Trifecta is a stented bioprosthetic heart valve with a bovine pericardial sheet externally mounted on a titanium stent. This valve is applied only for aortic valve replacement (AVR), providing excellent hemodynamics and extremely low incidence of structural valve deterioration (SVD). A 76-year-old woman presented with dyspnea on effort 24 months after AVR with a 21-mm Trifecta valve. Echocardiography revealed severe aortic regurgitation with prolapse of a cusp of Trifecta valve, which suggested that she developed acute heart failure due to early SVD. In the operation, Trifecta valve had a cusp tear near the commissure with circumferential fibrous pannus ingrowth only at the inflow side. There was neither calcification nor infection. The Trifecta valve was successfully replaced with a new porcine bioprosthesis.

In recent years, the function of pharmacological chaperones as a “thermodynamic stabilizer” has been attracting attention in combination therapy. The coadministration of a pharmacological chaperone and recombinant human acid α-glucosidase (rhGAA) leads to improved stability and maturation by binding to the folded state of the rhGAA and thereby promotes enzyme delivery. This study provides the first example of a strategy to design a high-affinity ligand toward lysosomal acid α-glucosidase (GAA) focusing on alkyl branches on 1-deoxynojirimycin (DNJ); 5-C-heptyl-DNJ produced a nanomolar affinity for GAA with a K i value of 0.0047 μM, which is 13-fold more potent than DNJ. The protein thermal shift assay revealed that 10 μM 5-C-heptyl-DNJ increased the midpoint of the protein denaturation temperature (T m) to 73.6 °C from 58.6 °C in the absence of the ligand, significantly improving the thermal stability of rhGAA. Furthermore, 5-C-heptyl-DNJ dose dependency increased intracellular GAA activities in Pompe patient’s fibroblasts with the M519V mutation. The introduction of C5 alkyl branches on DNJ provides a new molecular strategy for pharmacological chaperone therapy for Pompe disease, which may lead to the development of higher-affinity and practically useful chaperones.

In lower exercise capacity patients with HF had slower [Formula: see text]O and [Formula: see text]E kinetics during and after the 6MWT.

L-ido-Deoxynojirimycin (L-ido-DNJ) itself showed no affinity for human lysosomal acid α-glucosidase (GAA), whereas 5-C-methyl-L-ido-DNJ showed a strong affinity for GAA, comparable to the glucose analog DNJ, with a Ki value...

Purpose: Consensus articles that explore rehabilitation exercise for heart failure (HF) mainly focus on stable patients with chronic HF. Results from investigations that focus on the relationship between clinical outcomes and exercise during rehabilitation of patients with acute heart failure (AHF) have produced insufficient data. The aim of this study was to evaluate the correlation between clinical outcomes and dyspnea during in-hospital early rehabilitation in patients with AHF. Methods: Dyspnea was measured using a 5-point Likert scale (5PLS) during rest and at the initiation of upright sitting and standing. Dyspnea was defined as 5PLS ≥2. The primary endpoint was combined all-cause death or rehospitalization for HF. Results: A total of 221 patients were included in this study; 81 patients (37%) died or were hospitalized during the follow-up period. In patients with dyspnea during upright sitting and standing, the event-free ratio was significantly lower compared with patients without dyspnea (P = .008 and P < .001, respectively). Body mass index (hazard ratio [HR] = 0.91, P = .011), noninvasive positive pressure ventilation usage (HR = 1.96, P = .042), and 5PLS ≥2 at the initiation of standing (HR = 2.63, P = .008) were detected as predictors of primary endpoint. New York Heart Association class IV at admission (OR = 3.17, P = .0114) and pre-admission Katz ADL index <6 (OR = 3.76, P = .0007) were isolated as risk factors for dyspnea when standing. Conclusions: Dyspnea during standing was associated with unfavorable events in patients with AHF as a comprehensive indicator.

Background The incidence of postoperative venous thromboembolism (VTE) is high in patients with inflammatory bowel disease. We aimed to analyze the incidence and predictive factors of postoperative VTE in patients with ulcerative colitis. Methods Patients with ulcerative colitis who underwent colon and rectum surgery during 2010–2018 were included. We retrospectively investigated the incidence of postoperative VTE. Results A total of 140 colorectal surgery cases were included. Postoperative VTE was detected in 24 (17.1 %). Portal–mesenteric venous thrombosis was the most frequent VTE (18 cases; 75 %); of these, 15 patients underwent total proctocolectomy (TPC) with ileal pouch–anal anastomosis (IPAA). In univariate analysis, VTE occurred more frequently in patients with neoplasia than in those refractory to medications (27.2 % vs. 12.5 %; p < 0.031). TPC with IPAA was more often associated with VTE development (28 %) than total colectomy (10.5 %) or proctectomy (5.9 %). On logistic regression analysis, TPC with IPAA, total colectomy, long operation time (> 4 h), and high serum D-dimer level (> 5.3 µg/mL) on the day following surgery were identified as predictive risk factors. Conclusions Postoperative VTE occurred frequently and asymptomatically, especially after TPC with IPAA. Serum D-dimer level on the day after surgery may be a useful predictor of VTE.

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