Impact of Hemoglobin Level, White Blood Cell Count, Renal Dysfunction, and Staphylococcus as the Causative Organism on Prediction of In-Hospital Mortality from Infective Endocarditis.

Infective endocarditis (IE) is a highly fatal disease in cases of delayed diagnosis and treatment, although its incidence is low. However, there have been few single-center studies in which the risk of in-hospital death from IE was stratified according to laboratory findings on admission and the organism responsible for IE. In this study, a total of 162 patients who were admitted to our hospital during the period from 2009 to 2021, who were suspected of having IE according to the modified Duke classification, and for whom IE was confirmed by transesophageal echocardiography were retrospectively analyzed. Patients were observed for a mean-period of 43.7 days with the primary endpoint being in-hospital death. The in-hospital death group had a lower level of hemoglobin (Hb), higher white blood cell (WBC) count, lower level of estimated glomerular filtration rate (eGFR), and higher frequency of Staphylococcus being the causative agent than those in the non-in-hospital death group. In overall multivariate analysis, Hb, WBC count, eGFR, and Staphylococcus as the causative agent were identified to be significant prognostic determinants. IE patients with Hb < 10.6 g/dL, WBC count > 1.4 × 104/µL, eGFR < 28.1 mL/minute/1.7 m2, and Staphylococcus as the causative agent had significantly and synergistically increased in-hospital death rates compared to those in other IE patients. Low level of Hb, high WBC count, low eGFR, and Staphylococcus as the causative agent of IE were independent predictors of in-hospital mortality, suggesting that these 4 parameters may be combined to additively stratify the risk of in-hospital mortality.

Decreased cholinesterase level combined with renal dysfunction and sympathetic denervation associated with increased cardiac mortality in systolic heart failure.

Aims: Cardiac mortality in patients with heart failure (HF) is likely to be aggravated by malnutrition, assessed by serum cholinesterase (ChE) level, as well as by kidney dysfunction or impairment of cardiac sympathetic denervation. Their prognostic interactions, however, have not been determined. Methods: A total of 991 systolic HF patients were enrolled in our HF database following clinical evaluation including evaluation of the nutrition state and assessment of standardized heart-to-mediastinum ratio (sHMR) of iodine-123-labeled meta-iodobenzylguanidine activity. Patients were followed up for an average of 43 months with the primary endpoint of fatal cardiac events (CEs). Results: The CE patient group had a lower level of ChE, lower estimated glomerular filtration rate (eGFR) and lower late sHMR than those in the non-CE patient group. A five-parameter model with the addition of serum ChE selected in the multivariate logistic analysis (model 2) significantly increased the AUC predicting risk of cardiac events compared with a four-parameter model without serum ChE (model 1), and net reclassification analysis also suggested that the model with the addition of serum cholinesterase significantly improved cardiac event prediction. Moreover, in overall multivariate Cox hazard analysis, serum ChE, eGFR and late sHMR were identified to be significant prognostic determinants. HF patients with two or all of the prognostic variables of serum ChE < 230 U/L, eGFR < 48.8 ml/min/1.73 m2 and late sHMR < 1.90 had significantly and incrementally increased CE rates compared to those in HF patients with none or only one of the prognostic variables. Conclusion: Decreases in cholinesterase level and kidney function further increase cardiac mortality risk in HF patients with impairment of cardiac sympathetic innervation.

Mitral valve infective endocarditis in a dialysis patient with a tunneled dialysis catheter and prior MitraClip® implantation: an autopsy case.

BACKGROUND: The number of patients with heart disease who can benefit from treatment is continuing to increase due to the widespread use of cardiac implantable devices. Accordingly, the number of cardiac device-related infective endocarditis (CDRIE) cases has been increasing year by year. We report a very rare experience of performing an autopsy on a patient who died of CDRIE at the site of MitraClip ® implantation, which has recently been developed as a treatment option for severe mitral regurgitation. In addition to hematoxylin-eosin (H-E) staining, Elastica-Masson staining in the present case revealed destruction of all of the atrial, trabecular, fiber and myocardial layers. CASE PRESENTATION: The patient was hemodialyzed with a dialysis catheter. Hemodialysis treatment was difficult due to functional mitral regurgitation caused by cardiac dysfunction, and the MitraClip® procedure was performed. However, he subsequently developed a fever and dialyzation became difficult again, and he was admitted to the cardiology department. Echocardiography revealed a large vegetation at the site of MitraClip® implantation and a diagnosis of CDRIE was made. Guidelines recommend removal of the device and surgical intervention. However, considering the patient's general condition, a decision was made at a heart team conference to give priority to antibiotic therapy. However, the patient did not respond to antibiotic therapy and died of septic shock. CONCLUSION: To our knowledge, this is the first reported case of CDRIE and death after MitraClip® implantation that resulted in an autopsy. Furthermore, not only H-E staining but also Elastica-Masson staining was performed, and it was confirmed that there was significant valve tissue destruction. In the future, the MitraClip® procedure, even though it is minimally invasive, should be carefully considered in immunocompromised patients.

Infective endocarditis of transcatheter atrial septal occluder devices: A case report.

Infective endocarditis (IE) is caused by bacterial vegetation in valves, but it can also occur in implanted mechanical devices. We report a rare case of IE occurring at the site of percutaneous atrial septal closure devices in a patient in her 50s that had been placed for residual defects on a closure patch in her childhood for an atrial septal defect (ASD). She also had a medical history of distal pancreatectomy for insulinoma in her 40s and had insulin-dependent diabetes mellitus, which means she had been immunocompromised.She visited our hospital with complaints of fever and lumbar pain. A computed tomography scan revealed liver abscess. In blood, urine, and drainage specimens submitted for culture testing, extended spectrum beta-lactamase-producing Escherichia coli was cultured in all specimens. Echocardiography showed vegetation at the atrial septal closure devices. In accordance with IE therapy, removal of the atrial septal patch and closure device was performed after antibiotic treatment for 6 weeks.Because the atrial septal patch was calcified and the two devices implanted on the patch were not well covered by neointima, bacteria could easily form vegetation. Percutaneous residual ASD closure on an atrial patch, especially for immunocompromised hosts, should be carefully considered. Learning objective: In general, neointima forms and coats a closure device several years after its insertion. However, as in the present case, the closed atrial septal patch may be severely calcified and the neointima may not be sufficiently formed on the closure device, and infective endocarditis may occur at the site of implantation. In some cases, the indication for closure device implantation after atrial septal patch closure should be carefully considered.

Prognostic value of nutritional parameters in systolic heart failure with renal dysfunction.

Although it is known that assessment and management of the nutritional status of patients are important for treatment of patients with heart failure (HF), there are currently no established indicators. Therefore, we investigated the effects of nutritional parameters as well as conventional parameters on the prognosis of HF patients. A total of 1954 consecutive HF patients with left ventricular ejection fraction (LVEF) less than 50% were enrolled in this study. Transthoracic echocardiography was performed and conventional parameters for HF patients and parameters to assess nutritional status were measured in all patients. Patients were followed up with a primary endpoint of lethal cardiac events (CEs) for 30.2 months. During the follow-up period, cardiac events were documented in 619 HF patients. The CEs group had a lower level of cholinesterase (201.5U/L vs 265.2U/L, P <0.0001), lower estimated GFR (35.2 ml/min/1.73m2 vs 50.3ml/min/1.73m2, P< 0.0001), and lower Geriatric Nutritional Risk Index (GNRI) (91.9 vs 100.0, P< 0.0001) than those in the non-CEs group. Serum cholinesterase, estimated GFR, and GNRI were identified as significant prognostic determinants in multivariate analysis. ROC analyses revealed cut-off values of serum cholinesterase, estimated GFR, and GNRI of 229U/L, 34.2 ml/min/1.73m2, and 95.6, respectively, for identifying high-risk HF patients. HF patients with serum cholinesterase< 229U/L, estimated GFR<34.3 ml/min/1.73m2, and GNRI< 95.6 had a significantly greater rate of CEs than that in the other patients (P<0.0001). Low serum cholinesterase and low GNRI can predict cardiac mortality risk in systolic HF patients with renal dysfunction.

Impact of catheter contact angle on lesion formation and durability of pulmonary vein isolation.

PURPOSE: This study is aimed to evaluate the impact of catheter contact angle on lesion formation and durability of pulmonary vein isolation (PVI). METHODS: Both in vitro experiment and retrospective observational study were conducted. For in vitro experiment, radiofrequency lesions were created on explanted swine hearts in three different catheter contact angles (0°, 45°, and 90°). In the retrospective observational study, we assessed patients who had undergone repeat catheter ablation due to atrial fibrillation recurrence after initial PVI. When pulmonary vein (PV) reconnection was observed, we analyzed the previous ablation points within and without the gap area. The gap areas were where ablation had changed the PV activation sequence or eliminated the PV potential in the repeat session. RESULTS: In the in vitro experiment, lesion width was the smallest (5.3 ± 0.4 mm) in perpendicular contact compared to 0° (vs 5.8 ± 0.5 mm, p = 0.040) and 45° (vs 6.4 ± 0.4 mm, p < 0.001). In the retrospective observational study, we assessed 666 tags of 16 patients with PV reconnections, and 60 tags were in the gap area. Tags in the gap area had longer interlesion distance (odds ratio [OR] 1.49, p < 0.001), greater contact force variability (OR 1.03, p = 0.008), and higher rate of perpendicular contact (OR 3.26, p < 0.001) on multivariate analysis. CONCLUSIONS: Perpendicular contact was associated with a smaller lesion and higher rate of PV reconnection.

Improved risk-stratification in heart failure patients with mid-range to severe abnormalities of QRS duration and systolic function using mechanical dyssynchrony assessed by myocardial perfusion-gated SPECT.

BACKGROUND: The use of left ventricular mechanical dyssynchrony (LVMD), which has been reported to be responsible for unfavorable outcomes, might improve conventional risk-stratification by clinical indices including QRS duration (QRSd) and systolic dysfunction in patients with heart failure (HF). METHODS AND RESULTS: Following measurements of 12-lead QRSd and left ventricular ejection fraction (LVEF), three-dimensional (3-D) LVMD was evaluated as a standard deviation (phase SD) of regional mechanical systolic phase angles by gated myocardial perfusion imaging in 829 HF patients. Patients were followed up for a mean period of 37 months with a primary endpoint of lethal cardiac events (CEs). In an overall multivariate Cox proportional hazards model, phase SDs were identified as significant prognostic determinants independently. The patients were divided into 4 groups by combining with the cut-off values of LVEF (35% and 50%) and QRSd (130 ms and 150 ms). The groups with lower LVEF and prolonged QRSd more frequently had CEs than did the other groups. Patient groups with LVEF < 35% and with 35% ≦ LVEF < 50% were differentiated into low-risk and high-risk categories by using an optimal phase SD cut-off value of both QRSd thresholds. CONCLUSIONS: 3-D LVMD can risk-stratify HF patients with mid-range as well as severe abnormalities of QRSd and systolic dysfunction.

Impaired Cardiac Sympathetic Innervation Increases the Risk of Cardiac Events in Heart Failure Patients with Left Ventricular Hypertrophy and Mechanical Dyssynchrony.

BACKGROUND: Left ventricular mechanical dyssynchrony (LVMD), left ventricular hypertrophy, and impaired cardiac sympathetic innervation are closely related to the development of heart failure (HF) and unfavorable outcomes. METHODS AND RESULTS: A total of 705 consecutive HF patients with reduced left ventricular ejection fraction (EF) < 50% were registered in our hospital HF database. LVMD and left ventricular mass index (LVMI) were evaluated three-dimensionally by gated myocardial perfusion SPECT. LVMD was measured as a heterogeneity index (phase SD) of the regional contraction phase angles calculated by Fourier analysis. Cardiac sympathetic innervation was quantified as a normalized heart-to-mediastinum ratio (HMR) of the 123I-metaiodobenzylguanidine (MIBG) activity. The patients were followed up with a primary end point of lethal cardiac events (CEs) for 42 months. CEs were documented in 246 of the HF patients who had a greater phase SD, greater LVMI, and lower MIBG HMR than those in HF patients without CEs. In the overall multivariate analysis, phase SD, LVMI, and MIBG HMR were identified as significant CE determinants. The three biomarkers were incrementally related to increases in CE risks. CONCLUSIONS: Assessment of cardiac sympathetic innervation can further stratify patients with systolic heart failure at increased cardiac risk identified by left ventricular hypertrophy and mechanical dyssynchrony.

Edwardsiella tarda Native Valve Infective Endocarditis in a Young and Non-Immunocompromised Host: A Case Report.

BACKGROUND Infective endocarditis (IE) is an infectious disease that occurs in valves, centered on the endocardium and ventricular septal defects. It is a serious disease that is easily misdiagnosed and has a high mortality rate if left untreated. Edwardsiella tarda is an extremely rare cause of IE, especially in young and non-immunocompromised hosts. CASE REPORT A woman in her 20s presented to our hospital with fever of unknown cause and liver dysfunction. She was admitted to the Department of Gastroenterological Medicine owing to suspicion of gastrointestinal infection. Gastrointestinal examination, including contrast-enhanced computer tomography and endoscopic ultrasonography, was performed; however, there were no significant findings. Liver dysfunction improved spontaneously, but her fever did not improve with antibiotic treatment. Transthoracic echocardiography was performed on day 9 of hospitalization because E. tarda was detected in a blood culture test, revealing vegetation at the mitral valve. Asymptomatic cerebral infarction was shown by brain magnetic resonance imaging, and mitral valvuloplasty was performed on day 14. After surgery, transthoracic echocardiography was performed on day 22, showing no vegetation or mitral regurgitation. However, postoperative transesophageal ultrasonography performed on day 29 revealed severe mitral regurgitation. Redo mitral valvuloplasty was performed on day 38. She clinically improved and was discharged on day 67. CONCLUSIONS This is the first case in which E. tarda was diagnosed as the causative agent of IE on a native valve in a young and non-immunocompromised host. Aggressive source control resulted in a good clinical outcome.

Independent Association of Fatty Liver Index With Left Ventricular Diastolic Dysfunction in Subjects Without Medication.

Nonalcoholic fatty liver disease has been reported to be potentially linked to cardiovascular disease. Fatty liver index (FLI) is a noninvasive and simple predictor of nonalcoholic fatty liver disease. However, little is known about the relationship between FLI and cardiac function, especially in a general population. We investigated the relationships of FLI with echocardiographic parameters in 185 subjects (men/women: 79/106) of the Tanno-Sobetsu Study, a population-based cohort, who were not being treated with any medication and who underwent echocardiography. FLI was negatively correlated with high-density lipoprotein cholesterol and peak myocardial velocity during early diastole (e'; r = -0.342, p <0.001), an index of left ventricular (LV) diastolic function, and ratio of peak mitral velocities during early and late diastole (E/A) and was positively correlated with age, systolic and diastolic blood pressures, creatinine, uric acid, homeostasis model assessment of insulin resistance, high-sensitivity C-reactive protein, ratio of mitral to myocardial early diastolic peak velocity (E/e'), left atrial volume index and LV mass index. No significant correlation was found between FLI and LV ejection fraction. Stepwise multivariable regression analysis showed that FLI was independently and negatively associated with e' after adjustment of age, gender, high-density lipoprotein cholesterol, homeostasis model assessment of insulin resistance, and high-sensitivity C-reactive protein. Conversely, e' was independently and negatively associated with FLI after adjustment of age, gender, systolic blood pressure, and LV ejection fraction. In conclusion, elevated FLI is independently associated with LV diastolic dysfunction in a general population without medication. FLI would be a novel marker of LV diastolic dysfunction as an early sign of myocardial injury.

Prognostic value of 6-min walk stress echocardiography in patients with interstitial lung disease.

BACKGROUND: The 6-min walk test (6MWT) provides prognostic information for patients with interstitial lung disease (ILD). Parameter determined by Doppler echocardiography after the 6MWT (6 MW stress echocardiography) is shown to be a predictor of future development of pulmonary hypertension in patients with connective tissue disease. However, the clinical utility of 6 MW stress echocardiography in predicting cardiopulmonary events in patients with ILD remains unknown. We examined whether parameters determined by 6 MW stress echocardiography independent predictors of adverse events in patients with ILD. METHODS: Echocardiographic examinations were performed in 68 consecutively enrolled patients with ILD (age, 65 ± 10 years, 65% men). A pressure gradient of tricuspid regurgitation (TRPG) and pulmonary vascular resistance (PVRecho) calculated using the following formula [PVRecho = (peak velocity of TR × 10/time-velocity integral of right ventricular outflow (RVOT-VTI)) + 0.16] were measured at baseline and at post 6MWT. Data for parameters of pulmonary functional tests and for 6MWT were collected. RESULTS: During a mean follow-up period of 22 ± 12 months, 22 patients experienced cardiopulmonary events. In univariate analysis, %VC, TRPG, PVRecho, TRPG post 6MWT, and PVRecho post 6MWT were significantly associated with cardiopulmonary events. Multivariate analysis using the Cox proportional hazards model indicated that %VC [hazard ratio (HR): 0.97, p = 0.009] and PVRecho post 6MWT (HR: 1.77, p = 0.004) were independent predictors of cardiopulmonary events in patients with ILD. CONCLUSIONS: In addition to parameters of pulmonary function tests, increased PVRecho post 6MWT is a significant predictor of cardiopulmonary events in patients with ILD. A 6 MW stress echocardiography is useful in assessing the risk of adverse events in patients with ILD.

Current clinical applications of speckle tracking echocardiography for assessment of left atrial function.

Two-dimensional speckle tracking echocardiography (2D-STE) enables assessment of left atrial (LA) function with better reproducibility and less load dependence than those with conventional methods. Data regarding LA functions determined by 2D-STE in patients with various cardiovascular diseases have recently been accumulating. LA strain has emerged as a novel parameter with the potential of improving the diagnostic accuracy of left ventricular diastolic dysfunction and the prognostic value in patients with heart failure and stroke. Furthermore, LA dysfunction determined by 2D STE can provide incremental prognostic information to conventional echocardiographic parameters in patients with cardiovascular diseases. Hence, 2D-STE appears to be a promising technique for diagnosis and therapeutic decision-making. Data regarding LA functions determined by three-dimensional speckle tracking echocardiography (3D-STE) have also been accumulating. Here, I review recent studies, especially those published after 2016, on clinical applications of LA function assessed by 2D-STE and 3D-STE and summarize the remaining problems to be solved for improving the clinical utility of these techniques.

Impact of Frailty on Bleeding Events Related to Anticoagulation Therapy in Patients With Atrial Fibrillation.

BACKGROUND: Although anticoagulation is the key treatment to prevent stroke in patients with atrial fibrillation (AF), including elderly patients, anticoagulation is sometimes withheld for elderly people because of concerns about frailty. However, it remains unknown whether frailty increases bleeding events.Methods and Results:A total of 120 consecutive non-valvular AF patients admitted with symptoms of AF or congestive heart failure were included in this study. Frailty was assessed using the Cardiovascular Health Study (CHS) frailty index. We performed a retrospective analysis of the risk factors associated with major bleeding events. After a median follow-up of 518 days, major bleeding events occurred in 17 (14.2%) patients. Patients with major bleeding events had a higher CHS frailty index (P=0.015). The cutoff value for high-risk CHS frailty index was 2 (area under the ROC curve: 0.68 [95% confidence interval (CI): 0.57-0.78]). The event-free rates at 2 years were 97.6% (95% CI: 83.9-99.7) in patients with a CHS frailty index <2 and 59.6% (95% CI: 27.9-81.0) for those with a CHS frailty index ≥2 (P<0.001). CONCLUSIONS: Frailty is associated with increased bleeding events related to anticoagulant therapy in patients previously hospitalized with AF. Greater care should be taken with patients with a CHS frailty index ≥2.

Synergistic prognostication of left ventricular hypertrophy and three-dimensional mechanical dyssynchrony in heart failure.

AIMS: In this study, we investigated the prognostic interplay of left ventricular hypertrophy and mechanical dyssynchrony (LVMD), both of which can be measured three-dimensionally by gated myocardial perfusion imaging (MPI), in patients with chronic systolic heart failure (HF). METHODS AND RESULTS: In 829 consecutive HF patients with reduced left ventricular ejection fraction less than 50%, LVMD was evaluated as a standard deviation (phase SD) of regional onset of mechanical contraction phase angles. A phase histogram was created by Fourier phase analysis applied to regional time-activity curves obtained by gated MPI. Left ventricular mass index (LVMI) was measured by Corridor 4DM version 6.0. Patients were followed up with a primary endpoint of lethal cardiac events (CE) for a mean interval of 34 months. CE were documented in 223 (27%) of the HF patients. The CE group had a greater phase SD and a greater LVMI than those in the non-CE group. Patients in the CE group had a more advanced age, greater New York Heart Association (NYHA) functional class, left ventricular cavity size, and left atrial diameter or septal E/e' and lower kidney or cardiac function than did patients in the non-CE group. Phase SD > 37 and LVMI > 122.7 g/m2 were identified as optimal cut-off values by receiver operating characteristic analyses for discrimination of the most increased risk HF subgroup from others (P < 0.0001). When classified into four patient subgroups using both cut-off values, HF patients with phase SD > 37 (LVMD) and LVMI > 122.7g/m2 had the highest CE rate among the subgroups (P < 0.0001). Univariate analysis and subsequent multivariate analysis with a Cox proportional hazards model showed that phase SD and LVMI were significant independent predictors of CE with hazard ratios of 1.038 (confidence interval [CI], 1.024-1.051, P < 0.0001) and 1.005 (CI, 1.001-1.008, P = 0.0073), respectively, as well as conventional clinical parameters such as age, NYHA class, estimated glomerular filtration rate (eGFR), and BNP concentration. Patients with increased phase SD and LVMI had incrementally improved prognostic values of clinical parameters including age, NYHA functional class, eGFR, and BNP with increases in the global χ2 value: 5.9 for age; 139.5 for age and NYHA; 157.9 for age, NYHA, and eGFR; 163.9 for age, NYHA, eGFR, and BNP; 183.4 for age, NYHA, eGFR, BNP, and phase SD; and 192.5 for age, NYHA, eGFR, BNP, phase SD, and LVMI. CONCLUSIONS: Three-dimensionally assessed LVMD has independent prognostic values and can improve the risk stratification of chronic HF patients synergistically in combination with conventional clinical parameters.

Paradoxical Prognostic Implications of Visceral Adiposity for Lethal Cardiac Events in Association with Impaired Cardiac Sympathetic Innervation in Chronic Heart Failure.

Background: Obesity increases the risk for development of heart failure (HF) but, when present is likely to be related to better outcomes in patients with HF. This study aimed to clarify the paradoxical prognostic values of visceral obesity in association with cardiac sympathetic function in HF patients. Methods and Results: A total of 653 consecutive patients with systolic HF who underwent visceral adiposity area (VAA) measurements using a computed tomographic technique were divided into 3 groups: VAA 1, area <80 cm2; VAA 2, area 80-140 cm2; VAA 3, area >140 cm2. Sympathetic innervation was quantified by 123I-MIBG cardiac activity. Patients were followed up for an average of 22 months with a primary endpoint of lethal cardiac events (CE). The CE group (n=200) had a lower late heart-to mediastinum ratio (HMR) and a smaller VAA than those in the non-CE group. Rates of overall CE/HF death were inversely correlated with VAA: 39.2% ± 6.2% for VAA 1, 27.4% ± 19.9% for VAA 2 and 24.1% ± 15.3% for VAA 3. In addition to sudden cardiac death rate, lethal arrhythmic event rate increased in association with visceral fat obesity: 3.0% for VAA 1, 7.5% for VAA 2 and 8.8% for VAA 3. Late HMR identified high-risk sub-populations in each group. Conclusion: Visceral obesity has paradoxical prognostic implications in terms of HF mortality and lethal arrhythmic/sudden cardiac death events. Cardiac sympathetic denervation and quantitative visceral adiposity are synergistically associated with overall cardiac mortality, contributing to better risk stratification of HF patients.

Assessment of prognosis in immunoglobulin light chain amyloidosis patients with severe heart failure: a predictive value of right ventricular function.

Although the benefit of updated therapeutic regimens, including bortezomib, on the survival of immunoglobulin light chain (AL) amyloidosis patients with heart failure (HF) has been reported, predictors of mortality in the patients treated with the updated therapy remain unclear. We retrospectively enrolled AL amyloidosis patients who had severe HF at the time of diagnosis and received the updated therapy, including bortezomib (n = 19, 61 ± 6 years old, 68% male). Severe HF was defined as the presence of both NYHA functional class III or IV and BNP > 200 pg/ml or NT-pro-BNP > 900 pg/ml. One-year mortality rate during follow-up after commencement of the treatment was 37%. Left ventricular morphological parameters and indexes of left ventricular diastolic function on admission were similar in the non-survivors and survivors. However, non-survivors had higher incidences of atrial fibrillation and ventricular tachycardia, higher serum total bilirubin levels (1.34 ± 0.55 vs. 0.61 ± 0.29 mg/dl), higher right atrial volume index (RAVI 49.7 ± 29.9 vs. 27.3 ± 6.8 ml/m2), lower tricuspid annular peak velocities during systole (RVs' 8.0 ± 1.8 vs. 11.6 ± 3.7 cm/sec) and late diastole (RVa' 3.4 ± 0.9 vs. 11.4 ± 5.3 cm/sec), and larger inferior vena cava dimension (22.7 ± 6.4 vs. 16.3 ± 4.9 mm) than those in survivors. Kaplan-Meier curve analyses showed that larger RAVI and lower RVs' and RVa', but not left ventricular systolic/diastolic dysfunction, predicted higher mortality during 1-year follow-up. The present results suggest that the presence of right-sided heart abnormality on admission is associated with high 1-year mortality in AL amyloidosis patients with severe HF under the updated therapeutic regimens.

Remaining challenges in the diagnosis of early stage cardiac sarcoidosis.

Despite the requirement for histopathological evidence to make a definite diagnosis of cardiac sarcoidosis, the sensitivity of endomyocardial biopsy is still low. Recently, Japanese Circulation Society suggests a new strategy that patients diagnosed clinically do not require the endomyocardial biopsy evidence. Physicians should familiarize themselves with such paradigm shifts.

Risk classification of pulmonary arterial hypertension by echocardiographic combined assessment of pulmonary vascular resistance and right ventricular function.

Which combination of clinical parameters improves the prediction of prognosis in patients with pulmonary arterial hypertension (PAH) remains unclear. We examined whether combined assessment of pulmonary vascular resistance and right ventricular function by echocardiography is useful for classifying risks in PAH. In 41 consecutive patients with PAH (mean age of 48.9 ± 17.3 years, 31 females), a 6-min walk test, pulmonary function test, and echocardiography were performed at baseline and during PAH-specific therapies. The study endpoint was defined as a composite of cardiovascular death and hospitalization for PAH and/or right ventricular failure. During a follow-up period of 9.2 ± 8.7 months, 18 patients reached the endpoint. Multivariate regression analysis showed that the ratio of tricuspid regurgitation pressure gradient to the time-velocity integral of the right ventricular outflow tract (TRPG/TVI) and tricuspid annular plane systolic excursion (TAPSE) during PAH-specific treatment were independent prognostic predictors of the endpoint. Using cutoff values indicated by receiver operating characteristic analysis, the patients were divided into four subsets. Multivariate analyses by Cox's proportional hazards model adjusted for age, sex and body mass index indicated that subset 4 (TRPG/TVI ≥ 3.89 and TAPSE ≤ 18.9 mm) had a significantly higher event risk than did subset 1 (TRPG/TVI < 3.89 and TAPSE > 18.9 mm): HR = 25.49, 95% CI 4.70-476.97, p < 0.0001. Combined assessment of TRPG/TVI and TAPSE during adequate PAH-specific therapies enables classification of risks for death and/or progressive right heart failure in PAH.