Left sinus of Valsalva aneurysm causing acute myocardial infarction with compression of the left main coronary trunk.

Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly that commonly originates from the right or noncoronary sinuses and rarely from the left sinus. SVA is usually diagnosed in the setting of clinical sequelae of a rupture. We herein report a case of an unruptured left SVA presenting as acute myocardial infarction. A 54-year-old woman with a history of radical operation for patent ductus arteriosus in childhood was transferred to our hospital. An electrocardiogram indicated extensive myocardial ischemia with ST elevation. Urgent coronary angiography was performed but was unable to identify the left coronary artery. Subsequent aortography and computed tomography revealed a large SVA originating from the left sinus and compressing the left coronary artery. The patient died after approximately one month of intensive care, including mechanical circulatory support and coronary artery bypass grafting. Autopsy confirmed that the left main coronary trunk was stretched and compressed by the SVA and revealed unexpected atherosclerosis in the left anterior descending artery. Although a left SVA is an extremely rare anomaly, it occasionally provokes fatal myocardial infarction. Since an SVA might hinder performing percutaneous coronary intervention, cardiac surgery should be considered when myocardial ischemia is recognized. Learning objective: We herein report a case of an unruptured left sinus of Valsalva aneurysm (SVA) with acute myocardial infarction. Urgent percutaneous coronary intervention (PCI) was unsuccessful, as the left coronary artery was compressed by the SVA. The patient died after intensive care, including coronary artery bypass grafting (CABG). SVA, especially from the left sinus, is extremely rare but occasionally provokes myocardial infarction by compressing the coronary arteries. Because SVA might hinder PCI, CABG should be considered when myocardial ischemia is recognized.

Initial results of transcatheter modification of left atrial appendage by obliteration with device in patients with nonvalvular atrial fibrillation: Real-world data from the TERMINATOR registry.

BACKGROUND: Percutaneous left atrial appendage closure (LAAC) has increased for those who need alternative to long-term anticoagulation with non-valvular atrial fibrillation (NVAF). METHODS AND RESULTS: From September 2019, after initiating WATCHMAN (Boston Scientific, Maple Grove, MN, USA) device implantation, we established Transcatheter Modification of Left Atrial Appendage by Obliteration with Device in Patients from the NVAF (TERMINATOR) registry. Utilizing 729 patients' data until January 2022, we analyzed percutaneous LAAC data regarding this real-world multicenter prospective registry. A total of 729 patients were enrolled. Average age was 74.9 years and 28.5 % were female. Paroxysmal AF was 37.9 % with average CHADS2 3.2, CHA2DS2-VASc 4.7, and HAS-BLED score of 3.4. WATCHMAN implantation was successful in 99.0 %. All-cause deaths were 3.2 %, and 1.2 % cardiovascular or unexplained deaths occurred during follow-up [median 222, interquartile range (IQR: 93-464) days]. Stroke occurred in 2.2 %, and the composite endpoint which included cardiovascular or unexplained death, stroke, and systemic embolism were counted as 3.4 % [median 221, (IQR: 93-464) days]. Major bleeding defined as BARC type 3 or 5 was seen in 3.7 %, and there was 8.6 % of all bleeding events in total [median 219, (IQR: 93-464) days]. CONCLUSIONS: These preliminary data demonstrated percutaneous LAAC with WATCHMAN device might have a potential to reduce stroke and bleeding events for patients with NVAF. Further investigation is mandatory to confirm the long-term results of this strategy using this transcatheter local therapy instead of life-long systemic anticoagulation.

Severe mitral regurgitation in chronic adult T-cell leukemia/lymphoma with granulomatous valvular inflammation.

Adult T-cell leukemia/lymphoma (ATLL) is a mature peripheral T-cell neoplasm caused by human T-cell leukemia virus type I (HTLV-1) infection. Besides the oncogenic property, HTLV-1 causes HTLV-1-associated myelopathy/tropical spastic paraparesis and certain inflammatory diseases via a complex host immune response to latent virus infection. Cardiac involvement of ATLL is rare, with the majority of cases being disclosed in postmortem autopsy in patients with advanced subtypes. We herein report the case of a 64-year-old female patient with indolent chronic ATLL with severe mitral regurgitation. Although the condition of ATLL was stable, dyspnea on exertion gradually progressed over the course of three years and echocardiography revealed marked thickening of the mitral valve. Finally, the patient experienced hemodynamic collapse with atrial fibrillation and underwent surgical valve replacement. The removed mitral valve was grossly edematous and swollen. A histological examination revealed a granulomatous reaction mimicking the active phase of rheumatic valvulitis, with the infiltration of ATLL cells that were immunohistochemically positive for CD3, CD4, FoxP3, HLA-DRα, and CCR4. The postoperative course was uneventful, with the exception that Sjögren's syndrome was noted. The history of rheumatic fever was unclear, and such unique valvular pathology was presumably related to autoimmune mechanisms associated with HTLV-1 infection. Learning objective: We report a case of chronic adult T-cell leukemia/lymphoma (ATLL) with isolated valvular infiltration with a unique histology of granulomatous reaction. Human T-cell leukemia virus type I infection may accelerate autoimmune reactions and cardiac inflammation, irrespective of indolent clinical subtype. Among ATLL cases, possible progression of valvular insufficiency and heart failure in patients with cardiac symptoms should be carefully evaluated.

[Can Echocardiography Adjust Corrected Contrast Injection Condition in Coronary CT Angiography?]

PURPOSE: The purposes were to search which factor of cardiac function in echocardiography correlates with the CT value, to correct contrast injection conditions with cardiac function in addition to suppress error in the contrast effect between patients, and to achieve the target CT value (350 HU) in coronary computed tomography angiography (CCTA). METHODS: In 112 patients (conventional group), the contrast material was administered at a fractional dose (FD) of 21 mgI/kg/s. We measured the aortic CT value in the coronary origin part. In 112 patients (correction group), the contrast material was administered at corrected injection conditions with the most correlated functional factor and CT value. RESULTS: The CT value of the conventional group was an average of 400.8±51.5 HU. The most correlated factor with the CT value was stroke volume [SV (r=-0.555)]. The CT value of the conventional group was an average of 360±46 HU. The case of the aim CT level was improved from 46% to 74%. In the correction group, the average value of FD was 18.5 mgI/kg/s. This enabled the reduction of the contrast material in 95% of patients. CONCLUSION: The best correlation was obtained between the CT value of coronary arteries and SV. The contrast medium injection conditions were corrected for cardiac function in addition to body weight. As a result, we were able to control the CCTA target CT value of 300 to 400 HU at our hospital.

Management of anticoagulant therapy using a portable point-of-care international normalized ratio device and social networking service in a patient with a left ventricular assist device.

Device infection and stroke are still frequently reported as complications of left ventricular assist devices, and strict management of anticoagulation therapy is sometimes difficult at the time of infection status. We report the case of a 55-year-old man with a HeartMate II (Abbott, Inc., Abbott Park, IL, USA) as a bridge to cardiac transplantation. The patient measured his prothrombin time-international normalized ratio (PT-INR) by himself using a point-of-care device at home and reported the result promptly on a social networking service (SNS). Physicians instructed the patient on how to adjust his dose of warfarin based on the result and suggested the next time of measurement on the SNS. Until cardiac transplantation, we adjusted the dose of warfarin 106 times using the SNS because of unexpected PT-INR fluctuations caused by antibiotics. The time in the therapeutic range was maintained at 83.2% without complications, including major bleeding, stroke, or pump replacement; however, there was transient intra-pump thrombosis triggered by severe dehydration due to hyperthyroidism. .

Strategy for estimating optimal heart rate in refractory heart failure with relative sinus bradycardia: A case report.

Relative bradycardia (mild sinus bradycardia) is one of the major barriers for the effective treatment of hemodynamically unstable patients with heart failure and reduced cardiac output. We report a case of a man aged 58 years with an old broad anterior myocardial infarction and relative bradycardia (about 60 bpm) suffering from symptoms of congestive heart failure at rest in spite of optimal medical therapy, including the use of the inotropes. Transvenous atrial pacing during right heart catheterization indicated that an increase in heart rate (up to 80 bpm) improved hemodynamics immediately. Implantation of a pacemaker (atrial pacing of 80 bpm) was effective for stabilizing the heart failure symptoms. Transvenous atrial pacing during right heart catheterization is effective for estimating the optimal heart rate in patients with heart failure and relative bradycardia. .

A reduction of BMI predicts the risk of rehospitalization and cardiac death in non-obese patients with heart failure.

BACKGROUND: Low body mass index (BMI) has been associated with decreased survival in patients with heart failure (HF), although Obesity is an important risk factor for cardiovascular disease. HF patients with a relatively higher BMI tend to live longer, which is known as "Obesity Paradox". However, cardiac cachexia is another determinant of prognosis in HF patients. This study investigated whether a change in BMI is associated with either prognosis or frequency of hospitalizations in patients with HF. METHODS: We correlated changes in BMI to prognosis and frequency of hospitalizations in patients who were hospitalized for decompensated HF. A total of 971 HF patients were initially evaluated, and 81 patients with repeat HF admissions were included. RESULTS: The average change in BMI was -0.05 ±â€¯0.15, -0.87 ±â€¯0.56, -1.03 ±â€¯0.34, and -1.97 ±â€¯0.33 in patients who were hospitalized twice, three times, four times, and over five times, respectively. The reduction in BMI correlated with the frequency of hospitalizations (P < 0.01). We compared patients with increased BMI (group I, n = 38) versus decreased BMI (group D, n = 43) between the first and second discharge. The rate of hospitalization in group D was higher than in group I, and group D had a lower survival rate. The reduction of BMI was a significant and independent risk factor for cardiac death (HR, 4.17; 95% CI, 1.53 to 14.6). CONCLUSIONS: Losing body weight in HF patients was a significant predictive factor of the frequency of hospitalizations and increased mortality.

Successful percutaneous retrieval of a micra transcatheter pacing system at 8 weeks after implantation.

An 86-year-old woman suffering from repeated methicillin-resistant Staphylococcus aureus (MRSA) bacteremia underwent percutaneous lead extraction using an excimer laser. Since negative blood cultures were confirmed three times after lead extraction under intravenous infusion of anti-MRSA drugs, a Micra transcatheter pacing system (Micra TPS) was implanted 7 days after the lead extraction. Although infusion of anti-MRSA drugs was continued for 5 weeks, MRSA was isolated in four separate samples of blood cultures 3 weeks after the discontinuation of the anti-MRSA therapy. The micra TPS was successfully retrieved using a steerable sheath and snare at 8 weeks after implantation.

Acute Mitral Regurgitation of Unknown Etiology Associated with Disseminated Intravascular Coagulation Eventually Diagnosed as Enterococcus Faecalis Infective Endocarditis by Mitral Valve Surgery.

BACKGROUND Infective endocarditis is prevalent worldwide and the modified Duke criteria have been used universally to diagnose this condition. However, making the correct diagnosis is rather difficult because the clinical presentation and findings of blood tests are non-specific. CASE REPORT A 70-year-old female complaining of dyspnea for 5 days with acute mitral regurgitation was transferred to our hospital. She had acute heart and respiratory failure and disseminated intravascular coagulation. Although infective endocarditis was suspected, repeated blood cultures and transesophageal echocardiography could not reveal any findings of infective endocarditis. Because the etiology of her condition was not determined by various examinations, mitral annuloplasty was required to treat her mitral regurgitation, and was performed for definitive diagnosis and treatment revealing the presence of vegetation on the mitral valve. Enterococcus faecalis was detected by cultures of the mitral valve and blood after the surgery. CONCLUSIONS It can be very difficult to diagnose infective endocarditis correctly, especially when a case fails to fulfill the modified Duke criteria. In such a case, only cardiac surgery might enable us to make an accurate diagnosis and save a patient's life.

Very late perforation by a Riata implantable cardioverter-defibrillator lead-A case report.

Cardiac perforation by a transvenous lead is an uncommon but serious complication. We herein present a case of very late perforation of a Riata implantable cardioverter-defibrillator lead, which occurred 8 years after implantation. The patient was successfully treated with percutaneous lead extraction using an excimer laser. Lead perforation should be considered, even after years from implantation.

The first multicenter, randomized, controlled trial of home telemonitoring for Japanese patients with heart failure: home telemonitoring study for patients with heart failure (HOMES-HF).

Home telemonitoring is becoming more important to home medical care for patients with heart failure. Since there are no data on home telemonitoring for Japanese patients with heart failure, we investigated its effect on cardiovascular outcomes. The HOMES-HF study was the first multicenter, open-label, randomized, controlled trial (RCT) to elucidate the effectiveness of home telemonitoring of physiological data, such as body weight, blood pressure, and pulse rate, for Japanese patients with heart failure (UMIN Clinical Trials Registry 000006839). The primary end-point was a composite of all-cause death or rehospitalization due to worsening heart failure. We analyzed 181 recently hospitalized patients with heart failure who were randomly assigned to a telemonitoring group (n = 90) or a usual care group (n = 91). The mean follow-up period was 15 (range 0-31) months. There was no statistically significant difference in the primary end-point between groups [hazard ratio (HR), 0.95; 95% confidence interval (CI), 0.548-1.648; p = 0.572]. Home telemonitoring for Japanese patients with heart failure was feasible; however, beneficial effects in addition to those of usual care were not demonstrated. Further investigation of more patients with severe heart failure, participation of home medical care providers, and use of a more integrated home telemonitoring system emphasizing communication as well as monitoring of symptoms and physiological data are required.

The relationship between neutrophil to lymphocyte ratio, endothelial function, and severity in patients with obstructive sleep apnea.

BACKGROUND: Obstructive sleep apnea (OSA) is characterized by repetitive intermittent hypoxia and reoxygenation during sleep with elevated oxidative stress and promotes the development of atherosclerosis, as demonstrated by vascular dysfunction and chronic inflammation. An increased neutrophil to lymphocyte ratio (NLR) has been recognized to be a novel inflammatory biomarker for systemic inflammation. OBJECTIVES: We evaluated whether the NLR reflects the severity of OSA and if continuous positive airway pressure (CPAP) treatment ameliorates the endothelial function and NLR in patients with OSA. METHODS: We enrolled 95 patients with suspected OSA and 29 patients who received CPAP therapy for 3 months. We evaluated the number of endothelial progenitor cells (EPCs) and NLR, the levels of nitric oxide (NOx) and asymmetric dimethylarginine (ADMA), and the endothelial function according to the flow-mediated dilatation (FMD) before and after CPAP treatment. RESULTS: The levels of apnea-hypopnea index demonstrated an inverse relationship with the FMD and a positive relationship with the NLR. Moreover, NLR is an independent factor suggested for the presence of severe OSA. CPAP therapy increased the levels of EPC and NOx and decreased the level of ADMA. CPAP treatment also improved the FMD and decreased the NLR. CONCLUSIONS: NLR and endothelial dysfunction significantly correlates with the severity of OSA and FMD and other biochemical parameters improved and NLR decreased significantly after CPAP treatment.

Successful treatment of primary cardiac lymphoma causing ST-elevation myocardial infarction by percutaneous coronary intervention combined with chemotherapy.

A 76-year-old immunocompetent woman presented to our hospital with general fatigue. Her blood pressure was 60/40 mm Hg and pulse rate was 110 bpm. An electrocardiogram showed ST-elevation in the II, III and aVF leads with complete atrioventricular block. An echocardiogram and CT revealed pericardial effusion and a 6 cm solid tumour lying anterior to the heart. The right coronary artery (RCA) ran through the centre of the tumour, which bulged into the right atrium for 35 mm and vibrated. Emergent coronary angiography revealed 99% stenosis with delay at the proximal RCA; however, intravascular ultrasound showed no atheromatous changes, and the RCA was compressed by the extravascular mass. Successful coronary stenting improved the coronary flow. The following day, a biopsy was performed via thoracotomy without any events, the results of which showed diffuse-type large B-cell lymphoma histologically. Chemotherapy gradually reduced the tumour size, and the patient became stable haemodynamically.

Home telemonitoring study for Japanese patients with heart failure (HOMES-HF): protocol for a multicentre randomised controlled trial.

INTRODUCTION: Despite the encouraging results from several randomised controlled trials (RCTs) and meta-analyses, the ability of home telemonitoring for heart failure (HF) to improve patient outcomes remains controversial as a consequence of the two recent large-scale RCTs. However, it has been suggested that there is a subgroup of patients with HF who may benefit from telemonitoring. The aim of the present study was to investigate whether an HF management programme using telemonitoring could improve outcomes in patients with HF under the Japanese healthcare system. METHODS AND ANALYSIS: The Home Telemonitoring Study for Japanese Patients with Heart Failure (HOMES-HF) study is a prospective, multicentre RCT to investigate the effectiveness of home telemonitoring on the primary composite endpoint of all-cause death and rehospitalisation due to worsening HF in recently admitted HF patients (aged 20 and older, New York Heart Association classes II-III). The telemonitoring system is an automated physiological monitoring system including body weight, blood pressure and pulse rate by full-time nurses 7 days a week. Additionally, the system was designed to make it a high priority to support patient's self-care instead of an early detection of HF decompensation. A total sample size of 420 patients is planned according to the Schoenfeld and Richter method. Eligible patients are randomly assigned via a website to either the telemonitoring group or the usual care group by using a minimisation method with biased-coin assignment balancing on age, left ventricular ejection fraction and a history of ischaemic heart disease. Participants will be enrolled until August 2013 and followed until August 2014. Time to events will be estimated using the Kaplan-Meier method, and HRs and 95% CIs will be calculated using the Cox proportional hazards models with stratification factors. TRIAL REGISTRATION: The study is registered at UMIN Clinical Trials Registry (UMIN000006839).

[Late-onset type ornitine transcarbamirase deficiency (OTCD) with fulminant onset following a fatal prognosis].

A 16-year-old man was admitted to our hospital with nausea, general fatigue, and consciousness disturbance along with extreme hyperammoniemia eight days after the onset of symptoms. Familial history and the high concentration of orotic acid in urine lead us to a diagnosis of OTCD. We immediately initiated intensive treatment such as continuous hemodiafiltration and sodium benzoate administration; however, the patient died twelve days after admission. Since OTCD is not so rare and can be found in all ages, it should be considered fundamental for evaluation of hyperammoniemia. This case suggested that for a better prognosis of OTCD patients it is very important to prevent such an onset, and to make an as early as possible diagnosis and start to treatment.