out-of-office blood pressure (BP), however, knowledge concerning the detailed behavior of nocturnal blood pressure (BP) and pulse rate (PR) is limited.A total of 190 participants (64 ± 15 years, 46.3% males) underwent ABPM for diagnosis of hypertension or evaluation of hypertensive therapy. BP and PR were measured automatically by the oscillometric method. From the hourly average ABPM values, the nocturnal time courses (0 AM to 6 AM) of SBP and PR were determined and compared to each other.In general, SBP fell to the lowest level at around midnight and started to increase progressively towards dawn while PR stayed unchanged until 7 AM. Age and gender affected the time course of SBP, most distinctly in the female patients aged ≥ 60 years. The time course of the increase of SBP was very similar in the patients, with BP dipping and non-dipping. The cardiothoracic ratio (CTR) slightly and renal dysfunction modestly facilitated the increase of nocturnal SBP. The nocturnal increase in SBP was not accompanied by an increase of PR in any group or subgroup. The pathophysiology and clinical significance of the early and exclusive increase in nocturnal BP need to be investigated.Average ABPM values in these hypertensive patients showed that BP starts to increase toward dawn without an increase in PR and that this discrepant behavior between BP and RP was most distinct in females 60 or older. The mechanism and clinical significance of such a discordant variation in BP and PR need to be elucidated.

Fractional flow reserve (FFR) has become the gold standard for diagnosing ischemia in angiographically intermediate epicardial coronary artery stenosis. This study investigated the clinical outcomes and predictors of revascularization deferral based on FFR.In this retrospective cohort study, we assessed 474 lesions (440 patients) where revascularization was deferred based on the FFR value. Minimum lumen diameter and %-diameter stenosis were measured. Calcification was graded as none, mild, moderate, or heavy. The synergy between percutaneous coronary intervention with taxus and cardiac surgery (SYNTAX) score I was also determined. The primary outcome was ischemia-driven target lesion revascularization (TLR) in deferred lesions within 3 years. Patients were also assigned into two groups based on FFR value.The average age of the patients was 69.7±10.4 years. The average FFR value was 0.86±0.05. Stable angina pectoris was noted in 298 (67.7%) cases, and in-stent restenosis (ISR) was present in 28 (5.9%). The average SYNTAX score was 7.2±4.2. The 3-year ischemia-driven TLR was 18 lesions (3.8%). Cox proportional hazard model revealed that the SYNTAX score and ISR were independent predictors for TLR in deferred lesions [hazard ratio (HR) =1.10, 95% confidential interval (CI): 1.01-1.19, P=0.03; HR =6.33; 95% CI: 2.25-17.8, P0.01, respectively]. The deferral group, with a low FFR value, tended to have higher TLR rates than other groups.Lesions with lower FFR values were associated with a higher incidence of ischemia-driven TLR than those with higher FFR values. SYNTAX score and ISR were predictors for ischemia-driven TLR at 3 years in the deferred lesions.

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. The patients were classified into reduced ejection fraction (EF35%; 657 (52%) patients) and preserved or moderately reduced EF (EF ≥ 35%; 599 (48%) patients).ES occurred in 49 (7%) and 36 (6%) patients in the EF35% and EF ≥ 35% groups (log-rank P = 0.297) during the median follow-up of 2.3 years. ICD with resynchronization therapy was associated with a lower incidence of ES in patients with EF35%. Non-ischemic heart disease and diuretics were associated with ES in patients with EF ≥ 35%. During the follow-up, 10/49 (20%) patients with ES and 80/608 patients (13%) without ES died in patients with EF35%, while 7/36 (19%) patients with ES and 38/563 patients (7%) without ES died in those with EF ≥ 35%. We have created 4 Cox multivariate models. All models showed approximately 2-fold higher hazard ratios in patients with EF ≥ 35% compared to EF35%.Our study showed that the determinants of ES differed between EF35% and EF ≥ 35%. The impact of ES for mortality was numerically higher in EF ≥ 35% than in EF35%, although a significant interaction was not detected.

OBJECTIVE To investigate the prevalence and outcomes of asymptomatic coronary artery disease (CAD) in patients with the first episode of ischemic stroke. METHODS Patients admitted to our hospital between November 2001 and January 2009 for the episode of an acute ischemic stroke/transient ischemic attack were included. Stress-Tl-201 scintigraphy was performed and followed by coronary angiography (CAG). The prevalence and risks for asymptomatic CAD, and long-term outcomes were studied. RESULTS Of 1309 patients, only 15 (1.1%) patients presented with a history of CAD. Excluding 406 patients because of severity, systemic infection, early transfer to another hospital, or a refusal to participate, myocardial scintigraphy was performed in 903 patients (mean age, 72 ± 10 years, male 63.9%), and myocardial ischemia was diagnosed in 214 patients (23.7%). Of these patients, 76 patients underwent CAG, and showed significant stenosis (>75%) of a coronary artery in 61 (80.3%) patients. The risk factors for positive scintigraphy findings and CAG were high-grade premature complexes via Holter monitoring (P < .0001), enlarged left ventricle (P = .0051) and wall motion abnormalities (P = .0014) observed on echocardiography, and carotid artery stenosis observed in magnetic resonance angiography imaging (P < .0001). During the follow-up periods of 83 ± 47 months and of 91 ± 47 months, 17.2% of scintigraphy-positive and 2.8% of scintigraphy-negative patients developed episodes of myocardial ischemia, respectively (P < .001). CONCLUSIONS Symptomatic CAD was rare in Japanese patients with the first episode of ischemic stroke, but asymptomatic CAD was identified by stress Tl-201 myocardial scintigraphy in one-quarter of the patients. Positive scintigraphy was associated with asymptomatic CAD and future cardiac events.

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.We analyzed data from the Nippon Storm Study, a prospective observational study of 1570 patients that was conducted at 48 Japanese ICD centers. We allocated 1549 participants to AF and non-AF groups, compared their clinical data at the time of enrollment, and monitored the incidences of mortality, hospitalization, and appropriate and inappropriate ICD/CRT-D therapy during a median 28 months. When the AF (n = 257, 16.6%) and non-AF-(n = 1292, 83.4%) groups were compared, the AF group was older (67.7 vs. 61.4 years; p0.0001), and had lower left ventricular ejection fraction (38.0 ± 17.0% vs. 43.5 ± 18.9%; p0.0001). During follow up, mortality was significantly higher in the AF than the non-AF group (p0.0001). In multivariate analysis, AF was significantly associated with all-cause mortality [p = 0.013; hazard ratio (HR)=1.62]. Inappropriate ICD/CRT-D therapy occurred in 40/257 patients (15.6%) and AF was associated with a higher prevalence of inappropriate ICD/CRT-D therapy (p0.0001; HR=2.25).The presence of AF at ICD/CRT-D implantation carries subsequent independent risks of 1.62-fold for death and 2.25-fold for inappropriate therapy.

Background J waves may develop during coronary angiography (CAG). Patients and results Seven patients (61±6 years, 6 male) had vasospastic angina. ST-segment elevation and ventricular fibrillation were documented in all patients. CAG revealed normal arteries, but slurring or notching (J waves) with an amplitude of 0.20±0.06 mV appeared for the first time (n=6) or in an augmented manner (n=1) with distinct alterations in QRS morphology when contrast medium was injected into the right coronary artery. Conclusion In patients with vasospastic angina, J waves observed during CAG can be a manifestation of a local conduction delay caused by contrast medium-induced myocardial ischemia.

BACKGROUND: This study aimed to evaluate the characteristics and clinical outcomes (major bleeding [MB] and thromboembolic events [TEEs]) of atrial fibrillation (AF) patients with chronic kidney disease (CKD)who receiveddirect oral anticoagulant (DOAC) therapy. METHODS: Data prospectivelycollected from a single-center registry containing 2,272 patients with DOAC prescription for AF (apixaban [n=1,014], edoxaban [n=267], rivaroxaban [n=498], and dabigatran[n=493]) were retrospectively analyzed. Patients were monitored for two years and classified into the CKD (n=1460) andnon-CKD groups(n=812). MB and TEEs were evaluated. RESULTS: The mean age was 72±10 years, with the CHADS2,CHA2DS2-VASc, and HAS-BLED scores being 1.95±1.32, 3.21±1.67, and 1.89±0.96,respectively.Incidence rates of MB and TEEs were 2.3%/year and 2.1%/year, respectively. The CKD groupwasolderand had lower body weight and higher CHADS2,CHA2DS2-VASc, and HAS-BLED scoresthanthe non-CKD group.Kaplan–Meier curve analysis revealed that the incidence of MB and TEEs was higher in the CKD group. Multiple logistic regression analysis in the CKD group revealed thatage andstroke history were independent determinants of TEEs, and low body weighttended to be a determinant of MB.The inappropriate low dose use was higher for apixaban than other DOACs in the CKD group. Consequently, for apixaban, the incidence of stroke was significantly higherin the CKD group than in the non-CKD group. CONCLUSIONS: Patients with CKDwere characterized by factors that predisposed them to MB and TEEs, such as older age and low body weight. In a single-center registry, only treatment with apixaban in the CKD group led to a higher incidence of TEEs.

Background - 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. Methods - We classified a pathogenicity of 141 KCNQ1 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. Results - Among 141 KCNQ1 variants, 61 (43.3%), 55 (39.0%), and 25 (17.7%) variants were classified into pathogenic (P), likely pathogenic (LP), and variant of unknown significance (VUS), respectively. Multivariable analysis showed that proband (HR = 2.53; 95%CI = 1.94-3.32; p KCNQ1 evaluation was useful for risk stratification not only in family members but also in probands. A clinical score (0~4) based on proband, QTc (≥500ms), variant location (MS or C-loop) and P variant by ACMG/AMP guidelines allowed identification of patients more likely to have arrhythmic events. Conclusions - Comprehensive evaluation of clinical findings and pathogenicity of KCNQ1 variants based on the ACMG/AMP-based evaluation may stratify arrhythmic risk of congenital long-QT syndrome type 1.

Ambulatory blood pressure monitoring (ABPM) is used for the evaluation of out-of-office blood pressure (BP), however, knowledge concerning the detailed behavior of nocturnal blood pressure (BP) and pulse rate (PR) is limited.A total of 190 participants (64 ± 15 years, 46.3% males) underwent ABPM for diagnosis of hypertension or evaluation of hypertensive therapy. BP and PR were measured automatically by the oscillometric method. From the hourly average ABPM values, the nocturnal time courses (0 AM to 6 AM) of SBP and PR were determined and compared to each other.In general, SBP fell to the lowest level at around midnight and started to increase progressively towards dawn while PR stayed unchanged until 7 AM. Age and gender affected the time course of SBP, most distinctly in the female patients aged ≥ 60 years. The time course of the increase of SBP was very similar in the patients, with BP dipping and non-dipping. The cardiothoracic ratio (CTR) slightly and renal dysfunction modestly facilitated the increase of nocturnal SBP. The nocturnal increase in SBP was not accompanied by an increase of PR in any group or subgroup. The pathophysiology and clinical significance of the early and exclusive increase in nocturnal BP need to be investigated.Average ABPM values in these hypertensive patients showed that BP starts to increase toward dawn without an increase in PR and that this discrepant behavior between BP and RP was most distinct in females 60 or older. The mechanism and clinical significance of such a discordant variation in BP and PR need to be elucidated.

Introduction Type 2 diabetes (T2DM) is associated with cardiovascular death, including sudden cardiac death due to arrhythmias. Patients with an implantable cardioverter-defibrillator (ICD) are also at high risk of developing a clinically significant ventricular arrhythmia. It has been reported that sodium–glucose cotransporter 2 (SGLT2) inhibitors can reduce cardiovascular deaths; however, the physiological mechanisms of this remain unclear. It is, however, well known that SGLT2 inhibitors increase blood ketone bodies, which have been suggested to have sympatho-suppressive effects. Empagliflozin (EMPA) is an SGLT2 inhibitor. The current clinical trial titled “Placebo-controlled, double-blind study of empagliflozin (EMPA) and implantable cardioverter-defibrillator (EMPA-ICD) in patients with type 2 diabetes (T2DM)” was designed to investigate the antiarrhythmic effects of EMPA. Methods The EMPA-ICD study is a prospective, multicenter, placebo-controlled, double-blind, randomized, investigator-initiated clinical trial currently in progress. A total of 210 patients with T2DM (hemoglobin A1c 6.5–10.0%) will be randomized (1:1) to receive once-daily placebo or EMPA, 10 mg, for 24 weeks. The primary endpoint is the number of clinically significant ventricular arrhythmias for 24 weeks before and 24 weeks after study drug administration, as documented by the ICD. The secondary endpoints of the study are the change from baseline concentrations in blood ketone and catecholamine 24 weeks after drug treatment. Conclusion The EMPA-ICD study is the first clinical trial to assess the effect of an SGLT2 inhibitor on clinically significant ventricular arrhythmias in patients with T2DM and an ICD. Trial registration Unique trial number, jRCTs031180120 (https://jrct.niph.go.jp/latest-detail/jRCTs031180120). Electronic Supplementary Material The online version of this article (10.1007/s13300-020-00924-9) contains supplementary material, which is available to authorized users.