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Background: The efficacy of a therapy for patients with transthyretin amyloid cardiomyopathy (ATTR-CM) has not been proven, but tafamidis has been associated with favorable outcomes. However, echocardiographic details of the association of tafamidis with cardiac morphology remain undetermined. Moreover, whether the efficacy of tafamidis varies with the degree of cardiac involvement remains unknown. Using echocardiography, this study investigated the impact of tafamidis on the cardiac morphology of patients with ATTR-CM. Methods and Results: Of 52 consecutive patients with biopsy-proven ATTR-CM at Kobe University Hospital, we included 41 for whom details of follow-up echocardiographic examinations after the administration of tafamidis were available. All patients underwent standard and speckle-tracking echocardiography before and a mean (±SD) of 16±8 months after the administration of tafamidis. No significant changes were observed in any representative echocardiographic parameters after the administration of tafamidis. Furthermore, there were no significant changes observed in subgroup analyses (e.g., left ventricular [LV] ejection fraction ≥50% vs.

Background Heterogeneity of heart failure (HF) with preserved ejection fraction (HFpEF) would contribute to the difficulty in identifying effective treatments, and interest in the phenogrouping of HFpEF as a potential means for predicting patients who respond to cardioprotective drugs has been increasing. Methods We studied 468 first-hospitalized HFpEF patients among 1971 acute-hospitalized HF patients from KUNIUMI Registry Acute Cohort. The primary endpoint was defined as HF-rehospitalization and cardiovascular death over a median follow-up period of 508 days. Results In HFpEF patients with left ventricular hypertrophy (LVH), patients prescribed renin-angiotensin-aldosterone-system (RAAS) inhibitors had similar outcomes compared to those without (HR, 0.77; 95% CI 0.51-1.16; p = 0.21), and the outcome was also similar between patients with and without RAAS inhibitors’ prescription in HFpEF patients without LVH. Moreover, in HFpEF patients with LVH and mild-moderate chronic kidney disease (CKD), which was determined as an estimated glomerular filtration rate (eGFR) of 30-60 mL/min/1.73 m2, patients prescribed RAAS inhibitors had significantly favorable outcomes compared to those without (HR 0.39; 95% CI 0.19-0.80; p = 0.01). In HFpEF patients with LVH and severe CKD, which was defined as eGFR

Background: Because the effectiveness of strengthening guideline-based therapy (GBT) to prevent heart failure (HF) rehospitalization of chronic HF patients remains unclear, this study investigated the characteristics of HF patients in the Kobe University Heart Failure Registry in Awaji Medical Center (KUNIUMI) acute cohort. Methods and Results: We studied 254 rehospitalized HF patients from the KUNIUMI Registry. Optimized GBT was defined as a Class I or IIa recommendation for chronic HF based on the guidelines of the Japanese Circulation Society. The primary endpoint was all-cause death or first HF rehospitalization after discharge. Outcomes tended to be more favorable for patients who had rather than had not received optimized GBT (hazard ratio [HR] 0.82; 95% confidence interval [CI] 0.57-1.19; P=0.27). Similarly, among New York Heart Association (NYHA) Class IV patients, outcomes tended to be more favorable for those who had rather than had not undergone optimized GBT (HR 0.73; 95% CI 0.47-1.12; P=0.15). Importantly, outcomes were significantly more favorable among NYHA Class IV patients aged

A sigmoid septum is a state of angulation between the basal interventricular septum and the ascending aorta. Although considered to have no clinical importance, it may cause left ventricular outflow tract obstruction (LVOTO) in a hypercontractile state. Percutaneous transluminal septal myocardial ablation (PTSMA) is a catheter-based therapy aimed at improving drug-refractory symptoms in patients with hypertrophic obstructive cardiomyopathy. Few studies have reported the use of PTSMA for patients with LVOTO caused by sigmoid septum. We present a successful case of a 71-year-old female patient who presented with a long history of exertional dyspnea. The presence of a sigmoid septum was revealed on echocardiography. At rest, the left ventricular outflow tract pressure gradient was low and there were no signs of systolic anterior motion (SAM) of the mitral valve; however, during Valsalva maneuver, the gradient increased significantly, and SAM could be seen. We successfully performed PTSMA, resulting in a significant lowering of the pressure gradient and disappearance of SAM. The patient’s symptoms dramatically improved by the following day.

Background: The clinical frailty scale (CFS) predicts late mortality in patients undergoing transcatheter aortic valve replacement. We evaluated the CFS and other parameters associated with 1-year mortality after balloon aortic valvuloplasty (BAV). Methods and Results: Between January 2013 and May 2018, 148 patients with severe aortic stenosis (AS) who underwent BAV at the present hospital were enrolled. We recorded pre-procedural CFS grade, baseline characteristics, echocardiographic, and hemodynamic parameters. To investigate the potential risk to patients before BAV, we evaluated the Society of Thoracic Surgeons (STS) score. After patients who underwent surgical aortic valve replacement, transcatheter aortic valve replacement or repeat BAV were excluded, we investigated 1-year survival. Of 127 patients, 41 (32.3%) died ≤1 year after BAV, 8 of whom (19.5% of all-cause deaths) had cardiac deaths. Higher grade of CFS and STS score significantly correlated with 1-year mortality. Severe frailty and the high operative risk group (CFS ≥7 and STS score ≥8.7%) had an extremely poor prognosis (1-year mortality, 81.2%). Conclusions: In this BAV cohort, severe frailty was a predictor of 1-year mortality in elderly patients with severe AS.

A 58-year-old woman with pallor on her left hand due to chronic hemodialysis presented with a recent intractable skin ulcer on her left 3rd finger; the skin perfusion pressure (SPP) was 19 mmHg. Preoperative angiography revealed an occluded proximal left radial artery, no communication between the ulnar and superficial palmar arteries, several collaterals from the left ulnar to the radial artery, and no visualization of the finger arteries. Successful endovascular therapy to the occluded radial artery increased flow to the arteriovenous fistula (AVF), but not to the fingertips. Slightly compressing the AVF augmented the flow and wound blush at the wound sites on the 3rd fingertip, leading to a diagnosis of hemodialysis access-induced distal ischemia (HAIDI). Surgical AVF banding with intra-operative SPP monitoring improved the SPP to 34 mmHg, leading to complete wound healing over 1 month with a preserved AVF. We performed a bilateral temporal artery biopsy and diagnosed giant cell arteritis. As the angiographic wound blush at wound sites is reportedly an important factor for wound healing, angiography with AVF manual compression is essential to diagnose HAIDI and evaluate the blood flow for wound healing.

Patient initials or identifier number O.M ### Relevant clinical history and physical exam A 58 years-old woman suffered from paleness on her left hand during hemodialysis for several years. She presented to our hospital with an intractable skin ulcer emerged on her left 3rd finger (Figure 1

Patient initials or identifier number E.S ### Relevant clinical history and physical exam A 87 years-old woman suffers from coldness of her each finger every winter. This ambulatory woman had rest pain in her right foot 2 months ago, and referred to our hospital with a deeply erythematous on