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BackgroundProtein induced by vitamin K absence-II (PIVKA-II) is produced by the liver during hepatoma and upon warfarin administration. Those patients have disturbed protein synthesis and glycosylation in the liver. This decreases the number of γ-carboxyglutamyl (Gla) residues on prothrombin, converting prothrombin into PIVKA-II. The mechanism of this conversion, however, is not clearly understood.MethodsProthrombin was isolated from healthy and warfarin-treated individuals whose liver function of protein production was quantitatively normal. Glycan structures in the purified prothrombin containing PIVKA-II were qualitatively analyzed by high performance liquid chromatography after labeling the glycan with fluorophore 2-aminobenzamide.ResultsThe concentration of PIVKA-II was significantly higher in the warfarin-treated individuals than in the healthy individuals (PConclusionsWarfarin therapy leads to lower amounts of prothrombin and Gla residues within prothrombin without exerting qualitative and quantitative change in glycan profile and protein synthetic function in the liver.

Background It remains unclear whether beta‐blocker use at hospital admission is associated with better in‐hospital outcomes in patients with acute decompensated heart failure. Methods and Results We evaluated the factors independently associated with beta‐blocker use at admission, and the effect of beta‐blocker use at admission on in‐hospital mortality in 3817 patients with acute decompensated heart failure enrolled in the Kyoto Congestive Heart Failure registry. There were 1512 patients (39.7%) receiving, and 2305 patients (60.3%) not receiving beta‐blockers at admission for the index acute decompensated heart failure hospitalization. Factors independently associated with beta‐blocker use at admission were previous heart failure hospitalization, history of myocardial infarction, atrial fibrillation, cardiomyopathy, and estimated glomerular filtration rate 2 . Factors independently associated with no beta‐blocker use were asthma, chronic obstructive pulmonary disease, lower body mass index, dementia, older age, and left ventricular ejection fraction P P P for interaction 0.04). Conclusions Beta‐blocker use at admission was associated with lower in‐hospital mortality in patients with acute decompensated heart failure. Registration URL: https://www.upload.umin.ac.jp/ ; Unique identifier: UMIN000015238.

Veno-arterial extracorporeal membrane oxygenation (VA ECMO) is a powerful device for treatment of patients with life-threatening heart failure. Although bleeding is often associated with VA ECMO and sometimes results in a fatal outcome, its precise causes remain unknown. On the other hand, excessive high shear stress in the cardiovascular system causes acquired von Willebrand syndrome (aVWS), characterized by loss of von Willebrand factor (vWF) large multimers. vWF large multimers of five consecutive patients treated with VA ECMO were quantitatively evaluated using the vWF large multimer indices, defined as the ratio of the large multimer ratio of a patient to that of a healthy subject analyzed simultaneously. All 5 patients exhibited oozing type of bleeding at the skin insertion sites under treatment with PCPS at flow rates of 2.5–3.0 l/min/m2, including two severe cases of bleeding; one patient had massive gastrointestinal bleeding and another had hemothorax. Their vWF large multimer indices were 20.8, 28.8, 27.6, 51.0, and 31.0% (means 31.8 ± 11.4%). Surprisingly, these values are much lower than those observed in severe aortic stenosis reported previously by us (Tamura et al. in J Atheroscler Thromb 22:1115–1123, 2015), where vWF multimer indices in 31 severe aortic stenosis patients with peak pressure gradient through the aortic valves of 85.1 ± 29.4 mmHg were 75.0 ± 21.7% (p

Aortic stenosis (AS), a late complication of thoracic radiation therapy for chest lesions, is often coincident with porcelain aorta or hostile thorax. We herein report a 59-year-old man with a history of mediastinal Hodgkin lymphoma treated with radiation therapy but later presenting with heart failure caused by severe AS. Severe calcification in the mediastinum and around the ascending aorta made it difficult to perform surgical aortic valve replacement. The patient therefore underwent transcatheter aortic valve implantation (TAVI). It is important to recognize radiation-induced AS early, now that TAVI is a well-established treatment required by increasing numbers of successfully treated cancer patients.

ObjectiveThis observational study aimed to examine the prognostic association of angiotensin-converting enzyme inhibitors (ACE-I)/angiotensin receptor blockers (ARB) in different left ventricular ejection fraction (LVEF) categories.MethodsIn 3717 patients enrolled in the KCHF Registry, a multicentre registry including consecutive patients hospitalized for acute heart failure (HF), we assessed patient characteristics and association between ACE-I/ARB and clinical outcomes according to LVEF. In the three LVEF categories (reduced LVEF [HFrEF], mid-range LVEF [HFmrEF] and preserved LVEF [HFpEF]), we compared the patients with ACE-I/ARB as discharge medication and those without, and assessed their 1-year clinical outcomes. We defined the primary outcome measure as a composite of all-cause death and HF hospitalization.ResultsThe 1-year cumulative incidences of the primary outcome measure were 36.3% in HFrEF, 30.1% in HFmrEF and 33.8% in HFpEF (log-rank P = 0.07). The adjusted risks of the ACE-I/ARB group relative to the no ACE-I/ARB group for the primary outcome measure were significantly lower in HFrEF and HFmrEF (HR 0.66 [95%CI 0.54-0.79], PConclusionsACE-I/ARB for patients who were hospitalized for acute HF was associated with significantly lower risk for a composite of all-cause death and HF hospitalization in HFrEF and HFmrEF, but not in HFpEF. ACE-I/ARB might be a potential treatment option in HFmrEF as in HFrEF.

Several studies have reported a correlation between right ventricular (RV) and left ventricular (LV) systolic dysfunction in adults with repaired tetralogy of Fallot (TOF). However, data are lacking regarding the relationship between RV and LV diastolic dysfunction assessed by 2-dimensional speckle-tracking echocardiography. We studied 69 adults with repaired TOF (mean age 34 years, 61% male) who had been regularly followed up and had routinely undergone echocardiography. In addition to conventional echocardiography, global longitudinal strain (GLS) and early diastolic strain rate (SRe) of both ventricles were assessed using 2-dimensional speckle-tracking echocardiography. Results were compared with 30 age- and sex-matched controls. RV and LV GLS were decreased in TOF patients compared with controls (− 18.4 ± 3.3% vs. −23.5 ± 4.2%, p − 1 vs. 1.47 ± 0.41 sec− 1, p = 0.003 and 1.29 ± 0.42 sec− 1 vs. 1.63 ± 0.42 sec− 1, p

The management of idiopathic dilated cardiomyopathy (DCM) is well established. However, a subset of patients do not have recovery from or have recurrences of left ventricular (LV) dysfunction despite receiving optimal medical therapy. There are limited long-term follow-up data about LV function and the predictive value of iodine-123-metaiodobenzylguanidine (123I-MIBG) scintigraphy, especially among the Japanese population. We retrospectively investigated 81 consecutive patients with DCM (mean LV ejection fraction (EF) 28 ± 7.5%) who had undergone 123I-MIBG scintigraphy before starting β-blockers. According to chronological changes in LVEF, study patients were classified into three subgroups: sustained recovery group, recurrence group, and non-recovery group. The outcome measure was cardiac death. Mean age was 59 ± 11 years and median follow-up was 11.5 (5.8–15.0) years. Thirty-six patients had recovery, 11 had recurrences, and 34 did not have recovery. The sustained recovery group had the best cardiac death-free survival, followed by the recurrence and non-recovery groups. Prolonged time to initial recovery was associated with recurrence of LV dysfunction. Large LV end-diastolic diameter and reduced heart to mediastinum ratio were associated with poor prognosis. In conclusion, with β-blocker therapy, 14% of patients showed recurrences of LV dysfunction. Thus, careful follow-up is needed, keeping in mind the possibility of recurrence, even if LVEF once improved, especially in patients whose time to initial recovery was long. 123I-MIBG scintigraphy provides clinicians with additional prognostic information.

Background: The dual antiplatelet therapy (DAPT) score was developed to estimate ischemic and bleeding risks from the DAPT study. However, few studies validated its utility externally. We sought to validate the utility of the DAPT score in the Japanese population. Methods: In a pooled cohort of 3 studies conducted in Japan (the CREDO-Kyoto [Coronary Revascularization Demonstrating Outcome Study in Kyoto] Registry Cohort-2, RESET [Randomized Evaluation of Sirolimus-Eluting Versus Everolimus-Eluting Stent Trial], and NEXT [NOBORI Biolimus-Eluting Versus XIENCE/PROMUS Everolimus-Eluting Stent Trial]), we compared risks for ischemic and bleeding events from 13 to 36 months after percutaneous coronary intervention among patients with a DAPT score ≥2 (high DS) and a DAPT score Results: Among 12 223 patients receiving drug-eluting stents who were free from ischemic or bleeding events at 13 months after percutaneous coronary intervention, 3944 patients had high DS and 8279 had low DS. The cumulative incidence of primary ischemic end point (myocardial infarction/stent thrombosis) was significantly higher in high DS than in low DS (1.5% versus 0.9%, P =0.002), whereas the cumulative incidence of primary bleeding end point (GUSTO moderate/severe) tended to be lower in high DS than in low DS (2.1% versus 2.7%, P =0.07). The cumulative incidences of cardiac death, myocardial infarction, and stent thrombosis were also significantly higher in high DS than in low DS (2.0% versus 1.4%, P =0.03; 1.5% versus 0.8%, P =0.002; 0.7% versus 0.3%, P P P =0.03, respectively). Conclusions: In the current population, the DAPT score successfully stratified ischemic and bleeding risks, although the ischemic event rate was remarkably low even in high DS. Further studies would be warranted to evaluate the utility of prolonged DAPT guided by the DAPT score.

Backgrounds: Patients with aortic stenosis (AS) have a high prevalence of aortic plaque. However, no data exist regarding the clinical significance and prognostic value of aortic plaque in AS patients. This study examines the impact of aortic plaque on the rate of progression and clinical outcomes of AS. Methods: We retrospectively investigated 1812 transesophageal echocardiographic examinations between 2008 and 2015. We selected 100 consecutive patients (mean age; 75.1 ± 7.4 years) who showed maximal aortic jet velocity (AV-Vel) ≥ 2.0 m/s by transthoracic echocardiography (TTE) and received follow-up TTE (mean follow-up duration 25 ± 17 months), and the mean progression rate of AV-Vel was calculated. Clinical and echocardiographic characteristics, including severity of aortic plaque, and cardiac events were examined. Results: At initial TTE, mean AV-Vel was 3.68 ± 0.94 m/s and mean aortic valve area 0.98 ± 0.32 cm2. Mean progression rate of AV-Vel was 0.41 m/s/year in 38 patients with severe aortic plaque, and − 0.03 m/s/year in the remaining 62 patients without severe aortic plaque. Severe aortic plaque (odds ratio[OR], 8.32) and hemodialysis (OR, 6.03) were independent predictors of rapid progression. The event-free survival rate at 3 years was significantly lower in patients with severe aortic plaque than in those without (52% vs 82%, p = 0.002). Severe aortic plaque (hazard ratio[HR], 2.89) and AV-Vel at initial TTE (HR, 3.28) were identified as independent predictors of cardiac events. Conclusion: Severe aortic plaque was a predictor of rapid progression and poor prognosis in AS patients. Evaluation of aortic plaque provides additional information regarding surgical scheduling and follow-up.

Immune light-chain (AL) amyloidosis with cardiac involvement is associated with a high mortality despite improved therapeutic regimens, but there are few reports on prognostic predictors and chronological changes in cardiac morphology and function. Prognosis and its predictors were evaluated in 36 consecutive patients with cardiac AL amyloidosis. Chronological changes in cardiac morphology and function were also evaluated. The median follow-up period was 0.95 years. The median survival time and the 3-year death-free rate after diagnosis in all-cause and cardiac deaths were 0.85 and 1.06 years and 26% and 36%, respectively. Differences in the median survival time due to left ventricular (LV) wall thickness at diagnosis were not evident. Being female and diastolic wall strain (DWS), as a measure of diastolic stiffness, were independent predictors of all-cause death in the multivariable analysis. The receiver operating characteristic analysis revealed that a DWS cut-off value of 0.189 had a sensitivity of 78% and a specificity of 72% for predicting all-cause death within 1 year after diagnosis (area under the curve = 0.726). The LV size and the stroke volume decreased and DWS worsened during the short-term follow-up period in patients who died within 1 year compared with patients who were alive after 1 year. The prognosis for patients with cardiac AL amyloidosis was poor, and DWS may be a significant predictor of prognosis. Narrowing of the LV cavity and progressive diastolic dysfunction were evident in patients with a poor prognosis.

Background Recently, the Embolic Risk French Calculator (ER-Calculator) was designed to predict symptomatic embolism (SE) associated with infective endocarditis (IE), but external validation has not been reported. This study aimed to determine predictors of SE and the diagnostic accuracy of the ER-Calculator in left-sided active IE among a Japanese population. Methods This retrospective cohort study included 166 consecutive patients with a definite diagnosis of left-sided IE from 1994 to 2015 in our institution. SE during the period after initiation of antibiotic therapy was defined as new SE and embolism during the period before initiation of antibiotic therapy was defined as previous embolism. The primary endpoint was new SE. Results The mean age of patients was 63 ± 17 years. New SE occurred in 23 (14%) patients at a median of 6 days (interquartile range: 2.5–12.5 days) after initiation of antibiotic therapy. The cumulative incidence of new SE at 12 weeks was 18.2%. The 2-week probability by the ER-Calculator as well as previously reported predictors, such as previous embolism, vegetation length (>10 mm), and their combination, were associated with a high risk of new SE. By receiver operating characteristic analysis, the area under the curve of the 2-week probability by the ER-Calculator for prediction of new SE was 0.75 and the optimal cut-off value was 8%. A 2-week probability >8% by the ER-Calculator was the most useful predictor of new SE (hazard ratio 3.63, 95% confidence interval 1.50–8.37; p = 0.006), which was more remarkable for fatal embolic events (hazard ratio 13.9, 95% confidence interval 3.19–95.4; p = 0.004). Conclusions The ER-Calculator is a useful predictor of new SE. Predictive ability is more remarkable for critical embolic events.

Background Patients with atrial fibrillation (AF) without structural heart diseases can show severe tricuspid regurgitation (TR), especially among aged people. The aim of this study was to clarify the actual management, prognosis, and prognostic factors for severe isolated TR associated with AF without structural heart diseases. Methods and results We retrospectively investigated actual management in 178 consecutive patients with severe isolated TR associated with AF between 1999 and 2011 in our institution. Prognosis and its predictors were also investigated in 115 patients (68 persistent TR and 47 transient TR) who were followed-up for >1year. During the follow-up period (mean: 5.9years), event free rate from death due to right-sided heart failure (RHF) was 97% at 5years. Persistent TR was associated with higher risk of hospitalization due to RHF than transient TR (log-rank P=0.048) and death due to RHF were all seen in patients with persistent TR who experienced hospitalization due to RHF. Among patients with persistent TR, right ventricular outflow tract dimension >35.3mm, right atrial area >40.3cm 2 , and tenting height >2.1mm were associated with higher risk of hospitalization due to RHF (adjusted hazard ratio: 3.32, 3.83, and 2.89, respectively; P=0.003, 0.002, and 0.009, respectively). Conclusion The prognosis of severe isolated TR associated with AF was good with a focus on cardiac death. However, the incidence of cardiac death increased among patients who experienced hospitalization due to RHF. Larger right ventricular outflow tract dimension, right atrial area and tenting height were predictors of hospitalization due to RHF.

Although very late recurrences (VLRs) (first recurrence >12 months after the last catheter ablation) of atrial fibrillation (AF) after multiple catheter ablation procedures are rare, it remains a critical issue. The risk factors for VLRs remain largely unclear. From December 2011 to April 2014, 253 patients underwent an initial catheter ablation. Of the 253 patients, 21 had AF recurrences within 1 year after the last catheter ablation. The study was conducted in the remaining 232 patients. Left ventricular diastolic dysfunction (LVDD) was assessed by echocardiography using composite categories with tissue Doppler imaging and left atrial volume measurements, i.e., a septal e′

There are few longitudinal data regarding aortic plaque. This study aimed to examine chronological changes in aortic plaques with transesophageal echocardiography (TEE), and to clarify the risk factors of aortic plaque progression. Among 2,675 consecutive patients who underwent TEE, we retrospectively investigated 252 patients who underwent follow-up TEE with an interval >3 years. The thickness and morphology of aortic plaques were examined. Chronological changes in aortic plaques were investigated by comparing baseline and follow-up TEE. Clinical factors, laboratory data, and medications were evaluated. Among 252 study patients, the grade of aortic plaques was unchanged in 213 (group U), but progression was observed in 32 (group P) and regression in 7 patients (group R). Patients in group P were older; they had a higher prevalence of coronary artery disease, hypertension, smoking habit, and moderate or severe plaque at baseline TEE; more patients were using statins and no warfarin; and they had higher creatinine levels than those in group U. In multivariate analysis, moderate or severe plaques at baseline TEE were the strongest predictor of plaque progression. Among 50 patients who showed moderate or severe plaque at baseline TEE, smoking habit and no anticoagulation therapy were predictors of plaque progression. In conclusion, aortic plaques should be followed up using TEE in patients with moderate or severe plaque at baseline TEE.

Aortic valve replacement (AVR) for chronic aortic regurgitation (AR) with a decreased ejection fraction (EF) leads to improvement in left ventricular (LV) function, but there are no reports on late recurrence of LV dysfunction over long-term after AVR. This study aimed to identify frequency and predictors of late recurrent LV dysfunction after AVR.We retrospectively investigated 58 consecutive patients undergoing AVR for severe chronic AR and with follow-up echocardiography for5years after AVR. Late recurrence of LV dysfunction was defined as an EF of50% late after AVR and a 10% reduction in the EF compared with that observed at 1year after AVR.The mean follow-up period was 10.3±5.2years. The preoperative EF was50% in 21 (36%) patients, but it was normalized at 1year after AVR in all patients except for one. However, late recurrence of LV dysfunction developed in 7 (12%) of the 58 patients. These patients showed significantly higher LV end-diastolic and end-systolic diameters before and at 1year after AVR, a lower EF and relative wall thickness before AVR, a higher LV mass index at 1year after AVR, and a higher incidence of preoperative and postoperative atrial fibrillation than those without late recurrence.Late recurrent LV dysfunction may occur after AVR for severe chronic AR despite EF being once normalized. Early surgery proceeding remarkable LV enlargement and maintaining sinus rhythm are important for LV function over the long-term after AVR.

Background Impact of hyponatremia improvement on prognosis in patients with acute decompensated heart failure (ADHF) remains unclear. Methods Patients hospitalized for ADHF at 19 hospitals in Japan were enrolled between October 2014 and March 2016. Hyponatremia was defined as serum sodium concentration less than 135 mmol/l. Primary endpoint was composite of all-cause death and heart failure rehospitalization one year after discharge. Results Among 3805 patients enrolled, 486 patients with hyponatremia at admission showed higher in-hospital mortality (13.3% vs. 5.4%, p Patient characteristics Group P (n=143) Group I (n=253) p value Age (years) 81 (72–86) 81 (72–87) 0.73 Female 71 (49.7) 110 (43.5) 0.24 Ischemic etiology 42 (29.4) 81 (32.0) 0.58 Prior hospitalization 62 (43.7) 98 (39.5) 0.42 SBP at admission (mmHg) 140±36 144±38 0.40 HR at admission (bpm) 92±23 95±29 0.27 Atrial Fibrillation 47 (32.9) 103 (40.7) 0.12 NYHA class IV 60 (42.2) 138 (54.8) 0.02 Intravenous inotropic use 35 (24.5) 59 (23.3) 0.80 LVEF Conclusion Improvement of hyponatremia at discharge was not associated with better prognosis in patients hospitalized for ADHF.

The changes in cardiac function that occur after pericardiocentesis are unclear. An understanding of the effect of pericardiocentesis on right ventricular (RV) and left ventricular (LV) function is clinically important. This study was performed to assess RV and LV function with echocardiography before and after pericardiocentesis. In total, 19 consecutive patients who underwent pericardiocentesis for more than moderate pericardial effusion were prospectively enrolled from August 2015 to October 2017. Comprehensive transthoracic echocardiography was performed before, immediately after (within 3 h), and 1 day after pericardiocentesis to investigate the changes in RV and LV function. The mean age of all patients was 72.6 ± 12.2 years. No pericardiocentesis-related complications occurred during the procedure, but one patient died of right heart failure 8 h after pericardiocentesis. After pericardiocentesis, RV inflow and outflow diameters increased (p 0.05 versus values before pericardiocentesis), and the parameters of RV function (tricuspid annular plane systolic excursion, tricuspid lateral annular systolic velocity, fractional area change, and RV free wall longitudinal strain) significantly decreased (p 0.001 versus values before pericardiocentesis). These abnormal values or RV dysfunction remained 1 day after pericardiocentesis (p 0.05 versus values immediately after pericardiocentesis). Conversely, no parameters of LV function changed after pericardiocentesis. Of 19 patients, 13 patients showed RV dysfunction immediately after pericardiocentesis and 6 patients did not. RV free wall longitudinal strain before pericardiocentesis in patients with post-procedural RV dysfunction was reduced compared to those without post-procedural RV dysfunction ( - 18.9 ± 3.6 versus - 28.4 ± 6.3%; p = 0.005). The area under the curve values for prediction of post-procedural RV dysfunction was 0.910 for RV free wall longitudinal strain. The occurrence of RV dysfunction after pericardiocentesis should be given more attention, and pre-procedural RV free wall longitudinal strain may be a predictor of post-procedural RV dysfunction.

In patients with subpulmonic infundibular ventricular septal defect (VSD), postoperative progression of aortic regurgitation (AR) sometimes occurs despite early operation before the development of AR. The present study was aimed to identify the occurrence rate and predictors of late AR progression after VSD repair alone.We retrospectively investigated 91 consecutive patients who underwent subpulmonic infundibular VSD repair alone and were followed up with echocardiography for3 years postoperatively. The clinical backgrounds and chronological changes in postoperative AR were evaluated.The median follow-up period after VSD repair was 13.4 years. Among 91 patients, 7 patients showed postoperative AR progression (AR progression group) and 84 patients did not (No AR progression group). No patient in AR progression group revealed more than moderate AR preoperatively. The incidence of postoperative VSD leakage was significantly higher in AR progression group than No AR progression group (43.0% vs 2.4%, respectively; p0.01). No significant differences were present in sex, age, preoperative AR severity, VSD diameter or rate of cusp herniation. All patients in AR progression group showed deformity of the right coronary cusp or leaflet, resulting in AR progression.Among patients with subpulmonic infundibular VSD, the incidence of late AR progression after VSD repair alone was unexpectedly high (7.7%). Postoperative VSD leakage may be a significant risk factor for late AR progression. Long-term follow-up of postoperative AR is needed even for patients who undergo VSD repair alone.

Background Prothrombin time (PT) can provide a qualitative assessment of the relative intensity of anticoagulation by rivaroxaban. More than ten types of assay are available for the measurement of PT in clinical settings, but it is not yet fully understood whether their interactions with rivaroxaban are uniform or inconsistent. Methods We examined 139 blood samples from patients taking rivaroxaban. We measured PT using five different commercially available assays. We also evaluated the estimated rivaroxaban concentration using a chromogenic anti-factor Xa assay. Results The median estimated concentration of rivaroxaban was 192 ng/ml (interquartile range 85–284 ng/ml). The correlation coefficient (r) between PT and the estimated concentrations of rivaroxaban was as follows: Thromborel S, r = 0.768; Thrombocheck PT, r = 0.861; Coagpia PT-N, r = 0.909; Neoplastin Plus, r = 0.882; and Triniclot PT Excel S, r = 0.870. The gradients of the regression plots differed more than fourfold, and the standard deviation of the regression line ranged from 1.001 to 2.980, which tended to be higher for the assays with the higher regression slope gradients. Conclusion The estimated concentration of rivaroxaban varied greatly depending on the assay, so the PT measured in patients taking rivaroxaban should be interpreted with caution.

An asymptomatic 40-year-old woman with a first-degree atrioventricular block presented a right atrial mass in transthoracic echocardiograms. Transesophageal echocardiograms showed abnormally thickened tissue on the interatrial septum, which extended around the aortic annulus. Multimodality examinations demonstrated lesions in the heart, lungs, liver, and spleen, suggesting sarcoidosis. She was diagnosed with cardiac sarcoidosis after we detected granulomas in a lung specimen. A right atrial mass shrunk following steroid therapy. We should therefore consider the possibility of cardiac sarcoidosis when we see wall thickening and a mass echo in the atrium. These signs may point to an early-phase lesion of cardiac sarcoidosis.

Background There are few data on the long-term prognosis and chronological changes in left ventricular (LV) function after aortic valve replacement (AVR) in patients with severe chronic aortic regurgitation (AR) among the Japanese population.Methods and Results:We retrospectively investigated the long-term prognosis in 80 consecutive patients with severe chronic AR who underwent AVR. Additionally, 65 patients with follow-up echocardiography at 1 year after AVR were investigated to evaluate chronological changes in LV function. The mean follow-up period was 8.9±5.2 years. Freedom from all-cause death and cardiac death at 10 years after AVR was 76% and 91%, respectively. The preoperative ejection fraction (EF) and estimated glomerular filtration rate were independent predictors of all-cause death. Preoperative EF, LV end-systolic diameter, and diabetes might be useful predictors of cardiac death. Among the 65 patients with follow-up echocardiographic data, LV function had normalized at 1 year after AVR in all patients, except for 2 who died of cardiac causes in the long-term after AVR. LV end-diastolic diameter, LV end-systolic diameter, and EF at 1 year after AVR might be useful predictors of long-term cardiac death. Conclusions In patients with severe chronic AR, preoperative LV dysfunction is remarkably improved at 1 year after AVR. Pre- and postoperative echocardiographic data are important for predicting long-term outcome after AVR. (Circ J 2016; 80: 2460-2467).

Cardiac inflammatory pseudotumor (IPT) is a rare disease and it is difficult to make a definitive diagnosis. A 77-year-old woman was admitted because of weight loss, fatigue and palpitation. Her medical history included ANCA associated systemic vasculitis and hepatitis-B. She had been taking oral

A 37-year-old man presented with heart failure caused by severe aortic regurgitation (AR). He had a history of being involved in a traffic accident 3 months earlier. Imaging tests at admission detected no abnormalities in the aortic valve or aortic wall; however, the left coronary cusp prolapsed slightly on transthoracic echocardiography. He underwent aortic valve replacement because of uncontrolled heart failure and severe AR. Intraoperatively, the intima of the aortic wall just above the commissure of the left and right coronary cusps was torn to the short axial direction. Local aortic tear was the final diagnosis for the subacute AR.

We describe a 38-year-old male who experienced several episodes of syncope after having ventricular fibrillation. The electrocardiographic monitoring after his hospitalization revealed repetitive polymorphic ventricular tachycardias. All polymorphic ventricular tachycardias were consistently initiated by a short-coupled monomorphic ventricular premature contraction (VPC). This VPC was suggested to originate from the inferoposterior region of the right ventricle (RV). Radiofrequency catheter ablation targeting the VPC was successfully performed, and the CARTO merge system (Biosense Webster Inc., Diamond Bar, CA, USA) revealed that the culprit region was the root of the posterior papillary muscle of the RV. A subsequent follow-up of 15 months has been uneventful.

BACKGROUND Constrictive pericarditis (CP) is characterized by impaired diastolic cardiac function leading to heart failure. Pericardiectomy is considered effective treatment for CP, but data on long-term clinical outcomes after pericardiectomy are limited.Methods and Results:We retrospectively investigated 45 consecutive patients (mean age, 59±14 years) who underwent pericardiectomy for CP. Preoperative clinical factors, parameters of cardiac catheterization, and cardiac events were examined. Cardiac events were defined as hospitalization owing to heart failure or cardiac death.Median follow-up was 5.7 years. CP etiology was idiopathic in 16 patients, post-cardiac surgery (CS) in 21, tuberculosis-related in 4, non-tuberculosis infection-related in 2, infarction-related in 1, and post-radiation in 1. The 5-year event-free survival was 65%. Patients with idiopathic CP and tuberculosis-related CP had favorable outcomes compared with post-CS CP (5-year event-free survival: idiopathic, 80%; tuberculosis, 100%; post-CS, 52%). Higher age (hazard ratio: 2.51), preoperative atrial fibrillation (3.25), advanced New York Heart Association class (3.92), and increased pulmonary artery pressure (1.06) were predictors of cardiac events. Patients with postoperative right-atrial pressure ≥9 mmHg had lower event-free survival than those with right-atrial pressure

Mutations in SCN5A have been reported to cause several types of hereditary arrhythmias (overlap syndrome). We herein report two patients with the overlapping phenotypes of juvenile sick sinus syndrome (SSS) and Brugada syndrome (BrS). The proband was a man who was in his twenties and had been diagnosed with both SSS and ventricular tachycardia (VT). A pilsicainide challenge test revealed a coved type ST segment elevation. His teenage brother also suffered from SSS, but no VT had been documented. A pilsicainide challenge failed to produce a Brugada-type ST elevation, but there was a marked prolongation of the His-ventricle interval. Their electrocardiograms at rest did not display any Brugada-type ST elevations. We identified a novel SCN5A (F1775Lfs*15) mutation in both patients, even though there was a phenotype discrepancy.

We herein report a case of a 52-year-old woman who presented with a history of recurrent palpitations that occurred during swallowing solid food. On a Holter electrocardiogram, paroxysmal atrial tachycardias (PATs) were detected while eating. We mapped the right atrium (RA) with a multipolar mapping catheter while she swallowed a rice ball and it was revealed that the earliest endocardial breakthrough was on the anterior septal side near the superior vena cava junction of the RA. We successfully eliminated PAT at both the site in the RA and the adjacent right superior pulmonary vein ostium. After ablation, no PAT was documented while eating.

Background The association between atrial septal defect (ASD) and mitral regurgitation (MR) is well known. However, data about the predictors of changes in MR after ASD closure are limited. The purpose of this study was to clarify the chronological changes in MR after ASD closure and the predictors of aggravation of MR. Methods In this single-center cohort study, we retrospectively investigated 129 consecutive adult patients (mean age, 53 ± 14 years) who underwent surgical ASD closure between 1987 and 2014. The MR grade was qualitatively classified as none, mild, moderate, or severe by echocardiography. Aggravation of MR was defined as an increase by two or more grades after ASD closure. Clinical factors and echocardiographic and catheterization data were evaluated. Results The mean follow-up period was 77 months. Aggravation of MR after ASD closure occurred in 16 patients (12%). The rate of perioperative atrial fibrillation was higher (odds ratio, 5.89), the anterior mitral leaflet was thicker (odds ratio, 1.91), and the posterior mitral leaflet length was shorter (odds ratio, 1.58) in patients with aggravation of MR than in the remaining 113 patients. The mechanism of aggravated MR was poor coaptation associated with annular dilatation, thickened anterior mitral leaflet, and shortened posterior mitral leaflet. Conclusions A thickened anterior mitral leaflet and shortened posterior mitral leaflet, combined with mitral annular dilation associated with atrial fibrillation and restored left ventricular geometry, may aggravate MR after ASD closure. Careful follow-up is needed for patients with atrial fibrillation, a thickened anterior mitral leaflet, or a shortened posterior mitral leaflet.

Background The optimal timing of aortic valve replacement (AVR) is controversial in patients with asymptomatic severe aortic stenosis (AS) except when very severe. Prediction of progression of severe AS is helpful in deciding on the timing of AVR. The purpose of this study was to clarify the predictors of progression rate and clinical outcomes of severe AS. Methods and results We retrospectively investigated 140 consecutive patients with asymptomatic severe AS (aortic valve area [AVA], 0.75-1.0 cm(2)). First-year progression rate and annual progression rate of AVA and of aortic jet velocity (AV-Vel) were calculated. Cardiac events were examined and the predictors of rapid progression and cardiac events were analyzed. The median follow-up period was 36 months. The median annual progression rate was -0.05 cm(2)/year for AVA and 0.22 m/s/year for AV-Vel. Dyslipidemia, moderate-severe calcification, and first-year AV-Vel progression ≥0.22 m/s/year were independent predictors of cardiac events. Cardiac event-free rate was lower in patients with AV-Vel first-year progression rate ≥0.22 m/s/year than in those with a lower rate. Diabetes and moderate-severe calcification were related to first-year rapid progression. Conclusions The annual progression rate of severe AS was -0.05 cm(2)/year for AVA and 0.22 m/s/year for AV-Vel. Patients with first-year rapid progression or severely calcified aortic valve should be carefully observed while considering an early operation. (Circ J 2016; 80: 1863-1869).

A 60-year-old man was admitted due to the onset of right coronary artery (RCA) aneurysms. Coronary angiography showed two RCA aneurysms and focal stenosis with limitations in the blood flow. Balloon angioplasty was performed. However, the follow-up coronary angiography showed restenosis, an enlarged proximal aneurysm and a newly formed aneurysm. The serum immunoglobulin G4 level was elevated to 1,350 mg/dL and fluorodeoxyglucose positron emission tomography showed increased uptake in the ascending aorta, so the patient was diagnosed with immunoglobulin G4-related vascular disease. The prevention of further enlargement of the aneurysms and an improvement in the RCA flow were achieved with steroid therapy. Steroid therapy may therefore be effective for immunoglobulin G4-related vascular disease.

Pericardiocentesis is performed to treat cardiac tamponade or diagnose the cause of pericardial effusion. Cardiogenic shock with right ventricular (RV) dysfunction is a rare complication after pericardiocentesis. We report a case of an 82-year-old man who suddenly suffered cardiopulmonary arrest 12 h after pericardiocentesis. A transthoracic echocardiogram showed remarkable RV dysfunction and tricuspid valve dysfunction. Tricuspid valve closure was severely impaired, and the tricuspid regurgitation signal showed laminar flow with an early peak. However, after treatment with high-dose inotropic drugs, hemodynamic parameters gradually recovered. A transthoracic echocardiogram performed 24 h later showed improved motion of the RV and the tricuspid valve, resulting in a reduction in tricuspid regurgitation. RV and tricuspid valve dysfunction after pericardiocentesis needs to be recognized as a critical complication. Physicians also need to pay attention to not only the amount of drainage but also underlying RV dysfunction.

Aim Aspirin is an antiplatelet drug widely used for the prevention of cardiovascular disease; however, it is known to increase bleeding events. A low response to aspirin was reported to correlate with poor prognosis in patients undergoing antiplatelet therapy with aspirin. The aim of this study was to evaluate the impact of the antiplatelet activity of aspirin on cardiovascular and bleeding events in Japanese patients. Methods We analyzed the clinical course of 239 Japanese patients undergoing antiplatelet therapy with aspirin for a median of 64 months in this study. Their residual platelet reactivity was examined at enrollment and after 2 years. The co-primary endpoints were the occurrence of major adverse cardiac and cerebrovascular events (MACCEs) and bleeding events. Results The annual incidence of MACCEs and major bleeding events was 3.7% and 0.48%, respectively. With defined criteria, 67 patients (28%) were classified as low responders based on the platelet aggregability measured at enrollment. Low response to aspirin was not associated with increased MACCEs, while it clearly increased MACCEs in patients less than 70 years old (low responders 36.9% vs. responders 14.8%, log rank p=0.008). Five major types of bleeding occurred in the responders, but not in low responders, although the difference was not statistically significant (p= 0.07). Conclusion Low response to aspirin was not associated with the increase of long-term MACCEs, while it increased MACCEs in patients less than 70 years old; however, it tended to decrease major bleeding events in Japanese patients.

Background: Severe tricuspid regurgitation (TR) sometimes develops late after left-sided valve surgery without left heart failure, pulmonary hypertension or rheumatic tricuspid valve. The purpose of the present study was to investigate clinical characteristics and mechanisms of severe isolated TR late after left-sided valve surgery. Methods and Results: A total of 372 consecutive patients who underwent left-sided valve surgery between 1990 and 2003 and who were followed up with echocardiography for at least 5 years, were retrospectively investigated. The mean follow-up period was 9.4 years. Clinical background, preoperative and postoperative echocardiographic parameters were evaluated. Among the 372 patients, severe isolated TR was detected in 23 patients, which developed at a mean of 8.6 years after surgery. Twenty-two of 23 patients had undergone mitral valve surgery. Multivariate logistic regression analysis identified the presence of preoperative atrial fibrillation and preoperative ejection fraction as independent determinants for the development of severe isolated TR. In patients with severe isolated TR, the tricuspid annular diameter and the right atrial area were already enlarged early after surgery and both of these increased prior to TR progression. Conclusions: Severe isolated TR developing late after mitral valve surgery is not uncommon, thus it is important to recognize this disease entity. Annular dilatation was the main cause of isolated TR and serial echocardiographic data are important to detect progression of isolated TR and to assess its mechanisms. (Circ J 2011; 75: 2902-2907)

We report the case of a 72-year-old man with a nonsustained ventricular tachycardia and a history of palpitations. He had a severely deformed thorax since childhood due to spinal caries. An integrated computed tomography image of the outflow tract region from the CartoSound® system revealed the detailed anatomical information around the origin of the tachycardia and that the left anterior descending coronary artery was very close (

Aim Severe gastrointestinal bleeding sometimes occurs in patients with aortic stenosis (AS), known as Heyde's syndrome. This syndrome is thought to be caused by acquired von Willebrand syndrome and is characterized by reduced large von Willebrand factor (vWF) multimers. However, the relationship between the severity of AS and loss of large vWF multimers is unclear. Methods We examined 31 consecutive patients with severe AS. Quantitative evaluation for loss of large vWF multimers was performed using the conventional large vWF ratio and novel large vWF multimer index. This novel index was defined as the ratio of large multimers of patients to those of controls. Results Loss of large vWF multimers, defined as the large vWF multimer index ＜80%, was detected in 21 patients (67.7%). The large vWF multimer ratio and the large vWF multimer index were inversely correlated with the peak aortic gradient (R = -0.58, p=0.0007, and R=-0.64, p＜0.0001, respectively). Anemia defined as hemoglobin ＜9.0 g/dl was observed in 12 patients (38.7%), who were regarded as Heyde's syndrome. Aortic valve replacement was performed in 7 of these patients, resulting in the improvement of anemia in all patients from a hemoglobin concentration of 7.5±1.0 g/dl preoperatively to 12.4±1.3 g/dl postoperatively (p＜0.0001). Conclusions Acquired von Willebrand syndrome may be a differential diagnosis in patients with AS with anemia. The prevalence of AS-associated acquired von Willebrand syndrome is higher than anticipated.

Double-chambered right ventricle (DCRV) is a rare condition. Stenosis of DCRV is progressive, and early surgical intervention is recommended for patients whose symptoms and/or pressure overload of right ventricular (RV) inflow are progressive. However, there are few data regarding the postoperative course of DCRV, and the surgical indications for asymptomatic patients remain to be determined. We retrospectively investigated 38 consecutive patients who were diagnosed with DCRV and underwent surgical intervention from 1981 to 2009. Moreover, we identified 29 patients in whom long-term follow-up transthoracic echocardiographic data were available and investigated the postoperative recurrence of DCRV by evaluating the systolic pressure of RV inflow before, immediately, and in the long term after surgical intervention. The mean follow-up period was 11.0 ± 8.8 years. There were no deaths and no surgical reinterventions during the long-term follow-up period. Among 29 patients with long-term follow-up echocardiographic data, there was no recurrence of DCRV. In these patients, the systolic pressure of RV inflow by echocardiography before, immediately, and long-term after surgical intervention was 80 ± 26, 30 ± 11, and 25 ± 6 mm Hg, respectively. In conclusion, the surgical outcomes and postoperative prognosis beyond 10 years of DCRV are favorable, and neither recurrence of DCRV nor fatal arrhythmias develop during the long-term follow-up period.

Sleep-disordered breathing (SDB) is recognized as a primary factor or mediator of atrial fibrillation (AF). We hypothesized that the severity of SDB among AF ablation candidates would be associated with left ventricular diastolic dysfunction (LVDD) even for subclinical SDB. A total of 246 patients hospitalized for initial pulmonary vein isolation (PVI) were analyzed. Known SDB cases were excluded. We measured the oxygen desaturation index (ODI) by pulse oximetry overnight as an indicator of SDB, and classified SDB severity by 3 % ODI as normal (ODI

Atrial fibrillation (AF) increases the left atrial (LA) volume and deteriorates LA function. Whether successful radiofrequency catheter ablation (RFCA) of persistent AF can reverse this process has not been yet established.Patients with persistent AF undergoing RFCA were evaluated with pre- and post- (at 6-months of follow-up) procedural multislice computed tomography (MSCT). The LA functions were assessed through LA time-volume curves.The study population consisted of 44 patients [age 64 (interquartile ranges: 58, 70) years old, 93% male]. Among those, 31 patients (70%) maintained sinus rhythm during the follow-up (no recurrence group; NR group). The remaining 13 patients were classified as the recurrence group (R group). A significant decrease in the minimal and maximal LA volumes was observed in both groups, although this was less pronounced in the R group. Only the NR group had an improvement in the LA expansion index [18% (13, 25) vs. 37% (23, 43), p0.001], ejection fraction [15% (11, 20) vs. 27% (19, 30), p0.001] and conduit function [17 ml/m(2) (13, 20) vs. 25 ml/m(2) (20, 34), p0.001]. An improvement of LV function was also observed only in the NR group.LA anatomical and functional reverse remodeling after RFCA of persistent AF was demonstrated by MSCT during follow-up, which was more pronounced in patients without AF recurrence.

Aim The optimal treatment strategy for patients with aortic atheroma is not well established because data regarding medical treatment for such patients are lacking, especially with respect to the Japanese population. The purpose of this study was to clarify the effects of medical treatment on the risk of embolic events and mortality in patients with severe aortic plaque. Methods We retrospectively investigated 75 consecutive patients with severe aortic plaque detected on transesophageal echocardiography (TEE) between 1995 and 2005. The occurrence of embolic events and all-cause death in the period after TEE was assessed. The cumulative incidence of subsequent embolic events and death was evaluated in relation to specific medical treatments, including statins, antiplatelet drugs and warfarin. Results Embolic events occurred in 27 patients (36%) and death occurred in 37 patients (49%) during follow-up (5.6±3.0 years). The patients who experienced embolic events had a significantly higher prevalence of previous embolic events, atrial fibrillation and hemodialysis than the patients who did not experience embolic events. Univariate and multivariate analyses showed that the use of statins and/or antiplatelet drugs was significantly associated with a low incidence of death but not with a low incidence of embolic events. On the other hand, warfarin exhibited neither beneficial nor harmful effects on the incidence of embolic events or death. Conclusions Statin and antiplatelet drugs have beneficial effects on the prognosis of patients with severe aortic plaque diagnosed on TEE.