Crystalglobulin-induced nephropathy (CIN) is a rare renal disorder associated with multiple myeloma, characterized by crystal-induced damage in multiple organs, with diagnosis based on the identification of crystals within affected tissues. Conversely, cardiac nonamyloidotic immunoglobulin deposition disease (CIDD) involves the deposition of nonamyloid light chains in the myocardium. In this study, we report a case of CIN combined with CIDD in a 55-year-old woman. Renal biopsy revealed crystalline structures within the interlobular artery, which stained positive for immunoglobulin G1 and lambda chains on fluorescent antibody analysis. Immunoelectron microscopy confirmed the deposition of lambda light chains within the crystalline structures. Fluorescent antibody staining demonstrated lambda-chain deposition in the skin, duodenum, and cardiac tissue. The patient was diagnosed with CIN and chemotherapy was initiated. However, the patient died owing to acute renal failure and rapidly progressive cardiac dysfunction. Autopsy findings revealed no crystalline structures in the myocardium, suggesting an association between CIN and CIDD. Reports on CIN are scarce, and its pathogenesis remains poorly understood. CIDD is also rare, and to date, no previous reports have described concurrent CIN and CIDD with such an abrupt clinical course. Early diagnosis and prompt therapeutic intervention are necessary.

Interleukin-6 (IL-6) plays a role in acute heart failure (AHF) and can predict death and re-hospitalization. However, the relationship between IL-6 levels and mortality, based on heart failure phenotype, remains unclear. This study aimed to clarify the association between IL-6 levels and mortality in patients with AHF with reduced ejection fraction (HFrEF) and with preserved ejection fraction (HFpEF). A retrospective observational study was conducted on 371 AHF cases admitted to our institution. Patients were categorized into two groups based on IL-6 levels using Receiver Operating Characteristic (ROC) curve analysis: those with IL-6 below 29.0 pg/mL (N = 239) and those above 29.0 pg/mL (N = 132). Mortality rates were assessed for both HFrEF and HFpEF patients to determine the differential impact of IL-6. The average age was 71 years, with 60

Patients with coronary artery disease (CAD) typically develop atherosclerosis proximal to the left anterior descending coronary artery (LAD). However, it is unclear how CAD location affects long-term prognosis in the era of drug-eluting stents. This study compared the clinical profiles and long-term outcomes of patients with and without LAD lesions after percutaneous coronary intervention (PCI). Of 2,497 consecutive patients with CAD who underwent PCI, 1,245 who underwent first-time PCI were enrolled. Baseline profiles and long-term prognoses, including major adverse cardiovascular events (MACE), were compared between patients with de novo CAD localized to the LAD (n=986) and non-LAD lesions (n=259). MACE included cardiovascular death, nonfatal myocardial infarction, and target vessel revascularization. Patients in the LAD group were more likely to be older; had an increased prevalence of diabetes, dyslipidemia, and impaired cardiac and renal function; and had significantly more multi-vessel disease, calcified lesions, and long lesions than the non-LAD group. Kaplan-Meier analysis revealed that the MACE rate was significantly higher in the LAD group (16.6

BACKGROUND:Various phenotypes of hypertrophic cardiomyopathy (HCM) may have fatal outcomes. Serum chloride levels are modulated by neurohormonal activation and are closely associated with the prognosis of patients with general heart failure. However, the association between serum chloride levels and the prognosis of patients with HCM remains unclear. Our aim was to elucidate the association between serum chloride levels and the prognosis of HCM. METHODS:We included 507 patients with HCM. Among them, we excluded individuals whose serum chloride levels were not assessed and those undergoing regular hemodialysis during the initial evaluation. As a result, 466 patients were included in the final analysis. The primary study endpoint was HCM-related mortality. RESULTS:Patients were stratified into three groups based on their initial serum chloride levels (tertile 1, serum chloride level ≤ 103 mEq/L; tertile 2, serum chloride level 104-105 mEq/L, and tertile 3, serum chloride level ≥ 106 mEq/L). Over a median follow-up period of 8.5 years, 66 patients reached the primary endpoint. Kaplan-Meier survival analysis indicated a significant among-group difference in the primary endpoint rates (log-rank p < 0.001). Multivariate Cox proportional hazard analysis demonstrated that tertile 1 was independently associated with an increased rate of reaching the primary endpoint, even after adjustment for various covariates (hazard ratio: 2.69, 95% confidence interval: 1.63-4.45). CONCLUSION:Our findings indicate that low serum chloride levels were associated with HCM-related mortality.

BACKGROUND:Timely initiation of temporary mechanical circulatory support (tMCS), with appropriate escalation and de-escalation strategies, is critical in managing cardiogenic shock (CS). However, how tMCS utilization and outcomes differ by CS etiology remains unclear. METHODS AND RESULTS:Using data from the Japan Registry for Percutaneous Ventricular Assist Device (J-PVAD), we evaluated the differences in tMCS use and outcomes among 3,678 Impella-supported patients with acute myocardial infarction-related CS (AMI-CS, n=2,418 (65.7%)), de novo heart failure-related CS (de novo HF-CS, n=758 (20.6%)), and acute-on-chronic HF-related CS (acute-on-chronic HF-CS, n=502 (13.7%)). The median shock-to-support time was significantly shorter in AMI-CS (123 min) than in de novo HF-CS (186 min) and acute-on-chronic HF-CS (205 min; P<0.001 for each). De novo HF-CS patients were more likely to receive multiple tMCS (64.2%) devices compared with AMI-CS (51.4%; P<0.001) and acute-on-chronic HF-CS (55.2%; P=0.001). Compared with de novo HF-CS, the adjusted odds ratio (OR) for in-hospital death was higher in AMI-CS (OR 1.34, 95% confidence interval (CI) 1.08-1.66; P=0.008) and acute-on-chronic HF-CS (OR 1.67, 95% CI 1.25-2.22; P<0.001). CONCLUSIONS:tMCS timing and utilization differed by CS type. De novo HF-CS was associated with the lowest in-hospital mortality rate.

The modified nutrition risk in critically ill (mNUTRIC) score was developed to quantify the risk of adverse events related to malnutrition in the intensive care unit setting. However, its prognostic value has not been examined in patients with acute heart failure (AHF). This study aimed to investigate the relationship between mNUTRIC score and all-cause mortality in AHF patients in the cardiac intensive care unit (CCU). We retrospectively examined 307 patients with AHF who were admitted to our CCU from April 2014 to March 2017. mNUTRIC score was calculated within 24 h of CCU admission. Patients were classified as either high nutritional risk (score ≥ 5) or low nutritional risk (score < 5). The primary endpoint was death from any cause. Median follow-up was 272 days (interquartile range 59–588). Kaplan–Meier survival analysis showed that overall survival was significantly worse in the high nutritional risk group (p < 0.001). In the multivariate analysis adjusted for chronic kidney disease, systolic blood pressure, hypoalbuminemia, anemia, and C-reactive protein concentration, mNUTRIC score ≥ 5 was an independent predictor of higher all-cause mortality (adjusted hazard ratio, 2.23; 95

Liver dysfunction is associated with poorer outcomes in patients with heart failure (HF). The albumin-bilirubin (ALBI) score, which combines serum albumin and total bilirubin levels, has recently emerged as a useful tool to assess liver function and predict prognosis, yet its role in predicting long-term outcomes in patients with acute heart failure (AHF) remains unclear.A total of 492 patients with AHF who were admitted to our hospital were included in the study. The patients were divided into two groups based on their ALBI score at discharge: < -2.25 and ≥ -2.25. The primary endpoint was a composite of all-cause mortality and rehospitalization for HF.The mean age of the patients was 70 years, and 63% were male. During a median follow-up period of 189 days, patients with an ALBI score ≥ -2.25 had a significantly higher risk of the composite endpoint compared to those with an ALBI score < -2.25 (17.0% versus 7.4%, respectively; HR: 1.82, 95% CI: 1.34-2.49; P < 0.001). Multivariable Cox proportional hazards models confirmed that an ALBI score ≥ -2.25 was an independent predictor of poor outcomes, even after adjusting for factors related to HF (HR: 2.00, 95% CI: 1.29-3.13; P = 0.002).The ALBI score at discharge may be useful for risk stratification of all-cause mortality and rehospitalization for HF in patients with AHF.

AbstractBackgroundThe association between corrected QT (QTc) interval and life‐threatening cardiac events in patients with hypertrophic cardiomyopathy (HCM) remains unclear. This study sought to investigate whether the prolonged QTc was associated with HCM‐related death in patients with HCM.MethodsWe included 445 patients with HCM (mean age 51 ± 16 years, 67% men). The QTc interval was measured at the time of the initial evaluation and the patients were classified into those with and without QTc prolongation, which was defined as a QTc interval >450 ms. HCM‐related death was defined as a combined endpoint of sudden death or potentially lethal arrhythmic events, heart failure‐related death, and stroke‐related death.ResultsProlonged QTc interval was found in 120 patients (26.4%) at the time of enrollment. Over a median (IQR) follow‐up period of 8.1 (4.6–11.9) years, a total of 67 patients (15.1%) experienced HCM‐related deaths including 57 (12.8%) with the endpoint of sudden death or potentially lethal arrhythmic events. In a multivariable analysis that included prolonged QTc interval and the risk factors for life‐threatening events, prolonged QTc interval was independently associated with an HCM‐related death (adjusted hazard ratio [HR]: 1.91; 95% confidence interval [CI]: 1.16–3.16; p = .011) and this trend also persisted for the combined endpoint of sudden death or potentially lethal arrhythmic events (adjusted HR: 2.01: 95% CI: 1.17–3.46; p = .012).ConclusionsIn this cohort of patients with HCM, QTc prolongation may be associated with HCM‐related death, including the endpoint of sudden death or potentially lethal arrhythmic events.

BACKGROUND:Data on shock severity and bleeding events in patients with temporary mechanical circulatory support (tMCS) are limited. We investigated the relationship between the Society for Cardiovascular Angiography and Interventions (SCAI) shock stage classification and bleeding events in patients with tMCS. METHODS:We evaluated the data of 285 consecutive patients with tMCS who were admitted to our institution between June 2019 and May 2022. At the time of tMCS initiation, 81 patients (28.4%) were in SCAI stage A, 38 (13.3%) in stage B, 69 (24.2%) in stage C, 33 (11.6%) in stage D, and 64 (22.5%) in stage E. Multivariable logistic regression modeling was used to assess the association between the SCAI shock stage and in-hospital bleeding events. RESULTS:In-hospital bleeding occurred in 100 patients (35.1%). The bleeding event rate increased incrementally across the SCAI shock stages (stage A, 11.1%; stage B, 15.8%; stage C, 37.7%; stage D, 54.6%; stage E, 64.1%). In-hospital bleeding was associated with the SCAI shock stage (p < 0.001). Compared with stage A, the adjusted odds ratios for in-hospital bleeding were 1.48 (95% confidence interval [CI] 0.47-4.66), 6.47 (95% CI 2.61-10.66), 11.59 (95% CI 3.77-35.64), and 7.85 (95% CI 2.51-24.55) for stages B, C, D, and E, respectively. CONCLUSIONS:The SCAI shock stage predicted subsequent bleeding events in patients with tMCS. This simple scheme may be useful for tailored risk-based clinical assessment and management of patients with tMCS.

Stress urinary incontinence (UI) often develops after radical prostatectomy for prostate cancer, and in those patients with moderate-to-severe stress UI an artificial urinary sphincter (AUS) is implanted. Inguinal hernias (IHs) often occur after radical prostatectomy. As the prevalence of AUS implantation increases, it is possible to encounter patients with IHs undergoing AUS implantation (IHA). This study investigated our treatment and discussed an appropriate approach for IHAs. We retrospectively investigated patients who underwent IH repair with AUS implantation at our hospital from January 2018 to March 2023. We classified IHAs into Types A–D based on the positions of the IHs and AUS devices (the positions of the control pump, pressure-regulating balloon, and connecting tube). The hernia and control pump were ipsilateral in Types A and B, whereas the hernia and pressure-regulating balloon were ipsilateral in Types A and C. This study included 12 IHs of 11 patients. The median patient age was 77 years. We conducted open repair in nine patients with all types and laparoscopic repair in two patients with Type B. The median operation times for unilateral and bilateral repairs were 96 and 182 min, respectively. There were no complications with AUS or hernia surgeries. IHA has its own characteristics, and multidisciplinary knowledge thereof will help surgeons safely perform IH surgery.

BACKGROUND:Although diastolic dysfunction is the main pathophysiological feature of hypertrophic cardiomyopathy (HCM), it remains to be clarified whether parameters of diastolic function can reliably determine HCM prognosis. In patients with reduced left ventricular (LV) distensibility, chronic elevation of LV diastolic pressure is seen with a smaller than expected LV size. Accordingly, patients with HCM with severe LV diastolic dysfunction typically demonstrate left atrial (LA) dilation and a disproportionately smaller left ventricle. Therefore, we investigated the relationship between LA/LV diameter ratio, as a potential indicator of disease progression, and outcomes in patients with HCM. METHODS:We included 468 patients in whom LA and LV end-diastolic diameter were successfully evaluated by echocardiography at the initial assessment. We divided the patients into two groups: those with an LA/LV diameter ratio > 1 and those with an LA/LV diameter ratio ≤ 1. We compared the HCM-related death rates between the two groups. RESULTS:Of the 468 patients, 96 patients (20.5 %) with HCM showed an LA/LV diameter ratio > 1. In the univariate analysis, patients with an LA/LV diameter ratio > 1 had a significantly greater likelihood of HCM-related death than patients with an LA/LV diameter ratio ≤ 1 (log-rank p = 0.002). In the multivariate Cox proportional hazards analysis, when including LA/LV diameter ratio > 1 and imbalanced baseline variables, an LA/LV diameter ratio > 1 was an independent determinant of HCM-related death (adjusted hazard ratio: 1.87, 95 % confidence interval: 1.08-3.24; p = 0.024). CONCLUSION:LA/LV diameter ratio can be easily evaluated and may be useful for risk stratification of HCM-related death in patients with HCM.

The HeartMate Risk Score (HMRS), a simple clinical prediction rule based on the patients’ age, albumin, creatinine, and the international normalized ratio of the prothrombin time (PT-INR), is correlated with mortality in the cohort of left ventricular assist device (LVAD) recipients. However, in an aging society, an LAVD is indicated for only a small proportion of patients with acute heart failure (AHF), and whether the HMRS has prognostic implications for unselected patients with AHF is unknown. This study aimed to assess the prognostic value of HMRS categories on admission in patients with AHF. We analyzed 339 hospitalized patients with AHF who had albumin, creatinine, and the PT-INR recorded on admission. The patients were categorized as follows: the High group (HMRS > 2.48, n = 131), Mid group (HMRS of 1.58–2.48, n = 97) group, and Low group (HMRS < 1.58, n = 111). The endpoints of this study were all-cause death and readmission for heart failure (HF). During a median follow-up of 247 days, 24 (18.3

The prognosis of tetralogy of Fallot with pulmonary atresia (TOF/PA) is mainly determined by the development of major aorto-pulmonary collateral arteries (MAPCAs) that provide pulmonary blood perfusion. TOF/PA can be managed conservatively until adulthood in patients with adequate, but not excessive perfusion via MAPCAs. To the best of our knowledge, this is the first report of a patient with unrepaired TOF/PA who eventually developed descending aortic dissection (AD), and survived with medical treatment. A 46-year-old woman was referred to our hospital by her local cardiologist with exertional dyspnea. A three-dimensional (3-D) computed tomography (CT) performed prior to presentation showed a dilated thoracic aorta, three well-developed MAPCAs, and a patent ductus arteriosus (PDA), whereas the 3-D CT performed at presentation revealed a descending AD with the entry site at the proximal part of the thoracic descending aorta, and neither the MAPCAs nor the PDA originated from the area of the AD. The patient was treated medically and was discharged thereafter. In this case, 3D-CT taken 9 months prior to the dissection showed no involvement of MAPCAs in the dissection area and was useful to make a decision of conservative therapy.

Anomalous left coronary artery arising from the noncoronary cusp (LCANCC) is a rare congenital disorder. We herein describe a 17-year-old female patient with sudden cardiac arrest followed by refractory cardiogenic shock. LCANCC-induced acute myocardial infarction with left main coronary artery involvement was subsequently diagnosed, and the patient required a durable left ventricular assist device. .

Renal dysfunction is a known risk of sudden cardiac death in patients with ischemic heart disease. However, the association between renal dysfunction and sudden death in hypertrophic cardiomyopathy (HC) patients remains unknown. This study investigated the significance of an impaired renal function for the sudden death risk in a cohort of patients with HC. We included 450 patients with HC (mean age 52.9 years, 65.1% men). The estimated glomerular filtration rate (eGFR) was evaluated at the time of the initial evaluation. Renal dysfunction was defined as an eGFR <60 ml/min/1.73 m(2). Renal dysfunction was found in 171 patients (38.0%) at the time of enrollment. Over a median (IQR) follow-up period of 8.8 (5.0 to 12.5) years, 56 patients (12.4%) experienced the combined end point of sudden death or potentially lethal arrhythmic events, including 20 with sudden death (4.4%), 11 resuscitated after a cardiac arrest, and 25 with appropriate implantable defibrillator shocks. Patients with renal dysfunction were at a significantly higher risk of sudden death (Log-rank p = 0.034) and the combined end point (Log-rank p<0.001) than patients without renal dysfunction. After adjusting for the highly imbalanced baseline variables, the eGFR remained as an independent correlate of the combined end point (adjusted hazard ratio: 1.24 per 10 ml/min decline in the eGFR; 95% confidence interval 1.04 to 1.47; p = 0.013). In conclusion, an impaired renal function may be associated with an incremental risk of sudden death or potentially lethal arrhythmic events in patients with HC. (C) 2020 Elsevier Inc. All rights reserved.

BACKGROUND:Inflammation and malnutrition are common problems in patients who are hospitalized for acute heart failure (AHF). C-reactive protein (CRP) is an acute-phase reactant and nonspecific marker for evaluating systemic inflammation. There has been growing interest in prealbumin for nutritional assessment. Additionally, prealbumin is a negative acute-phase protein because its synthesis is suppressed in the inflammatory setting in which cytokines stimulate hepatic production of acute-phase proteins (e.g. CRP). Therefore, the CRP to prealbumin ratio (CP ratio) may be a comprehensive marker of inflammation and malnutrition. We evaluated the relationship of the CP ratio with mortality in patients with AHF. METHODS:We analyzed 257 hospitalized patients with AHF who had CRP and prealbumin levels examined on admission. RESULTS:The median CP ratio on admission was 0.57, with an interquartile range of 0.11 to 1.94. In receiver operating characteristic curve analysis, the area under the curve was 0.729 and the optimal cut-off point of the CP ratio for all-cause death was >1.60 (sensitivity: 67.5%; specificity: 77.6%; p = 0.003). Kaplan-Meier survival curves showed that patients with a high CP ratio (>1.60) had a significantly greater risk of all-cause, cardiac, and non-cardiac death (log-rank test, all p<0.001) than patients with a low CP ratio (≤1.60). Multivariable analysis adjusted for imbalanced baseline variables showed that a high CP ratio was independently associated with higher all-cause mortality (adjusted hazard ratio 3.88; 95% confidence interval 1.91-7.86; p<0.001). CONCLUSIONS:The ratio of two hepatic proteins, CRP and prealbumin, may be useful in risk stratification of patients with AHF.