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A 77‐year‐old woman with symptomatic paroxysmal atrial fibrillation (PAF) underwent pulmonary vein isolation (PVI), but subsequently experienced recurrence. In the second session, unidirectional left atrium (LA)‐left superior pulmonary vein (LSPV) conduction was revealed to exist at the carina of the LSPV. Left pulmonary vein (LPV) pacing performed in a cycle between 300 and 260 ms revealed rate‐dependent pulmonary vein (PV)‐LA conduction, and the location was estimated to be in the roof of the LSPV. PV isolation was achieved after ablation of two gaps. Consideration of the presence of rate‐dependent gaps may be useful to confirm bidirectional block lines after ablation.

Background The relationship between the timing of the first early recurrence and late recurrence after a single catheter ablation procedure for atrial fibrillation is controversial. Methods The Efficacy of Short-Term Use of Antiarrhythmic Drugs After Catheter Ablation for Atrial Fibrillation trial followed 2038 patients who underwent radiofrequency catheter ablation for atrial fibrillation. Results Of the patients, 907 (45%) had early recurrences within 90 days after the initial ablation. We divided these patients into two groups according to the timing of the first early recurrence episode, namely the ER1 group (early recurrence during the early phase; 0–30 days, n = 814) and ER2 group (early recurrence during the late phase; 31–90 days, n = 93). Three years after ablation, patients with early recurrences had a significantly lower event-free rate from late recurrences after a 90-day blanking period than patients without early recurrences (36.2% and 74.2%, respectively; log-rank, P Conclusion Early recurrences were strongly associated with late recurrences, especially in patients with the first recurrence episode at >1 month within the blanking period after a single ablation procedure. Therefore, these patients should undergo close observation during follow-up, when they had especially with non-paroxysmal atrial fibrillation.

Background Catheter ablation (CA) for atrial fibrillation (AF) is widely performed. However, the indication for CA in patients with asymptomatic persistent AF is still controversial. Methods Among 259 consecutive patients who were hospitalized for initial CA of AF, a total of 45 patients who had asymptomatic persistent AF were retrospectively analyzed. Quality of life (QOL) before and 1 year after CA was evaluated, and changes in the cardiac function over 5 years after CA were also examined. QOL was assessed using the AF QOL questionnaire (AFQLQ) developed by the Japanese Heart Rhythm Society. In addition, cardiac function was assessed by measuring the plasma B‐type natriuretic peptide (BNP) level, left ventricular ejection fraction (LVEF), left atrial diameter (LAD) with transthoracic echocardiogram, and left atrial (LA) volume with computed tomography (CT). Results The AFQLQ significantly improved after CA in terms of “symptom frequency” and “activity limits and mental anxiety.” The plasma BNP level, LVEF, and LAD significantly improved in the first 3 months after the first CA, with no significant changes thereafter (from 149.0 pg/dL [95% confidence intervals {CI}, 114.5‐183.5 pg/dL] to 49.8 pg/dL [95% CI, 26.5‐70.1], P, Before and after catheter ablation (CA) for asymptomatic persistent atrial fibrillation, quality of life (QOL) was assessed using the AF QOL questionnaire (AFQLQ), and cardiac function was assessed by measuring the plasma B‐type natriuretic peptide (BNP) level, left ventricular ejection fraction (LVEF), and left atrial diameter (LAD) with transthoracic echocardiogram and left atrial (LA) volume with computed tomography (CT). Improvement in QOL and cardiac function after CA for asymptomatic persistent AF was revealed.

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.

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.

Background/Introduction Cather ablation (CA) for atrial fibrillation (AF) as rhythm control therapy has widely spread. However, the indication of CA for the patients with asymptomatic AF is controversial (2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS). Purpose This study was aimed to examine the effect of CA for asymptomatic AF patients. Methods In a total of 522 consecutive patients who were hospitalized for the initial CA for AF, 123 patients (23.6%) who were asymptomatic were retrospectively analyzed. "Asymptomatic AF" was defined when the patients had no complaints during the medical interviews. Quality of life (QOL) was evaluated with the AF QOL Questionnaire score (AFQLQ, invented by the Japanese Society of Electrocardiology) before CA and at 1 year after a single CA. Results A total of 79 patients were persistent AF (64.2%). Sinus rhythm maintenance rate at 3 yeas after CA was 57.0% in a single session and 84.8% in multiple sessions. In cardiac echo data at baseline, 3 months, 6 months, and 1 year after CA, left ventricular ejection fraction (LVEF) and left atrium diameter (LAD) were improved in the initial three months after a single CA (LVEF; 62.8 ± 8.0%→64.4 ± 6.5%, p = 0.045, LAD; 39.7 ± 6.1mm→35.3 ± 7.0mm, p = 0.0002). In LA volume measured with CT before and after CA, LA reverse remodeling was observed (102.7 ± 32.3ml→72.4 ± 24.1ml, p Conclusions The improvement of LVEF and LA reverse remodeling can be expected at the early stage after CA, because of the reduction of AF burden. Moreover, the further improvement of QOL can be expected after CA, even in the patients with "asymptomatic" AF.

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.

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.

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).

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.

We report a tachyarrhythmia case of a 32-year-old female with a single ventricle and heterotaxy syndrome. She had surgery involving a total cavo-pulmonary connection procedure using an extra-cardiac conduit (EC) at the age of 17 years. A tachycardia was repetitively induced with single atrial extrastimuli. An activation map was created revealing a centrifugal propagation pattern from the high atrial wall adjacent to the EC. At that site, a structure resembling the crista terminalis was recognized with intracardiac echocardiography. Therefore, high output energy was required to eliminate the tachycardia. It was thought to be a sinoatrial nodal reentrant tachycardia.

Aims Radiofrequency catheter ablation (RFCA) has become widely used for drug-refractory atrial fibrillation (AF). However, there is a paucity of data on the long-term clinical outcomes after RFCA for AF. The aim of the present study was to investigate the very long-term outcomes after RFCA for AF in a large number of consecutive patients. Methods and results In this retrospective single-center study, we evaluated very long-term follow-up results in 1206 consecutive patients undergoing first RFCA for AF. The primary outcomes were adverse outcomes at 30-day as a safety outcome measure and event-free rates from recurrent atrial tachyarrhythmias as efficacy outcome measures. Final follow-up rate reached 99.3% with a mean follow-up duration of 5.0±2.5years. The incidence of overall 30-day adverse outcomes was 3.6% without death. The 10-year event-free rates from recurrent atrial tachyarrhythmias after the initial and last procedures were 46.9% and 76.4%, respectively. Arrhythmia recurrence occurred most commonly during the first year and decreased beyond 3-year, although it continued to occur at an annual rate of 2.0% and 1.3%, respectively, throughout the 10-year follow-up period. The cumulative 10-year incidences of stroke and major bleeding were 4.2% and 3.5%, respectively, with annual rates of 0.3%. Discontinuation rate of oral anticoagulation at 1-, 3-, and 10-year was 34.6%, 53.4%, 58.0% and 61.9%. Conclusions RFCA for AF provided favorable very long-term arrhythmia-free survival without much safety concerns. The 10-year rates of stroke and major bleeding were low even with discontinuation of oral anticoagulation in a large proportion of patients.

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.

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.

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

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 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.

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

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 (

We report a case of a 60-year-old man with recurrent poly- and monomorphic ventricular tachycardia related to a recent myocardial infarction. Due to drug-refractory ventricular tachycardia despite complete revascularization, he underwent catheter ablation. Afterwards, he was fitted with a wearable cardioverter defibrillator. Three months later, no ventricular tachycardia had been recorded and an electrophysiologic study failed to induce an episode. Thus, wearable cardioverter defibrillators are useful bridging devices pending a final decision to implant a cardioverter defibrillator.

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.

Background: Pulmonary vein isolation (PVI) has been used effectively to treat atrial fibrillation (AF). However, one of the most important limitations of catheter ablation for AF is the rate of recurrence of atrial tachyarrhythmias, due predominantly to electrical reconnection of the pulmonary veins (PVs). Therefore, it would be useful if we could detect electrical reconnection of the PVs noninvasively, using multi-slice computed tomography (MSCT). We hypothesized that the absence of PV contraction could represent persistent electrical PV isolation after catheter ablation. Materials and methods: Twenty-eight patients (59.5±11.2 years, 23 males) who underwent the second session for the recurrent AF were enrolled. MSCT was performed twice, before the first session and before the second session. Images were reconstructed at 10 phases of one cardiac cycle (from 5% to 95% of the R-R interval). Using multi-planar reconstruction, the location of the PV ostium was defined, and the ejection fraction (EF) of each PV was calculated as the volume change of the first 10mm from the ostium using Simpson's disc methods. Each pulmonary vein was divided into the two groups according to the presence of electrical reconnection. The EFs of each PV (right superior PV: RSPV, right inferior PV: RIPV, left superior PV: LSPV, left inferior PV: LIPV) were calculated in the first and the second MSCT scan. Results: The number of reconnected PVs were 15 in RSPV, 14 in RIPV, 12 in LSPV and 12 in LIPV, respectively. In PVs without electrical reconnection, the EFs of PVs significantly decreased in RSPV, RIPV and LSPV. However, in PVs with electrical reconnection, the EFs of PVs did not change significantly in all PVs (see table). View this table: Conclusion: MSCT may be useful for the non-invasive evaluation of electrical PV reconnection.

Background: Efficacy and safety of intravenous amiodarone (IV-AMD) for Japanese patients in a real clinical practice have not been well evaluated.Methods and Results: Between Feb. 2008 and Feb. 2011, a total of 69 patients received IV-AMD in Kyoto University Hospital. Mean age was 67±17 years, and 46 (67%) were male. Sixty six patients (96%) had structural heart diseases, including ischemic heart diseases in 38 (55%), non-ischemic cardiomyopathies in 11 (16%), and valve diseases in 9 (13%). The target arrhythmias were ventricular tachyarrhythmias in 39 (56.5%), and atrial tachyarrhythmias in 30 (43.5%). IV-AMD was administered for a median duration of 2.0 (1–11) days, which was effective in 69% of patients with ventricular tachyarrhythmia and in 70% of patients with atrial tachyarrhythmia. Adverse effects requiring discontinuation of the drug occurred in 9 patients (13.0%); bradycardia in 4 (5.8%), low blood pressure in 2 (2.9%), QT prolongation in 2 (2.9%), and torsade de pointes in 1 (1.4%). There was no interstitial pneumonia or hypothyroidism.Conclusions: IV-AMD was effective for both ventricular and atrial tachyarrhythmia in approximately 70% of patients with structural heart diseases. Care should be taken for adverse effects, especially for bradycardia, hypotension and QT prolongation.

Previous reports have shown that septal accessory pathways (APs) occasionally cause ventricular dyssynchrony and left ventricular (LV) dysfunction. However, right and left free-wall APs rarely affect LV function. A 52-year old male was admitted to our hospital due to NYHA class-IV heart failure. He had been diagnosed as dilated cardiomyopathy a year ago, and had received optimal medical therapy including ACE inhibitor, beta-blocker, diuretics, spironolactone, and digitalis. His ECG showed wide QRS (180 ms) with delta wave due to Type-B WPW syndrome. His UCG showed marked LV enlargement and dysfunction (LVDD=80 mm, LVEF=10%) with prominent ventricular dyssynchrony. Immediately after successful elimination of the AP located at posterolateral tricuspid annulus by radiofrequency catheter ablation (RFCA), his heart failure symptom markedly improved, and his BNP level decreased from 1392 to 401 (pg/ml). Because of prolonged QRS duration (120 ms) and suspected residual ventricular dyssynchrony by Tissue-Doppler UCG, he underwent CRT-D implantation 3 days after RFCA, although the device was set at DDDR-60 with long AV delay to preserve intrinsic ventricular conduction and excitation. After 6 months, his heart failure status was NYHA class-II. His UCG showed marked LV reverse remodeling (LVDD=52 mm, LVEF=41%), and his BNP level was 24 (pg/ml). This was considered a case of cardiomyopathy caused by ventricular dyssynchrony due to right free-wall AP.

Background: Activated partial thromboplastin time (aPTT) is recommended for monitoring anticoagulant activity in dabigatran-treated patients; however, there are limited data in Japanese patients. To clarify the relationship between plasma dabigatran concentration and aPTT, we analyzed plasma dabigatran concentration and aPTT at various time points following administration of oral dabigatran in a Japanese hospital. Methods: We enrolled 149 patients (316 blood samples) with non-valvular atrial fibrillation (NVAF) who were taking dabigatran. Patients had a mean age of 66.6±10.0 years (range: 35–84) and 66% were men. Plasma dabigatran concentrations and aPTT were measured using the Hemoclot® direct thrombin inhibitor assay and Thrombocheck aPTT-SLA®, respectively. Samples were classified into eight groups according to elapsed times in hours since oral administration of dabigatran. Results: Significantly higher dabigatran concentrations were observed in samples obtained from patients with low creatinine clearance (CLCr) (CLCr