Lehigh Valley Health Network

Objective Metabolic syndrome is a cluster of cardiovascular risk factors (central obesity, hypertension, dyslipidemia, and insulin resistance) that affects between 12.5% and 31.4% of adults worldwide. It correlates with increased risks of cardiovascular disease, diabetes, cancer, and overall mortality in a dose-dependent fashion. This review aims to provide primary care clinicians an updated review of the evidence on metabolic syndrome, with a focus on treatment. Design Scoping evidence review. Eligibility Criteria English-language studies of evidence Level I or II that focused on defining, diagnosing, and treating metabolic syndrome or its components. Information Sources PubMed and Cochrane Database of Systematic Reviews. Results Though evidence is still lacking for improved outcomes with treating the syndrome per se, addressing its individual components reduces risks. Lifestyle changes like weight loss and increased physical activity are first line. Surgical options assist with weight loss for certain patients. Pharmacotherapies like glucagon-like peptide-1 receptor agonists, sodium-glucose cotransporter-2 inhibitors, statins, and antihypertensives also have efficacy. Conclusions Metabolic syndrome is an independent risk factor for many poor health outcomes. Its individual components should be treated with medication and behavioral changes to reduce cardiovascular risk and prevent diabetes and its complications. More research is needed on how to treat the syndrome itself. A diagnosis of metabolic syndrome may be useful for motivating patients toward lifestyle changes, though more research is needed on how to treat the syndrome versus its components.

The Global Burden of Disease (GBD) study aims to characterize the worldwide prevalence and morbidity of major diseases, while PatientsLikeMe (PLM) is an online community providing patient-generated insights into lived experiences; for dermatologic conditions, quantitative comparisons of GBD and PLM data revealed expected demographic differences but also notable correlations, highlighting their potential as complementary data sources elucidating unmet patient needs and priorities.

BACKGROUND “Dangling choroid” is a prenatal sonographic marker of ventriculomegaly that measures the angle of choroid plexus (ChP) displacement in the lateral ventricle. To the authors’ knowledge, postnatal sequelae related to this pathology, besides hydrocephalus, have never been reported. OBSERVATIONS A female fetus was diagnosed with bilateral ventriculomegaly. Postnatally, the patient was diagnosed with hydrocephalus and macrocephaly secondary to aqueductal stenosis and underwent endoscopic third ventriculostomy with ChP cauterization. Intraoperatively, the septum pellucidum was incomplete, and the right-sided ChP glomus was contralaterally displaced and entangled with the left, with evidence of ischemic torsion and hemorrhage. LESSONS In this case of an ischemic ChP secondary to transventricular displacement of the glomus, at least two biomechanical events are relevant. First, the choroid fissure and velum interpositum can be thinned in the setting of ventriculomegaly. Second, stretching and perforation of the septum pellucidum can occur. Both changes can increase the mobility of a dangling choroid, occasionally leading to entanglement of the vascular pedicles. Preoperative recognition of this complication can help optimize surgical planning, e.g., using flexible endoscopy to facilitate complete ChP cauterization and changing the surgical approach if the ChP has been displaced. https://thejns.org/doi/10.3171/CASE24505

In this case report, we discuss the critical interdependence of structure and function in demonstrating systolic anterior motion (SAM) of the mitral valve after repeat heart transplantation, where residual apical tissue of the explanted heart remained in place. The resulting conformational changes led to anterior displacement of the mitral valve and persistent SAM.

Background Posterior vault distraction osteogenesis (PVDO) is an effective treatment for multisuture and syndromic craniosynostosis. It remains unclear how well the calvarium ossifies after PVDO, especially in older patients when they have plateaued in their cranial growth phase. The purpose of this study is to report outcomes associated with PVDO across a wide range of ages at our institution. Methods Operative details, distraction parameters, complications, and details of cranioplasty, if applicable, were obtained for all patients who underwent PVDO between March 2016 and July 2022. The surface areas of distraction sites and residual cranial defects were measured from computed tomography scans after a consolidation phase, and the percentages of the distraction regions with ossification were subsequently calculated. Results Fifteen patients underwent PVDO at a mean age of 5.1 years (0.5–18.7 y). The mean percentage of ossification among all patients was 80.4% (59.1%–95.3%). The degree of ossification was not significantly associated with age at the time of surgery at the univariate level (Pearson-r=−0.15, P =0.573). Three patients (20.0%) required titanium mesh cranioplasty for definitive reconstruction, which was significantly more likely in patients who were >4 years old at the time of PVDO ( P =0.04). Conclusions Complete ossification of the distracted area of the cranium is unlikely after PVDO. Ossification appears less robust with increasing age, although not statistically significant in our study population, likely due to the limited sample size. Future studies should assess the role of age, latency period, and distraction rate in minimizing residual defects.

Objectives The utility of thrombolysis and/or thrombectomy in patients with mild stroke and large vessel occlusion (LVO) remains inconclusive. This retrospective study compared short-term and long-term outcomes in patients treated with best medical therapy (BMT group) versus with intravenous thrombolytics and/or endovascular thrombectomy (intervention group). Methods Patients with acute ischemic stroke (AIS), LVO, and National Institutes of Health Stroke Score (NIHSS) ≤5 were included. Data collected includes demographics; hospital length of stay (LOS); modified Rankin scale (mRS) at admission, discharge, and follow-up; hemorrhagic conversion; and disposition. Bivariate analyses were conducted to compare outcomes between groups. Results Of the 29 patients, 15 were treated with BMT and 14 underwent intervention. Median hospital LOS was slightly longer in the intervention group (6.5 [IQR=4 to 12] vs. 5 [IQR=3 to 5] d, P =0.070). Everyone in the BMT group had a favorable outcome (mRS 0 to 2) at discharge and follow-up, unlike the intervention group’s rate at discharge (100% vs. 71.4%, P =0.042), This gap was closed at follow-up between BMT group (median=33 d, IQR=28 to 48) and intervention group (median=44.5 days, IQR=30 to 48) (100% vs. 85.7%, P =0.224). Hemorrhagic conversion rates were similar between groups. More patients in the BMT group were discharged home (80% vs. 42.9%, P =0.079). Conclusions Patients with AIS, low NIHSS, and LVO, who received thrombolytics and/or thrombectomy had longer hospital LOS, higher mRS at discharge and follow-up, and were less likely to be discharged home, though these trends were not statistically significant. Our study is limited by a small sample size and these findings should be further explored in larger studies.

Nanotechnology is an actively developing science and has its branches in many fields of science and technology. The delivery of drugs to the central nervous system (CNS) for the treatment of motor disorders and their passage through the blood–brain barrier (BBB) represents a significant challenge. There are active developments in the field of movement disorders, but one of the most significant challenges present for the treatment of movement disorders is to develop a unique chemical compound feasible to cross through the BBB. Multiple factors contribute to successful delivery of drugs to the CNS. The technologies that are being developed are aimed at the development of carriers that allow genetic material to pass through the BBB, enter the tissue, followed by introduction into the cytoplasm and nucleus to increase the expression of genes necessary for protein biosynthesis or suppress the expression of defective genes. Recent technological advancements in understanding movement disorders provided a great benefit in establishing novel nanomaterials that demonstrated significant efficacy in animal studies. Currently, nanotechnology provides a set of particles, functionally capable of enhancing the regeneration capacity of neurons through limiting oxidative stress, chemically modified drug transporters that are potential candidates to be implemented and studied in clinical trials, as they increase permeability of BBB by 4- to 10-fold. Nanovectors and gene therapy have a significant capacity to turn “off” undesired genes and turn “on” regeneration genes which is beneficial to limit progression of movement disorders.

Nanotechnology is rapidly advancing in fields such as healthcare. The idea of nanotoxicology arises from the fact that the small size of nanoparticles is the main cause for their adverse biological effects. Nanoparticles can cross the blood–brain barrier accidently during treatment or by inhalation from the environment, in addition to the intentional application for neurological disorders therapy. Consequently, nanoneurotoxicity is a source of concern despite the field’s significant success. Screening for basal cytotoxicity is the first toxicological assessment in drug development, and it can also be used to measure nanotoxicity. Certain cell lines can be used for specific neurotoxicity screening. In vivo studies on pharmacokinetics (PK) and pharmacodynamics (PD) are a reliable source of information during drug development and toxicological studies. Cytotoxicity screening methods can assess a large number of samples, but they lack the complexity of in vivo systems. Preclinical in vivo testing is more time consuming and more expensive than in vitro testing. A close consensus of in vitro and in vivo assessments is a prerequisite for efficient pharmaceutical development. In vivo models can be used to validate in vitro models and to verify outcomes of in vitro studies.

Background: Launched in 2023, the American College of Cardiology Medical Student Member Community (ACC MSMC) began recognizing Cardiology Interest Groups (CIGs) at medical schools worldwide. This initiative fosters a robust network among ACC medical student members and celebrates their contributions within their institutions and broader communities. We surveyed medical students from 50 recognized CIGs to evaluate the influence of this network on their members' career trajectories. Methods: An anonymous web-based survey was sent to 50 student leaders of CIGs recognized by the ACC from 20 countries and was distributed to their members. The objective was to evaluate the impact of their involvement in these groups on their clinical knowledge, research activities and publications, professional advancement, and the promotion of diversity and equity. Results: Of 215 respondents (71% ACC members), 49.3% identified as female, with significant representation from Asia (35.7%), and the USA (17.6%). The majority joined CIGs to explore cardiology as a career path (61%), build professional networks (60%), and participate in specialized workshops (59.5%). Most rated their access to CIG resources as excellent (73.8%), supporting ACC's mission to enhance diversity and inclusion. Regarding educational outcomes, 48.6% rated their knowledge acquisition in cardiology as excellent, and 41% noted excellent preparation for a cardiology career. Additionally, 26.4% of students published research papers post-CIG involvement, with 39% attending ACC or AHA conferences. Survey feedback also highlighted strong networking (38.6% excellent) and mentoring opportunities (36.2% excellent) provided by CIGs. Conclusions: CIGs significantly enrich medical students' professional development by offering extensive networking, education, research, and mentorship opportunities. They play a crucial role in nurturing a global interest in cardiology, thereby enhancing diversity within the field. This model serves as a valuable blueprint for other scientific societies considering the establishment of student interest groups.

Idiopathic ventricular fibrillation (VF) arrest can be a challenging diagnosis, especially when the etiology is unclear. We present a case of a young male who was found to have VF originating from the right ventricular (RV) moderator band (MB). A 19-year-old male with a history of idiopathic VF arrest with ICD placement presented with a complaint of sudden dizziness and lightheadedness. Upon device interrogation, he was found to have 17 seconds of fine-VF terminated by one out-of-hospital ICD shock. He reported non-adherence to daily nadolol. He was also noted to have a high burden of unifocal premature ventricular contractions (PVC) on telemetry. On EKG the morphology of PVCs was of a LBBB with a superior axis, suggesting origin from the MB. The patient then underwent an electrophysiological (EP) study, where it was felt that earliest site of activation was identified at the mid-inferior RV along a muscle band extending from mid inferior free wall, superiorly to the interventricular septum. He then had successful radiofrequency ablation at this site, with no post-ablation PVCs or ICD discharge, and was subsequently restarted on his home nadolol. Idiopathic VF is defined as VF in the absence of clinical cause. Our patient’s PVCs and fine-VF were highly suspicious for RV MB origination. We proceeded with EP mapping and identified the earliest site of PVC activation at the MB. Pace mapping revealed 95% matching at this site. Following ablation, no further PVCs were induced even with isoproterenol. Though not fully understood, the MB is becoming increasingly noted as a source for Purkinje-mediated ventricular arrhythmias. Possible mechanisms of arrhythmogenesis include fibrosis, stretching of fibers, and ion channel variations. Arrythmias from the MB are potentially responsive to catheter ablation. Early recognition of typical PVC morphology from the RV MB as the source of VF can provide an opportunity for timely intervention. Younger patients, especially, can be treated with catheter ablation to reduce their risk of sudden cardiac death.

Background: Cardiac amyloidosis is an underdiagnosed cause of heart failure. The mean survival rate without treatment is low, signifying the importance of early diagnosis. The prognosis depends on the time of diagnosis and the severity of the disease before recognition and treatment. We performed a systematic review to evaluate patient characteristics and mortality in individuals with cardiac amyloidosis. Methods: PubMed, Scopus and Cochrane were systematically searched from the database inception to May 2024 to evaluate for mortality outcomes in patients with cardiac amyloidosis. The statistical analysis was performed using R-Studio software, and proportions with 95% confidence intervals (CI) were calculated using a random-effects model. The Kaplan Meier survival plots was also plotted for eligible studies. Results: Eighteen studies involving 7,268 patients were included. Patients with cardiac amyloidosis mostly presented with dyspnea and peripheral edema. Among the electrocardiographic abnormalities, atrial fibrillation was observed in 31.1% of patients, whereas atrioventricular block was observed in 6.2% of patients. Furthermore, 37.2% of patients experienced heart failure, 36.3% experienced reduced ejection fraction, and 3.2% of patients experienced cardiogenic shock. Majority of the patients were found to have an increase in left ventricular wall thickness (77.8%). The mortality associated with cardiac amyloidosis varied from 0% to 100%, with a summary estimate rate of 54% (95% CI: 31% to 77%). The median survival rate for 50% patients was around 2 weeks, which declined significantly over the next one week resulting into the cumulative survival of over only 21 days. Conclusion: Our results highlight the importance of considering cardiac amyloidosis in the differential diagnosis of all patients with heart failure or non-ischemic cardiomyopathy, particularly when there is an increase in ventricular wall thickness. Our review revealed a high mortality rate associated with cardiac amyloidosis. Early diagnosis and better therapeutic modalities have the potential to improve patient outcomes and reduce mortality rates.

In this chapter, we explore the role of mixed reality (MxR), a synergy of virtual reality (VR) and augmented reality (AR), in neurosurgery. MxR marks a significant advancement in surgical planning and execution, enhancing precision, safety, and outcomes. We trace the evolution of neurosurgical techniques, positioning MxR as the latest innovation in a series of technological advancements aimed at improving surgical accuracy and patient safety. The chapter details VR’s role in providing immersive preoperative environments for detailed surgical rehearsal, utilizing 3D reconstructions of patient-specific neuroanatomy. AR complements this by overlaying critical digital information onto the surgeon’s real-time view, thus reducing cognitive load and enhancing focus during critical surgical phases. We specifically highlight MxR’s application in brain tumor and skull base surgery, demonstrating its capacity to facilitate meticulous planning and navigation through complex anatomical structures. The chapter includes case studies illustrating MxR’s effectiveness in cerebrovascular surgeries, such as arteriovenous malformations (AVM) and aneurysm treatments, and discusses its potential in neurosurgical training and custom implant design. Concluding with a discussion of the challenges in MxR adoption and future perspectives, the chapter underscores MxR’s potential to revolutionize neurosurgical interventions, making them safer, more efficient, and tailored to individual patient needs.

Introduction: Coronary in-stent restenosis (ISR) remains challenging despite stent technology advancements. Paclitaxel-coated balloons (PCB) offer a promising non-implant approach to deliver antiproliferative agents directly to the vessel wall. This meta-analysis aims to evaluate the comparative effectiveness of PCB versus uncoated balloons (UCB) in managing coronary ISR to address emerging safety concerns. Methodology: Medline, Scopus and Embase were searched until April 2024 for randomized controlled trials (RCTs) comparing PCB versus UCB in patients undergoing coronary ISR. Primary outcomes were in-segment late lumen loss (LLL), binary restenosis, target lesion revascularization (TLR), major adverse cardiac events (MACE), and mortality. Secondary outcomes included in-stent LLL, in-stent binary restenosis, myocardial infarction (MI), cardiac death, target vessel MI, and target vessel revascularization (TVR). Results: This meta-analysis includes seven RCTs with 1,408 patients (PCB = 864, UCB = 544). PCB significantly reduced in-segment LLL (MD= -0.50, 95% CI [-0.67, -0.33]; P < 0.00001), in-segment binary restenosis (RR= 0.25, 95% CI [0.14, 0.45]; P < 0.00001), target lesion revascularization (RR= 0.43, 95% CI [0.32, 0.58]; P < 0.00001), mortality (RR=0.56, 95% CI [0.39, 0.80]; P=0.001), and MACE (RR= 0.36, 95% CI [0.25, 0.51]; P < 0.00001). PCB also significantly decreased in-stent LLL (MD= -0.52, 95% CI [-0.72, -0.32]; P < 0.00001), in-stent binary restenosis (RR = 0.19, 95% CI [0.11, 0.35]; P < 0.00001), and TVR (RR = 0.45, 95% CI [0.29, 0.70]; P = 0.0003). PCB showed a short-term reduction in MACE and target lesion revascularization at 6 months, 1 year and 3 years of followup. No difference was observed in MI, cardiac death, or target vessel MI. Conclusion: In patients with coronary ISR, PCB are associated with reduced in-segment LLL, in-segment binary restenosis events, target lesion revascularization, mortality, MACE, in-stent LLL, in-stent binary restenosis and target vessel revascularization.

Despite multiple recent guidelines recommending the diagnosis and treatment of anemia before elective cardiac surgery, few institutions have formal programs or methods in place to accomplish this. A major limitation is the perceived financial shortfall and the leadership buy-in required to undertake such an initiative. The purpose of this advisory from the Society of Cardiovascular Anesthesiologists (SCA) Clinical Practice Improvement Committee with endorsement by the Society for the Advancement of Patient Blood Management (SABM) is to provide an overview of preoperative anemia management programs with an emphasis on the associated financial implications. This advisory reviews the evidence for preoperative anemia management programs in both cardiac and noncardiac surgery, discusses options for managing preoperative anemia, provides novel financial modeling regarding the implementation of preoperative anemia management programs, and describes implementation challenges, potential solutions, and opportunities for improvement.

Cameron lesions are a unique and relatively rare cause of upper gastrointestinal bleeding that appears in the mucosa of the gastric body in the presence of a large hiatal hernia. These lesions can be a source of occult bleeding and subsequent chronic iron deficiency anemia (IDA) but may often be missed on initial endoscopy, requiring repeat studies to diagnose. Prompt treatment for Cameron lesions is necessary to avoid the high mortality rate associated with them. We describe the case of a 36-year-old male patient who presented to the emergency department (ED) with shortness of breath (SOB) and chronic IDA of an unknown cause in the presence of a large hiatal hernia. The endoscopy showed multiple linear erosions in the stomach consistent with Cameron lesions. The patient was discharged but returned to the ED two more times before ultimately having his hiatal hernia surgically repaired. Though rare, Cameron lesions may be considered in the differential for patients presenting with recurrent SOB or with chest or abdominal pain, combined with refractory anemia of an unknown cause.