Familial hypercholesterolaemia is common in individuals who had a myocardial infarction at a young age. As many as one in 200 people could have heterozygous familial hypercholesterolaemia, and up to ...one in 300 000 individuals could be homozygous. The phenotypes of heterozygous and homozygous familial hypercholesterolaemia overlap considerably; the response to treatment is also heterogeneous. In this Review, we aim to define a phenotype for severe familial hypercholesterolaemia and identify people at highest risk for cardiovascular disease, based on the concentration of LDL cholesterol in blood and individuals' responsiveness to conventional lipid-lowering treatment. We assess the importance of molecular characterisation and define the role of other cardiovascular risk factors and advanced subclinical coronary atherosclerosis in risk stratification. Individuals with severe familial hypercholesterolaemia might benefit in particular from early and more aggressive cholesterol-lowering treatment (eg, with PCSK9 inhibitors). In addition to better tailored therapy, more precise characterisation of individuals with severe familial hypercholesterolaemia could improve resource use.
To investigate the success and safety of routine versus advanced inferior vena cava (IVC) filter retrieval techniques.
A retrospective review was performed of patients who underwent IVC filter ...placement and/or a retrieval attempt over a 10-year period. Retrieval technique(s), preretrieval computed tomography, preretrieval venography, and clinical/imaging follow-up for 30 days after retrieval were analyzed. Mean filter dwell time was 134 days (range, 0-2,475 d).
Filter retrieval was attempted 231 times in 217 patients (39% female, 61% male; mean age, 50.7 y), with success rates of 73.2% (169 of 231) and 94.7% (54 of 57) for routine and advanced filter retrieval techniques, respectively. The overall filter retrieval complication rate was 1.7% (four of 231); complications in four patients (with multiple complications in some cases) included IVC dissection, IVC intussusception, IVC thrombus/stenosis, filter fracture with embedded strut, IVC injury with hemorrhage, and vascular injury from complicated venous access. The rate of complications associated with filter retrievals that required advanced technique was significantly higher than seen with routine technique (5.3% vs 0.4%; P < .05). Longer dwell time, more transverse tilt, and presence of an embedded hook were associated with significantly increased rates of failed retrieval via routine technique (P < .05).
IVC filters can be retrieved with a high overall success rate (98.2%) and a low complication rate (1.7%) by using advanced techniques when the routine approach has failed; however, the use of advanced techniques is associated with a significantly higher complication rate.
To describe initial single-center experience with a thrombectomy device in managing right atrial and caval thrombi, tumors, and vegetations.
A retrospective analysis of AngioVac thrombectomy ...performed in 16 patients (mean age 53 y ± 13; 8 men, 8 women) between August 2013 and August 2015 was performed. Indications included right atrial mass/thrombus (6/16; 37.5%) and iliocaval thrombus (10/16; 62.5%). Procedural success was defined as aspiration of > 70% volume of atrial mass/thrombus or restoration of antegrade caval flow.
Procedural success was achieved in 4/6 (67%) right atrial masses/thrombi and 10/10 (100%) caval thrombi. All patients (8/8; 100%) with caval thrombus presenting with swelling/edema had improvement or resolution of symptoms. There were no procedural or periprocedural mortalities; complications included one major (6.3%; intraprocedural pulmonary embolus) and one minor (6.3%; access site hematoma not requiring transfusion) complication. Of 16 patients, 14 (87.5%) survived to discharge at a mean of 10 days ± 8 (range, 1-23 d), and 12 patients (75%) were alive at last known follow-up at a mean of 385 days ± 267 (range, 63-730 d). At a mean of 194 days ± 177 (range, 41-372 d), 4/16 (25%) patients were dead; no death was related to AngioVac thrombectomy. At a mean of 66 days ± 21 (range, 49-90 d) after intervention, 3/14 (21.4%) cases with procedural success had local recurrence of mass/thrombus.
AngioVac thrombectomy can be performed with high procedural success with clinical benefit in patients with right atrial and caval masses/thrombi.
Regional anesthesia and analgesia attenuate or prevent perioperative factors that favor minimal residual disease after removal of the primary carcinoma. Therefore, the authors evaluated prostate ...cancer recurrence in patients who received either general anesthesia with epidural anesthesia/analgesia or general anesthesia with postoperative opioid analgesia.
In a retrospective review of medical records, patients with invasive prostatic carcinoma who underwent open radical prostatectomy between January 1994 and December 2003 and had either general anesthesia-epidural analgesia or general anesthesia-opioid analgesia were evaluated through October 2006. The endpoint was an increase in postoperative prostate-specific antigen.
After adjusting for tumor size, Gleason score, preoperative prostate-specific antigen, margin, and date of surgery, the epidural plus general anesthesia group had an estimated 57% (95% confidence interval, 17-78%) lower risk of recurrence compared with the general anesthesia plus opioids group, with a corresponding hazard ratio of 0.43 (95% confidence interval, 0.22-0.83; P = 0.012) in a multivariable Cox regression model. Gleason score and tumor size (percent of prostate involved) were also independent predictors of recurrence (hazards ratios of 1.19 1.08, 1.52, P = 0.004, and 1.17 1.03, 1.34 for 10% size difference, P = 0.01, respectively). A similar association between epidural use and recurrence was obtained by comparing patients matched on the propensity to receive epidural versus general anesthesia.
Open prostatectomy surgery with general anesthesia, substituting epidural analgesia for postoperative opioids, was associated with substantially less risk of biochemical cancer recurrence. Prospective randomized trials to evaluate this association seem warranted.
Five consecutive cases in which the AngioVac aspiration cannula was used for the management of pulmonary embolism (PE) were retrospectively reviewed. Four cases (80%) presented with massive PE, and ...two (40%) were technically successful (reduction in Miller index ≥ 5). Four patients (80%) died at a mean of 7.3 days after the procedure, including one death related to right ventricular free wall perforation. Although the AngioVac aspiration cannula has shown clinical promise in a variety of clinical applications, early experience in the pulmonary arteries has shown limited success, and further study and careful patient selection are required.
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•Community Driven Data Representation for structural biology data.•Provides common data representation and tools that accelerates scientific discovery.•Provides data and software ...infrastructure for the Protein Data Bank (PDB) Core Archive.•Provides essential data infrastructure supporting FAIR data delivery.
PDBx/mmCIF, Protein Data Bank Exchange (PDBx) macromolecular Crystallographic Information Framework (mmCIF), has become the data standard for structural biology. With its early roots in the domain of small-molecule crystallography, PDBx/mmCIF provides an extensible data representation that is used for deposition, archiving, remediation, and public dissemination of experimentally determined three-dimensional (3D) structures of biological macromolecules by the Worldwide Protein Data Bank (wwPDB, wwpdb.org). Extensions of PDBx/mmCIF are similarly used for computed structure models by ModelArchive (modelarchive.org), integrative/hybrid structures by PDB-Dev (pdb-dev.wwpdb.org), small angle scattering data by Small Angle Scattering Biological Data Bank SASBDB (sasbdb.org), and for models computed generated with the AlphaFold 2.0 deep learning software suite (alphafold.ebi.ac.uk). Community-driven development of PDBx/mmCIF spans three decades, involving contributions from researchers, software and methods developers in structural sciences, data repository providers, scientific publishers, and professional societies. Having a semantically rich and extensible data framework for representing a wide range of structural biology experimental and computational results, combined with expertly curated 3D biostructure data sets in public repositories, accelerates the pace of scientific discovery. Herein, we describe the architecture of the PDBx/mmCIF data standard, tools used to maintain representations of the data standard, governance, and processes by which data content standards are extended, plus community tools/software libraries available for processing and checking the integrity of PDBx/mmCIF data. Use cases exemplify how the members of the Worldwide Protein Data Bank have used PDBx/mmCIF as the foundation for its pipeline for delivering Findable, Accessible, Interoperable, and Reusable (FAIR) data to many millions of users worldwide.
Acute pulmonary embolism is the third leading cause of cardiovascular death, with most pulmonary embolism-related mortality associated with acute right ventricular failure. Although there has ...recently been increased clinical attention to acute pulmonary embolism with the adoption of multidisciplinary pulmonary embolism response teams, mortality of patients with pulmonary embolism who present with hemodynamic compromise remains high when current guideline-directed therapy is followed. Because historical data and practice patterns affect current consensus treatment recommendations, surgical embolectomy has largely been relegated to patients who have contraindications to other treatments or when other treatment modalities fail. Despite a selection bias toward patients with greater illness, a growing body of literature describes the safety and efficacy of the surgical management of acute pulmonary embolism, especially in the hemodynamically compromised population. The purpose of this document is to describe modern techniques, strategies, and outcomes of surgical embolectomy and venoarterial extracorporeal membrane oxygenation and to suggest strategies to better understand the role of surgery in the management of pulmonary embolisms.
Immediate intervention is needed for aortoesophageal fistulas (AEF), a rare but highly lethal cause of massive gastrointestinal hemorrhage. Emergent thoracic endovascular aortic repair (TEVAR) is ...considered first-line treatment for massive bleeding from AEFs. We describe an unusual and challenging case of TEVAR coverage of an AEF involving the central aortic arch immediately followed by in vivo endograft fenestration to regain arch vessel perfusion. In vivo fenestration, currently a procedure for emergency or investigational purposes only, was shown to be life saving in our case. The main complications associated with the procedure included stroke and infection, requiring esophagectomy and cervical diversion as well as ongoing antibiotic treatment.
Purpose To assess the technical feasibility of the use of ferumoxytol-enhanced (FE) magnetic resonance (MR) angiography for vascular mapping before transcatheter aortic valve replacement in patients ...with renal impairment. Materials and Methods This was an institutional review board-approved and HIPAA-compliant study. FE MR angiography was performed at 3.0 T or 1.5 T. Unenhanced computed tomographic (CT) images were used to overlay vascular calcification on FE MR angiographic images as composite fused three-dimensional data. Image quality of the subclavian and aortoiliofemoral arterial tree and confidence in the assessment of calcification were evaluated by using a four-point scale (4 = excellent vascular definition or strong confidence). Signal intensity nonuniformity as reflected by the heterogeneity index (ratio between the mean standard deviation of luminal signal intensity and the mean luminal signal intensity), signal-to-noise ratio, and consistency of luminal diameter measurements were quantified. Findings at FE MR angiography were compared with pelvic angiograms. Results Twenty-six patients underwent FE MR angiography without adverse events. A total of 286 named vascular segments were scored. The image quality score was 4 for 99% (283 of 286) of the segments (κ = 0.9). There was moderate to strong confidence in the ability to assess vascular calcific morphology in all studies with complementary unenhanced CT. The steady-state luminal heterogeneity index was low, and signal-to-noise ratio was high. Interobserver luminal measurements were reliable (intraclass correlation coefficient, 0.98; 95% confidence interval: 0.98, 0.99). FE MR angiographic findings were consistent with correlative pelvic angiograms in all 16 patients for whom the latter were available. Conclusion In patients with renal impairment undergoing transcatheter aortic valve replacement, FE MR angiography is technically feasible and offers reliable vascular mapping without exposure to iodine- or gadolinium-based contrast agents. Thus, the total cumulative dose of iodine-based contrast material is minimized and the risk of acute nephropathy is reduced.
RSNA, 2017 Online supplemental material is available for this article.
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