Acquired von Willebrand syndrome in patients treated with veno-arterial extracorporeal membrane oxygenation.

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 < 0.0001), indicating that much higher grade of aVWS occurred in patients with VA ECMO than severe aortic stenosis patients. All the 5 patients treated with VA ECMO developed aVWS that was much more severe than in patients with severe aortic stenosis.

Subacute aortic regurgitation due to traumatic tear in the aortic wall.

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. .

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

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±7.4years) 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. RESULTS: At 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. 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.

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

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 (>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). CONCLUSIONS: The ER-Calculator is a useful predictor of new SE. Predictive ability is more remarkable for critical embolic events.

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

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 >40.3cm2, 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). 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.

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.