Emery-Dreifuss muscular dystrophy (EDMD) is a group of hereditary muscular dystrophy syndrome caused by deficiency of genes encoding nuclear envelope proteins. Patients having EDMD show the triad of muscle dystrophy, joint contracture, and cardiac disease. In almost all patients, cardiac involvement is prevalent and is the most severe aspect of EDMD. Cardiac disease is predominantly shown by conduction defects, atrial fibrillation/flutter, and atrial standstill. Sudden death and heart failure because of left ventricular dysfunction are important causes of mortality, particularly in those patients that have the LMNA mutation. Medical treatment of EDMD is limited to addressing symptoms and ambulation support; moreover, pacemaker implantation is necessary when there are severe conduction defects and bradycardia occurs. Note that automated defibrillation devices may be considered for those patients who have a high risk of sudden death, rate, or rhythm control. Also, anticoagulation should be initiated in those patients who have atrial fibrillation/flutter. Thus, for optimal management, a multidisciplinary approach is required.

Soluble suppression of tumorigenicity 2 (sST2), a biomarker representing myocardial fibrosis and inflammation, has been applied in risk stratification of patients with myocardial infarction (MI). However, whether primary PCI (PPCI) will eliminate the predictive value of sST2 in STEMI patients has not been well studied. Here, we conducted a prospective clinical trial to evaluate the correlation between sST2 and prognosis in STEMI patients undergoing PPCI. sST2 levels were measured in 295 STEMI patients (60.2 ± 10.8 years) at admission using a high sensitivity assay. Baseline sST2 levels were significantly associated with heart function, biomarkers of inflammation, and myocardial injury. During a 12-month follow-up, 19 patients had major adverse cardiovascular events (MACEs). Greater sST2 was continuously associated with a higher risk of incident MACEs. Such association remained even after adjusting for other risk factors in a multivariate Cox analysis. A baseline sST2 level in the highest quartile (≥ 58.7 ng/mL) was independently associated with mortality (HR: 5.01, 95%CI: 1.02-16.30, P = 0.048). More incident heart failure was seen in the group with greater sST2, however, the association was not significant after adjustment. Therefore, baseline sST2 may be useful to predict MACEs, especially mortality, in STEMI patients receiving PPCI.

High thrombus burden induced slow-flow and no-reflow during primary percutaneous coronary intervention (PCI) and is associated with a poor prognosis. We aimed to investigate whether a combined thrombus burden reduction therapy during primary PCI, could improve microcirculation and enhance cardiac function in the long-term.

Anterior wall STEMI patients with high thrombus burden were randomly assigned to receive a combined thrombus burden reduction therapy or thrombus aspiration alone. The primary end points included the percentage of patients with TMPG (TIMI myocardial perfusion grade) 3, STR (ST-segment resolution) above 70%, the index of microcirculatory resistance (IMR) and left ventricular ejection fraction (LVEF) difference.

Twenty-two patients in the combined interventional group and 24 in the control group completed 1-year follow-up. The percentages of patients with TMPG 3 (68.2% versus 33.3%, P = 0.006) and STR above 70% (63.6% versus 25%, P = 0.016) were significantly higher in the combined group. IMR was significantly lower in the combined interventional group (31.50 ± 13.39 U versus 62.72 ± 22.80 U, P = 0.002). At 3 months and 1 year, the overall LVEF value was better in the combined interventional group (42.1% versus 40.0%, P = 0.049; 41.9% versus 39.8%, P = 0.042), respectively. The IMR value was negatively correlated with the EF value at 3 months (r = −0.145, P = 0.013) and 1 year (r = −0.333, P = 0.031).

A combined thrombus burden reduction therapy during primary PCI can safely reduce thrombus burden, improve myocardial tissue perfusion, and improve cardiac function among STEMI patients with high thrombus burden. IMR might be a good predictor for post-myocardial infarction cardiac function.

Percutaneous coronary interventions to the proximal left anterior descending artery (pLAD)-acute myocardial infarction (AMI) are still challenging, especially in the ostial pLAD. Clinical outcomes of the ostial pLAD-AMI were not well investigated. The aim of the present study was to compare clinical outcomes of the ostial pLAD-AMI with those of the non-ostial pLAD-AMI. The primary endpoint was the major cardiovascular events (MACE), defined as the composite of cardiac death, AMI, stent thrombosis (ST), target lesion revascularization (TLR), and target vessel revascularization (TVR). Between January 2009 and March 2016, a total of 401 pLAD-AMI were included as the study population and were divided into 78 ostial pLAD-AMI (the ostial pLAD group), and 323 non-ostial pLAD-AMI (the non-ostial pLAD group). The median follow-up duration was 414 days. The MACE tended to be higher in the ostial pLAD group (8.0% at 30 days, 19.9% at 400 days) than in the non-ostial pLAD group (4.4% at 30 days, 12.9% at 400 days) without reaching statistical significance (P = 0.087). The prevalence of cardiac death was significantly higher in the ostial pLAD group (6.6% at 30 days, 9.5% at 400 days) as compared with the non-ostial pLAD group (3.1% at 30 days, 4.5% at 400 days) (P = 0.034). There were no significant differences in ST, AMI, TLR, or TVR. We concluded that, as compared with the non-ostial pLAD-AMI, the clinical outcomes of the ostial pLAD-AMI, especially cardiac death, tended to be worse, requiring special attention to the ostial pLAD-AMI.

Neuregulin-4 (Nrg4) is a newly discovered adipokine that is synthesized in many tissues and plays an important role in modulating systemic energy metabolism and in the development of metabolic disorders. However, little is known about the relationship between Nrg4 and coronary artery disease (CAD). In this study, we investigated the association between Nrg4 and the presence and severity of CAD.

We enrolled 73 patients diagnosed by coronary angiography (CAG) as having CAD and 32 controls. The CAD group was divided into two subgroups according to their SYNTAX score. Plasma levels of Nrg4 were measured in all participants and compared among different groups. The relationship between Nrg4 and CAD was analyzed. Receiver operating characteristic (ROC) analysis was conducted to evaluate the usefulness Nrg4 in assessing the presence and severity of CAD.

Nrg4 levels were negatively associated with the SYNTAX score (r = −0.401, P = 0.000). The patients with a higher SYNTAX score had significantly lower Nrg4 levels as compared with the low SYNTAX score subgroup and the controls (P < 0.05). The Nrg4 levels of the low SYNTAX score subgroup were much lower than controls (P < 0.05). Furthermore, an association between Nrg4 and CAD (odds ratio, 0.279; 95% confidence interval, 0.088-0.882) was observed. Nrg4 had 43.8% sensitivity and 96.9% specificity for identifying CAD, and 73.1% sensitivity and 87.3% specificity for identifying patients who had severe coronary artery lesions.

Nrg4 levels were found to be inversely associated with the presence and severity of CAD.

In previous magnetocardiography studies, magnetocardiograms (MCGs) have been obtained using superconducting quantum interference device (SQUID) systems. SQUID is the most sensitive instrument for measuring low-frequency magnetic fields, but it requires liquid helium for cooling, so operating costs are high. In contrast, magnetoresistive (MR) magnetometers function by detecting the change in resistance, caused by an external magnetic field, and have much lower costs. This study was aimed to evaluate feasibility of the MR sensor array for acquiring MCGs.

We used an MR sensor array, which was developed for measuring magnetic fields in the picotesla range, with a reduced noise level (TDK Corporation, Tokyo, Japan). A 30-channel MR sensor array was placed in a magnetically shielded room, and the cardiac magnetic field over the anterior chest walls of five healthy subjects was recorded.

For all five subjects, MCGs were successfully recorded using the MR sensor array. The cardiac magnetic field corresponding to P, QRS, and T waves on an electrocardiogram (ECG) was detectable by signals averaging 272 ± 27.5 beats.

An MR sensor array can be used to measure cardiac magnetic fields. Our results will contribute to the development of low-cost devices for recording MCGs, which will help develop non-invasive diagnostics in cardiovascular medicine.

Sudden unexplained nocturnal death syndrome (SUNDS) is prominent among northeast Thai men. This study tests the hypothesis that Thai men with positive family history of SUNDS display abnormal diurnal, autonomic nervous system responses to stress. Healthy northeast Thai men (20-49 years old) lived in the same rural area were divided into two groups based on their positive (PF) or negative family (NF1) history of SUNDS. A second control included Thai men with an NF history of SUNDS from a non-endemic area (NF2). All data were collected at 4:00-6:00 AM (nighttime) and 4:00-6:00 PM (daytime). All three groups displayed nighttime decreases in mean arterial pressure, heart rate, and blood glucose. Furthermore, all subjects displayed similar glucose tolerance and electrolyte balance. The tachycardic responses to a four-minute step test were similar among groups in the daytime, but the nighttime responses were significantly blunted in the PF group compared to either control group (about 20 bpm less). Tachycardic responses to a cold pressor test tended to decrease more during the nighttime in the PF compared to NF1 and NF2 groups, but the difference was not significant. Arterial pressure responses to the exercise were similar among the three groups during the nighttime, whereas in the NF2, daytime mean arterial pressures increased more than those in the other groups. The present data suggest that Thai men with a PF history of SUNDS display blunted sympathetic nervous system responses to stress during the nighttime, a potential factor that may trigger cardiac arrhythmias and contribute to SUNDS.

Left atrial appendage (LAA) closure (LAAC) has emerged as an alternative therapeutic approach to medical therapy for stroke prevention in patients with nonvalvular atrial fibrillation (NVAF). However, complex LAA anatomy may preclude its use. LAmbre is a new, self-expanding LAA occluder, and is highly adaptable to different LAA morphologies. We explored the feasibility, safety, and efficacy of LAAC using LAmbre device in NVAF patients with or without prior catheter ablation (CA). LAAC using LAmbre device was applied in NVAF patients with (group C) or without (group N) prior CA. Transesophageal echocardiography (TEE) was performed at 3, and 12 months post-LAAC. Among 17 LAAC patients (group C, 6 & group N, 11), 4 cases were implanted with special type devices, 5 were implanted with large devices. Besides one case of cardiac tamponade (N group), there were two minor peri-procedural complications only. Successful sealing of the LAA was documented in all the patients (100%) by TEE both post LAAC and at 3 months. At 3 months, no residual flow was achieved in 11 patients (64.7%); six patients (35.3%) had residual flow < 5 mm. There was no device dislocation or leakage during the mean of 30 months follow up. At 545 days after LAAC, one patient in group C experienced sudden death. Baseline, peri-procedural, and follow-up characteristics were similar between two groups (P > 0.05). LAAC with LAmbre device, subsequent to prior CA for AF, can be performed successfully and safely. The design and distinguishing features of this device could be of help in patients with complex anatomy of LAA.

The incidence of atrial tachycardia (AT) after rheumatic mitral valvular (RMV) surgery has been well described. However, there have been few reports on the characteristics, mechanism, and long-term ablation outcome of ATs after RMV surgery and concomitant Cox-MAZE IV procedure.

The present study reviewed consecutive patients who underwent AT ablation between May 2008 and July 2013. All patients were refractory to antiarrhythmic drugs (AADs) and had a history of RMV surgery and Cox-MAZE IV procedure. A total of 34 patients underwent AT ablation after RMV surgery and concomitant Cox-MAZE IV procedure, and presented 57 mappable and 2 unmappable ATs. The 57 mappable ATs included 14 focal-ATs and 43 reentry-ATs. Ten of the 14 focal-like ATs were located at the pulmonary vein (PV) antrum and border of a box lesion. Of the 43 reentry-ATs, 16 were marco-reentrant around the mitral annulus (MA) and 16 around the tricuspid annulus. There were 41 atypical ATs (non-cavotricuspid isthmus related) including 16 ATs related to the box lesion and 21 ATs related to other Cox-MAZE IV lesions. The AT were successfully terminated in 33 (97.1%) patients. After mean follow-up of 46.9 ± 15.7 months, 25 (73.5%) patients maintained sinus rhythm without AADs after a single procedure and 28 (82.4%) patients after repeated procedures.

The recurrent ATs after RMV surgery and concomitant Cox-MAZE IV were mainly reentry mechanism, and largely related to LA. An incomplete lesion or re-conductive gaps in a prior lesion might be the predominant mechanisms for these ATs. Catheter-based mapping and ablation of these ATs seems to be effective and safe during a long-term follow-up.

A novel, sensor-based, electromagnetic, non-fluoroscopic catheter visualization (NFCV) system shows tracked catheters directly on pre-acquired fluoroscopy or cine loops. We aimed to evaluate the effectiveness of this system in the setting of catheter ablation for idiopathic premature ventricular contractions/ventricular tachycardia (i-PVC/VT).

A total of 30 i-PVC/VT ablation procedures were performed using the NFCV system in conjunction with three-dimensional electroanatomic mapping system (3D-EMS) between January 2013 and April 2017. At the beginning of the procedure, cine loops of right and left anterior oblique views were obtained and replayed for subsequent mapping and ablation. Right ventriculography, aortography, or coronary angiography was performed, depending on the chamber of interest. We reviewed procedural parameters, comparing with the i-PVC/VT ablation procedure using conventional fluoroscopy (CvF) system (pre-, and post-NFCV implementation; 20 and 11 cases, respectively).

I-PVC/VTs were successfully eliminated in 26 patients (87%) in the NFCV group and in 26 (84%) in the CvF group (P = 1.000). The procedure time in the NFCV group was comparable to that in the CvF group (119.8 versus 125.0 minutes, respectively, P = 0.868); the total fluoroscopy time was significantly shorter in the NFCV group (3.3 versus 16.6 minutes, P < 0.001). One patient in the CvF group experienced cardiac tamponade, requiring pericardial drainage. No major complications were encountered in the NFCV group.

NFCV system, in conjunction with 3D-EMS, was safe and feasible for i-PVC/VT mapping and ablation. The system contributed to dramatically reduced fluoroscopy time, compared with CvF.

The hemodynamics and outcomes in patients with a small aortic annulus (SAA) after transcatheter aortic valve replacement (TAVR) with a second-generation transcatheter heart valve remain unclear. We investigated whether TAVR with a Sapien XT (Edwards Lifesciences, Irvine, California) influences postprocedural valve hemodynamics and long-term outcome in high-risk SAA patients compared with surgical aortic valve replacement (SAVR).

We retrospectively identified 94 SAA patients who underwent aortic valve replacement (TAVR = 35 and SAVR = 59). SAA was defined as an aortic annulus diameter ≤ 20 mm, measured by preprocedural transesophageal echocardiography.

The mean age was 80.2 years. The mean Society of Thoracic Surgeons-Predicted Risk of Mortality was 6.8%. The post-procedural peak transvalvular velocity and mean pressure gradient were significantly lower in the TAVR cohort than in the SAVR cohort, whereas the postprocedural aortic valve area was significantly higher in the TAVR cohort. Severe prosthesis-patient mismatch (PPM) occurred less frequently after TAVR than SAVR (TAVR 2.9% versus SAVR 22.0%, P = 0.01). The two-year mortality in SAA patients was similar between the two groups.

TAVR with a Sapien XT in SAA patients improved the valve hemodynamics and reduced the incidence of PPM compared with SAVR. TAVR patients had a similar 2-year mortality despite higher risk baseline characteristics. To avoid PPM and the consequent poor outcomes, TAVR can be considered an alternative option to SAVR in high surgical risk patients with SAA.

The aim of this study was to evaluate the safety and efficacy of a modified loop snare technique for retrieval of difficult inferior vena cava (IVC) filters and migrated coils.

A retrospective review of Günther Tulip filter retrievals between January 2014 and February 2017 was performed. A total of 316 IVC filter retrievals were attempted. In 25 cases, the standard technique had failed and our modified loop snare technique was subsequently attempted in 21 of these patients.

The retrievals were successful in 20 cases (mean dwell time, 42.6 days, range, 14-102 days). The dwell time of the one failure was 46 days. The retrieval rate increased from 92.1% with the standard technique to 98.4% with the combination of the standard and modified loop snare technique. Unsuccessful retrieval was due to migration and endothelialization of the filter. This technique can also be used to remove migrated coils which cannot be captured by standard techniques. There were no complications from the retrievals.

Tilt and endothelialization of filters are the main factors resulting in unsuccessful retrievals with the standard technique. In the present study, we describe an alternative technique for difficult IVC filter retrievals, which can also be used to capture migrated coils and occluders in the aorta and heart.

Percutaneous catheter closure of patent ductus arteriosus (PDA) is difficult when the ductus is large and long or shows calcification. We created a patient-specific 3-dimensional (3D) model for PDA, with which we simulated device deployment, thereby selecting the device/size in a patient-by-patient manner. We assessed whether this 3D model is effective for catheter PDA closure.

The 3D model was created in this institute, requiring 3 days and 90 US dollars. After its introduction, 7 consecutive patients (the study group) with severe PDA underwent closure with the aid of the 3D model. The control group consisted of 4 patients before 3D-model introduction, with all having severe PDA: the requirement of computed tomography was considered a criterion of severe or difficult-procedure-requiring PDA.

In all study group patients, the devices/sizes could be pre-selected based on the simulation, whereas devices were changed during the procedure in 2 of 4 in the control group. In the study group, compared with the control group, the fluoroscopic (median 31 [interquartile range of 16-42] versus 39 [19-71] minutes, respectively) and total procedural times (median 107 [interquartile range 67-114] versus 124 [78-184] minutes, respectively) were shorter. A questionnaire confirmed the doctors' understanding of the procedure.

This 3D model may be effective for percutaneous catheter closure of PDA. This may be especially true in cases of severe or difficult-procedure-requiring PDA.

Pulmonary hypertension (PH) with pulmonary vascular disease (PVD) is a progressive and debilitating disease associated with increased pulmonary vascular resistance (PVR). Biphasic right ventricular outflow tract (RVOT) Doppler flow is frequently seen in severe PH patients with PVD. In association with hemodynamics, the precise analysis of biphasic RVOT Doppler flow (RVDF) has not been fully elucidated. Therefore, the purpose of the present study is to analyze the relation between the hemodynamics and indices of biphasic RVDF in PH patients with PVD.

Seventy PH patients with biphasic RVDF were analyzed. All patients underwent transthoracic echocardiography and right heart catheterization. For the analysis of biphasic RVDF, the early waveform was determined as P₁ while the late waveform was determined as P₂. For each P₁ and P₂, the duration (D, seconds) and peak flow velocity (PFV, in m/second) were measured.

P₁D and P₂PFV were significantly correlated with PVR (P₁D: r = −0.542, P < 0.0001, P₂PFV: r = −0.513, P < 0.0001). Therefore, we propose a novel RVDF formula for estimation of PVR, as follows. PVR = 26 − 77 × P₁D - 14 × P₂PFV. The PVR could be estimated by this proposed formula (r = 0.649, P < 0.0001), which is derived from one Doppler image only unlike previously used PVR prediction formula.

P₁D and P₂PFV were associated with PVR. Moreover, this simple RVDF formula proposed herein can estimate PVR in PH patients with PVD.

This study aimed to examine the relationship between corin expression and circulating brain natriuretic peptide in patients with left ventricular (LV) dysfunction.

Circulating levels of B-type natriuretic peptide (BNP) can be an indicator of LV dysfunction. The 32-amino-acid BNP is cleaved by corin, a cardiac serine protease, from its108-amino-acid pro-brain natriuretic peptide (proBNP) precursor.

This study included 25 patients with idiopathic dilated cardiomyopathy (DCMP) and LV dysfunction and 44 heart transplant recipients with normal LV function who underwent diagnostic left and right heart catheterization. Blood samples were used to determine the ratio of plasma proBNP/BNP levels, and LV endomyocardial biopsies were used to determine the expression of NPPB, which encode BNP and corin, respectively, by quantitative reverse transcription-polymerase chain reaction.

Patients with DCMP revealed worse hemodynamic profiles and higher plasma proBNP and BNP levels than those of the transplant recipients. Myocardial NPPB expression was higher and CORIN expression was lower in the DCMP patients than in the transplant recipients. CORIN expression significantly correlated with NPPB expression (r = −0.585; P < 0.001), ejection fraction (EF; r = 0.694; P < 0.01), LV end-diastolic pressure (r = −0.373; P < 0.05), and indexed end-diastolic LV volume (r = −0.452; P < 0.001). In addition, the plasma proBNP/BNP levels inversely correlated with the CORIN expression (r = −0.362; P < 0.005).

Decreased myocardial CORIN expression and the corresponding higher levels of circulating unprocessed proBNP in DCMP may partly account for the relative BNP resistance observed in patients with LV dysfunction.

Sleep apnea hypopnea syndrome (SAHS) is an independent risk factor for various cardiovascular diseases. Electrocardiogram (ECG) features such as the RR, PR, QT, QTc, Tpe intervals and the Tpe/QT, Tpe/QTc ratios are used to predict and study cardiovascular diseases. It is not clear whether regular patterns of PR and Tpe-related features across sleep stages exist in SAHSs or healthy controls nor whether sleep stages affect the short- and long-range influences of respiratory events on ECG indices. We enrolled 36 healthy controls and 35 patients with SAHS in our study and analyzed the abovementioned ECG features. In the healthy controls, a significant regularity existed in these indices across sleep stages, which were weakened or disturbed in the patient group, especially the Tpe-related features. The differences between the patients and healthy controls were generally consistent across all sleep stages: patients had smaller RR, PR, QT and Tpe/QTc values, but larger QTc, Tpe and Tpe/QT values. After filtering the short-range influence of respiratory events, the differences in most features remained highly significant, except the QT interval. In the patient group, respiratory events decreased RR and PR intervals in most sleep stages and increased the Tpe-related features' values in deep sleep stages. These results may aid in the study of the relationships among SAHS, sleep disorders and cardiovascular diseases.

The progression of renal dysfunction reduces serum albumin and deteriorates the binding capacity of protein-bound uremic toxins. We evaluated the prognostic implications of serum indoxyl sulfate (IS) and albumin levels in patients with cardiovascular disease.

We prospectively enrolled 351 consecutive patients undergoing percutaneous revascularization for coronary artery disease or peripheral artery disease. The primary endpoint was all-cause mortality. Patients were assigned to four groups according to the median levels of serum IS (0.1 mg/dL) and albumin (3.9 g/dL).

During the median follow-up time of 575 days, 16 patients died. The IS level was significantly higher in nonsurvivors (0.33 versus 0.85 mg/dL, P < 0.05). On the Kaplan-Meier curve, the high IS/low albumin group presented the highest mortality rate (log-rank test, P < 0.01). Cox proportional hazard analysis revealed that high IS/low albumin (hazard ratio (HR): 5.33; 95% confidence interval (CI): 1.71-16.5; P < 0.01), diastolic pressure (HR: 0.94; 95% CI: 0.91-0.98; P < 0.01), prior stroke (HR: 4.54; 95% CI: 1.33-15.4; P = 0.01), and left ventricular ejection fraction (LVEF) (HR: 0.92; 95% CI: 0.88-0.96; P < 0.001) were associated with increased mortality. Furthermore, the combination of IS and albumin levels significantly conferred an additive value to LVEF for predicting mortality (C-statistic: 0.69 versus 0.80; P < 0.001; net reclassification improvement: 0.83; P < 0.001; integrated discrimination improvement: 0.02; P = 0.02).

A lower albumin level adds potentiating effects on IS as a prognostic factor for cardiovascular disease.

The aim of this study was to explore the function of miR-138 in the pathogenesis of degenerative calcific aortic valve disease (DCAVD).

Aortic valve calcification tissue and normal tissue from DCAVD patients were collected to detect the expression of miR-138 by qRT-PCR, and immunohistochemical staining was performed to identify the phenotype of valve interstitial cells. QRT-PCR was performed to analyze the expression of miR-138, Runx2, MSX2, and ALP at day 7 after osteogenic differentiation. Alkaline phosphatase activity assay was performed at day 14 after osteogenic differentiation. Alizarin red staining was used to analyze the calcium nodule formation. TargetScan was used to predict potential targets of miR-138. QRT-PCR and Western blotting were performed to analyze the expression of FOXC1 in valve interstitial cells (VICs). The aortic valve calcification was evaluated by quantitative analysis of the velocity in the aortic annulus and transvalvular pressure gradients.

In this study, we demonstrated the role of miR-138 in VIC osteogenesis. QRT-PCR results revealed miR-138 was significantly down-regulated in calcified aortic valves compared with non-calcified valves. MiR-138 overexpression inhibited VIC osteogenic differentiation in vitro, while down-regulation of miR-138 enhanced the process. Target prediction analysis and dual-luciferase reporter assay confirmed FOXC1 was a direct target of miR-138. Further research found FOXC1 overexpression promoted VIC osteogenic differentiation. In addition, animal experiments validated indirectly miR-138 could suppress aortic valve calcification.

Our findings suggest miR-138 could function as a new inhibitor of VIC osteogenic differentiation, which may act by targeting FOXC1.

The mechanism underlying thrombosis in atrial fibrillation (AF) is not yet clearly understood. The apelin/APJ axis parallel and counter-regulate with the angiotensin system. The present study hypothesizes that apelin/APJ axis exert its anti-thrombus effect in normal left atrial tissue and is disrupted by up-regulated renin-angiotensin-aldosterone system (RAAS) signaling during AF. The specimens of left atrial appendages collected from patients with rheumatic mitral stenosis who underwent valve replacement were divided into 3 groups: sinus rhythm, AF⁺/thrombus⁻, and AF⁺/thrombus⁺. The amounts of angiotensin II receptor subtype 1 (AT1), apelin/APJ and its downstream plasminogen activator inhibitor-1 (PAI-1) were detected by western blot. The expression of apelin/APJ was significantly decreased in the AF⁺/thrombus⁺ group compared with the sinus rhythm and AF⁺/thrombus⁻ groups. Meanwhile the expressions of AT1 and PAI-1 were highest in the AF⁺/thrombus⁺ group compared to the other two groups. Taken together, the present study reveals apelin/APJ axis might be correlated with thrombosis in patients with AF mediated by PAI-1.

Inflammation after myocardial infarction (MI) may be a major factor influencing ventricular remodeling, leading to congestive heart failure and arrhythmia. Therefore, inflammation in the heart needs to be monitored. Tenascin-C (TNC) is an extracellular matrix molecule not normally expressed, but it is strongly upregulated when associated with active inflammation. Based on this characteristic, we successfully imaged in vivo inflammatory lesions in rat models using ¹¹¹Indium (¹¹¹In)-labeled anti-TNC antibodies. The aim of the present study was to further assess the applicability of this molecular imaging probe to detect inflammatory activity in primate hearts.

We generated an MI model of cynomolgus monkeys (Macaca fascicularis) by coronary artery ligation and performed dual-isotope single-photon emission computed tomography (SPECT) imaging with an ¹¹¹In-labeled anti-TNC antibody Fab' fragment (¹¹¹In-TNC Fab') and ^99mtechnetium methoxy-isobutyl isonitrile (^99mTc-MIBI). Dual autoradiography was used to compare the uptake of ¹¹¹In-TNC Fab' with histology and immunostaining for TNC. Dual-isotope SPECT showed the regional myocardial uptake of ¹¹¹In-TNC Fab' complementary to a defect in the perfusion image by ^99mTc-MIBI. The high radioactivity of ¹¹¹In-TNC Fab' by autoradiography corresponded to immunostaining for TNC, which was observed in inflammatory lesions at the border zone between the infarcted and non-infarcted areas of the left ventricle and at the epi/pericarditis lesions of the right ventricle. These results demonstrate the potential of ¹¹¹In-TNC-Fab' imaging to monitor myocardial injury and inflammation and suggest the feasibility of the non-invasive detection of cardiac inflammation following acute MI in a preclinical stage before testing in humans.

CD36 is one of the important transporters of long-chain fatty acids (LCFAs) in the myocardium. We previously reported that CD36-deficient patients demonstrate a marked reduction of myocardial uptake of LCFA, while myocardial glucose uptake shows a compensatory increase, and are often accompanied by cardiomyopathy. However, the molecular mechanisms and functional role of CD36 in the myocardium remain unknown.

The current study aimed to explore the pathophysiological role of CD36 in the heart. Methods: Using wild type (WT) and knockout (KO) mice, we generated pressure overload by transverse aortic constriction (TAC) and analyzed cardiac functions by echocardiography. To assess cardiac hypertrophy and fibrosis, histological and molecular analyses and measurement of ATP concentration in mouse hearts were performed.

By applying TAC, the survival rate was significantly lower in KO than that in WT mice. After TAC, KO mice showed significantly higher heart weight-to-tibial length ratio and larger cross-sectional area of cardiomyocytes than those of WT. Although left ventricular (LV) wall thickness in the KO mice was similar to that in the WT mice, the KO mice showed a significant enlargement of LV cavity and reduced LV fractional shortening compared to the WT mice with TAC. A tendency for decreased myocardial ATP concentration was observed in the KO mice compared to the WT mice after TAC operation.

These data suggest that the LCFA transporter CD36 is required for the maintenance of energy provision, systolic function, and myocardial structure.

C-type natriuretic peptide (CNP) hyperpolarizes and relaxes the smooth muscle of blood vessels. We investigated whether G-protein-gated inwardly-rectifying K⁺ channels (GIRK) and large-conductance calcium-activated K⁺ channels (BK_Ca channels) were involved in CNP-evoked vasodilatation in human arteries. Isometric tension in human gastroepiploic arteries was measured using a wire myograph. Ion channel currents were recorded by the whole-cell patch-clamp technique. The concentration-dependent vasodilation induced by CNP was reduced significantly after inhibition of GIRK channels (by tertiapin-Q) or of BK_Ca channel (by paxilline). Immunochemical experiments showed that GIRK3 and GIRK4 subunits were expressed in human arteries. CNP also strongly increased the current density of GIRK and BK_Ca channels in human arterial smooth muscles. This suggested that the GIRK channel was functionally expressed in smooth muscle and vasodilation action was produced by CNP partly by opening the GIRK and BK_Ca channels in the human artery.

The initiation and progression of atherosclerotic cardiovascular disease (ASCVD) has always been associated with a series of risk factors. Evidences of statin therapy from randomized clinical trials are abundant, whereas discussions regarding patients with ASCVD without evidence-based risk factors are rare. Here, we describe a case of a 58-year-old woman who was diagnosed with ASCVD with none of these evidence-based risk factors. After four years of medical interventions, including atorvastatin, the patient recovered completely from severe chest pain with significant regression of atherosclerotic plaques in coronary arteries.

In this paper, we look at the case of a 79 years old male who received a Wiktor stent (WS) implantation for myocardial infarction in proximal left anterior descending artery 18 years ago. Eleven years later, an Everolimus eluting stent (EES; Xience V™) was implanted for the proximal edge restenosis of WS from mid left main trunk to the middle part of WS. Seven years after EES implantation, the angiography and optical coherence tomography revealed in-stent restenosis with severe stent recoil just distal to the overlapping zone of WS. In the present case, stent recoil seems to have occurred due to different radial stiffness and flexibility between the two stents.

We present a rare case of thrombus adhering to the puncture site of the atrial septum during left atrial appendage closure. By discussing the case, we suggest that some preventive measurements be taken during left atrial appendage closure and that conventional transesophageal echocardiography for the atrial septal puncture site be performed after the delivery sheath is removed.

Patients presenting with aborted cardiac arrest who display early repolarization generally are diagnosed with early repolarization syndrome. Therapeutic hypothermia is a standard strategy to improve neurological outcome in comatose patients after cardiac arrest. We present here a patient in whom therapeutic hypothermia exacerbated the J-wave amplitude and morphology, which resulted in episodes of refractory ventricular fibrillation.

His-bundle pacing has recently emerged as a means to maintain a physiological ventricular activation and eliminate the risk of pacing-induced myopathy associated with traditional right ventricular pacing. With His-bundle pacing, the exact stimulated structure and resulting excitation wavefront may be highly dependent on the pacing output, dimensions of the stimulatory electrodes, and orientation of the cathode and anode relative to the approximated conduction tissue and surrounding myocardium, owing to the juxtaposition of tissues with very different conduction properties. We herein present an 89-year-old woman with an infra-Hisian conduction disease in whom lower output pacing resulted in pure His-bundle pacing, and higher output pacing resulted in para-Hisian pacing that recruited diseased portions of the conduction system, narrowing the QRS complex.

Prostate cancer is the most common non-cutaneous malignancy in men and has been steadily rising in an aging society. Medical castration therapy is effective for metastatic prostate cancer, but the proarrhythmic properties have not been reported. We present a 71-year-old Japanese man with metastasis prostate cancer that, during medical castration therapy, had torsades de pointes (TdP) with a QT prolongation and ventricular fibrillation (VF). His QT interval diminished after discontinuing the medical castration, and he developed no further VF recurrences for 15 months. Medical castration is a rare but possible trigger of TdP with QT prolongation and VF.

A 60-year-old female with premature ventricular contractions (PVCs) originating from the bottom of the posteromedial papillary muscle of the left ventricle underwent radiofrequency catheter ablation (RFCA) using an irrigated-tip catheter. During ablation of the PVCs, a loud steam pop was observed. Intracardiac echocardiography (ICE) revealed a growing, hyperechogenic intramyocardial microbubble formation around the catheter tip. The formation disappeared slowly and completely, leaving an endocardial laceration without pericardial effusion. ICE imaging is valuable during a difficult RFCA procedure, because ICE reveals the exact anatomical position of the catheter and thus allows rapid evaluation of the occurrence of steam popping and any possible subsequent complication.

A 65-year-old Japanese man complaining of general malaise and presenting with high fever was admitted to our hospital. He had untreated diabetes and hepatocellular carcinoma with liver cirrhosis. Overall findings of the patient indicated sepsis. Two blood cultures were positive for Streptococcus dysgalactiae, a group C or G Streptococcus. Transthoracic and transesophageal echocardiography revealed vegetations on the aortic and mitral valves. Although antimicrobial therapy with aminobenzyl penicillin was effective for controlling infection, multiple cerebral embolisms occurred in the clinical course of the disease. Primary care doctors should consider invasive Streptococcus dysgalactiae infections when treating elderly patients with underlying diseases, and Streptococcus dysgalactiae should be included in the list of microorganisms considered to cause endocarditis in such patients.

Anderson-Fabry disease is a rare X-linked lysosomal storage disease caused by α-galactosidase A (α-GalA) gene variants and characterized by a large genotypic and phenotypic spectrum. Enzyme replacement therapy (ERT) using recombinant α-GalA has been approved for > 10 years as a specific therapy for the disease. However, the long-term clinical efficacy for cardiac manifestations has been equivocal because it depends on several factors such as genotype, sex, age, and disease severity at the initiation of ERT. We report the differences in the clinical effects of ERT continued for > 10 years in three patients with the same genotype. Left ventricular hypertrophy and myocardial dysfunction progressed in the heterozygote proband even under ERT, although disease progression was prevented in two sons of Case 1.

Takotsubo cardiomyopathy is a common disease, but Takotsubo cardiomyopathy complicated by ventricular septal perforation is very rare. We describe the case of a 92-year-old male who had Takotsubo cardiomyopathy complicated by ventricular septal perforation. We treated the patient medically without surgical or catheter interventions. In three weeks, his abnormal wall motion recovered completely, whereas the ventricular septal perforation remained open. He was ambulatory, subsequently discharged, and had been uneventful for one year. In this manuscript, we discuss the importance of non-invasive management for Takotsubo cardiomyopathy complicated by ventricular septal perforation.

A 70-year-old man with dyspnea was admitted to our department and received standard therapy for recurrent heart failure. He was diagnosed with polycystic kidney disease (PKD) in his thirties and received hemodialysis for 4 years before undergoing renal transplantation at age 45. Although his left ventricular ejection fraction (LVEF) was preserved in his 50s, LVEF decreased progressively from 61% to 24%, while left ventricular diastolic dimension (LVDd) increased from 54 mm to 65 mm between 63 and 69 years of age. Right ventricular endomyocardial biopsy demonstrated myocardial disarray and interstitial fibrosis. Genetic analysis identified a heterozygous frameshift mutation in PKD1, which encodes polycystin-1, a major causative gene of PKD. We detected PKD1 protein expression in myocardial tissue by immunostaining. Recent epidemiological studies and animal models have clarified the pathological correlation between ventricular contractile dysfunction and PKD1 function. Here, we present a case of old-age onset progressive cardiac contractile dysfunction with a PKD1 gene mutation.

A high percentage of patients with critical limb ischemia have concurrent chronic kidney disease (CKD). However, endovascular therapy (EVT) can be problematic in CKD patients. Thus, we developed a method of EVT using digital subtraction angiography (DSA) with diluted contrast medium (low-concentration DSA), wherein DSA parameters were adjusted for diluted contrast angiography (1:10 dilution). Herein, we report the case of an 88-year-old woman with a foot wound and severe CKD. Her estimated glomerular filtration rate was 7.9 mL/minute/1.73 m². Therefore, EVT was performed with low-concentration DSA. Control angiography revealed total occlusion of the anterotibial and posterotibial arteries as well as severe stenosis of the peroneal artery. EVT with ballooning of the below-the-knee (BTK) lesions resulted in sufficient flow to the wound. Angiographic images of sufficient quality and visible wound blush were obtained with 1:10 diluted contrast medium. Because only 20 mL of contrast medium was required, renal function was preserved. EVT using DSA with diluted contrast medium was shown to be an effective BTK intervention in this CKD patient.

In massive pulmonary thromboembolism, requiring cardiopulmonary resuscitation, venous-arterial extracorporeal membrane oxygenation can decompress the overloaded right atrium and ventricle while stabilizing hemodynamic status. However, vascular injuries occur in slightly less than 20% of cases during cannulation. In a 44-year-old woman with suspected pulmonary thromboembolism, a common femoral artery perforation occurred unexpectedly during cannulation for extracorporeal membrane oxygenation. The patient was unstable due to active bleeding. Fortunately, we occluded the bleeding site using a stent. Interventional cardiologists are frequently challenged by vascular injury during cannulation for extracorporeal membrane oxygenation. We recommend imaging modalities to prevent vascular complications. However, if vascular injuries occur, stent placement should be considered for patients with arterial perforation during cannulation for extracorporeal membrane oxygenation.