Yusuke Yoshikawa

Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index

AIMnSevere gastrointestinal bleeding sometimes occurs in patients with aortic stenosis (AS), known as Heydes 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.nnnMETHODSnWe 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.nnnRESULTSnLoss 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 Heydes 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).nnnCONCLUSIONSnAcquired 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.

Multiple Coronary Artery Aneurysms and Thoracic Aortitis Associated with IgG4-related Disease

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.

Actual management and prognosis of severe isolated tricuspid regurgitation associated with atrial fibrillation without structural heart disease

BACKGROUNDnPatients 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.nnnMETHODS AND RESULTSnWe 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 >40.3cm2, 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).nnnCONCLUSIONnThe 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.

Relationship between diastolic ventricular dysfunction and subclinical sleep-disordered breathing in atrial fibrillation ablation candidates

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 3xa0% ODI as normal (ODIxa0<xa05 events/h), mild (ODIxa0≤xa05 to <15 events/h), or moderate-to-severe (ODIxa0≥15 events/h). The LVDD was assessed by echocardiography using combined categories with tissue Doppler imaging and left atrial (LA) volume measurement. Among the participants, 42 patients (17.1xa0%) had LVDD. The prevalence of LVDD increased with the SDB severity from 8.6xa0% (normal) to 12.7xa0% (mild) to 40.0xa0% (moderate-to-severe SDB) (pxa0<xa00.0001). In the multivariate logistic regression analysis, the odds ratio of having LVDD in the moderate-to-severe SDB group (ODIxa0≥xa015) vs. normal group (ODIxa0<xa05) was 5.96 (95xa0% CI, 2.10–19.00, Pxa0=xa00.006). The presence of moderate-to-severe SDB in AF ablation candidates adversely affected LV diastolic function even during a subclinical state of SDB.

Diagnostic accuracy of the Embolic Risk French Calculator for symptomatic embolism with infective endocarditis among Japanese population

BACKGROUNDnRecently, 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.nnnMETHODSnThis 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.nnnRESULTSnThe 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 (>10mm), 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).nnnCONCLUSIONSnThe ER-Calculator is a useful predictor of new SE. Predictive ability is more remarkable for critical embolic events.

Impact of aortic plaque on progression rate and prognosis of aortic stenosis

BACKGROUNDSnPatients 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.nnnMETHODSnWe retrospectively investigated 1812 transesophageal echocardiographic examinations between 2008 and 2015. We selected 100 consecutive patients (mean age; 75.1±7.4years) who showed maximal aortic jet velocity (AV-Vel) ≥2.0m/s by transthoracic echocardiography (TTE) and received follow-up TTE (mean follow-up duration 25±17months), and the mean progression rate of AV-Vel was calculated. Clinical and echocardiographic characteristics, including severity of aortic plaque, and cardiac events were examined.nnnRESULTSnAt initial TTE, mean AV-Vel was 3.68±0.94m/s and mean aortic valve area 0.98±0.32cm2. Mean progression rate of AV-Vel was 0.41m/s/year in 38 patients with severe aortic plaque, and -0.03m/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 3years 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.nnnCONCLUSIONnSevere 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.

Risk Factors of Aortic Plaque Progression Evaluated by Long-Term Follow-Up Data With Transesophageal Echocardiography

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 >3xa0years. 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.

Successful Ablation with a Multipolar Mapping Catheter for Swallowing-induced Atrial Tachycardia

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.

Detecting Cardiac Sarcoidosis with a Right Atrial Mass Using Transthoracic Echocardiography

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.

Usefulness of a wearable cardioverter defibrillator combined with catheter ablation for ventricular tachyarrhythmia storms after a myocardial infarction: A case report.

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.