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Although devastating complications during simple coarctation of aorta repair now occur less frequently, some unique cases still require extra caution. Here, we report a case of coarctation of the aorta with a rare anomaly in the circle of Willis and an aberrant right subclavian artery, which required a thorough surgical strategy that prevented cerebral malperfusion. © The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.Anemia is a major and currently poorly understood clinical manifestation of hematopoietic aging. Upon aging, hematopoietic clones harboring acquired leukemia-associated mutations expand and become detectable, now referred to as clonal hematopoiesis (CH). To investigate the relationship between CH and anemia of the elderly, we explored the landscape and dynamics of CH in older individuals with anemia. From the prospective, population-based Lifelines cohort (n = 167 729), we selected all individuals at least 60 years old who have anemia according to World Health Organization criteria (n = 676) and 11 matched control participants. Peripheral blood of 1298 individuals was analyzed for acquired mutations at a variant allele frequency (VAF) of 1% or higher in 27 driver genes. To track clonal evolution over time, we included all available follow-up samples (n = 943). CH was more frequently detected in individuals with anemia (46.6%) compared with control individuals (39.1%; P = .007). Although no differences were observed regarding commonly detected DTA mutations (DNMT3A, TET2, ASXL1) in individuals with anemia compared with control individuals, other mutations were enriched in the anemia cohort, including TP53 and SF3B1. Unlike individuals with nutrient deficiency (P = .84), individuals with anemia of chronic inflammation and unexplained anemia revealed a higher prevalence of CH (P = .035 and P = .017, respectively) compared with their matched control individuals. Follow-up analyses revealed that clones may expand and decline, generally showing only a subtle increase in VAF (mean, 0.56%) over the course of 44 months, irrespective of the presence of anemia. Specific mutations were associated with different growth rates and propensities to acquire an additional hit. In contrast to smaller clones ( less then 5% VAF), which did not affect overall survival, larger clones were associated with increased risk for death. © 2020 by The American Society of Hematology.AIMS Intravascular ultrasound (IVUS) imaging can visualize vulnerable plaque features including attenuation (AP) and echolucency (ELP). While IVUS-derived vulnerable plaque features associate with microvascular obstruction during percutaneous coronary intervention, the relationship between these plaque features and clinical outcomes has not been established. This analysis aimed to evaluate the association of AP/ELP with cardiovascular events. METHODS AND RESULTS Serial IVUS imaging was reviewed in 1497 patients, followed for 18-24 months, with coronary artery disease from two clinical trials. Attenuated plaque and ELP were identified to measure each characteristics (AP arc, ELP area, and lengths), which permitted calculation of an AP index (API) and ELP volume. Attenuated plaque/ELP progression was defined as patients with any increase of API or ELP volume on serial imaging. The major cardiovascular events (MACEs) were defined as death, myocardial infarction, stroke, and coronary revascularization. AP or ELease email [email protected] Aortic valve-sparing root replacement is an excellent treatment option for patients with intact tricuspid aortic valves. However, the durability in patients with bicuspid aortic valves is still a matter of debate. The aim of this study was to analyse the short- and long-term outcomes in patients with bicuspid aortic valves. METHODS Between July 1993 and October 2015, a total of 582 patients underwent the David I procedure, 50 of whom had a bicuspid aortic valve. In these patients, the mean age was 46 ± 13 years, and 80% (n = 40) were men. Follow-up was complete for 100% of patients and comprised a total of 552 patient-years with a mean follow-up time of 11 ± 5.2 years. RESULTS In addition to the David procedure, cusp plication was performed in 34% (n = 17) of patients and commissure repair in 8% (n = 4) of patients. There were no in-hospital deaths, and 1 patient (2%) had a perioperative stroke. The 1-, 5-, 10- and 20-year survival rates of patients after discharge were 98%, 94%, 88% and 84%, respectively. The rates for freedom from valve-related reoperation at 1, 5, 10 and 20 years after initial surgery were 98%, 88%, 79% and 74%, respectively. Long-term freedom from reoperation was comparable to that of patients with tricuspid aortic valves (P = 0.2). CONCLUSIONS The David procedure has excellent short-term results in patients with a bicuspid aortic valve. In this study, the long-term durability of reimplanted bicuspid aortic valves was comparable to that of tricuspid valves. However, careful patient selection might have contributed to this, and further studies might be needed to definitely clarify this issue. © The Author(s) 2020. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.POEMS syndrome is a rare multisystem disease due to an underlying plasma cell (PC) dyscrasia. The pathophysiology of the disease remains unclear but the role of the monoclonal immunoglobulin (Ig) light chain (LC) is strongly suspected, due to the highly restrictive usage of two λ variable (V) domains (IGLV1-40 and IGLV1-44) and the general improvement of clinical manifestations following PC clone-targeted treatment. However, the diagnostic value of Ig LC sequencing, especially in case of incomplete forms of the disease, remains to be determined. Using a sensitive high-throughput Ig repertoire sequencing on RNA (RACE-RepSeq), we detected a λ LC monoclonal expansion in the bone marrow (BM) of 83% of patients with POEMS syndrome, including some in whom bone marrow tests routinely performed to diagnose plasma cell dyscrasia failed to detect λ+ monoclonal PCs. Selleck OTS964 Twenty-four of the 29 LC clonal sequences found (83%) were derived from the IGLV1-40 and IGLV1-44 germline genes, two from the closely related IGLV1-36 gene, and all were associated with an IGLJ3*02 junction (J) gene, confirming the high restriction of VJ region usage in POEMS syndrome.
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