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A growing number of people identify as transgender and gender non-binary in the USA and worldwide. Concomitantly, an increasing number of patients are receiving gender-affirming hormone therapy (GAHT) to achieve gender congruence. GAHT has far-ranging effects on clinical and subclinical markers of cardiovascular risk. Transgender patients also appear to be at higher risk for cardiovascular diseases compared to their cisgender peers and the impact of gender-affirming therapy on cardiovascular health is unclear. Studies on the effect of GAHT on cardiovascular outcomes are confounded by differences in GAHT regimens and methodological challenges in a diverse and historically hard-to-reach population. Current cardiovascular guidelines do not incorporate gender identity and hormone status into risk stratification and clinical decision-making. In this review, we provide an overview on the cardiometabolic impact and clinical considerations of GAHT for cardiovascular risk in transgender patients.

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Background COVID-19 has been the worst pandemic of this century, resulting in economic, social, and educational disruptions. Residency training is no exception, with training restrictions delaying the progression and graduation of residents. We sought to utilize simulation modelling to predict the impact on future cohorts in the event of repeated and prolonged movement restrictions due to COVID-19 and future pandemics of a similar nature. Method A Delphi study was conducted to determine key Accreditation Council for Graduate Medical Education-International (ACGME-I) training variables affected by COVID-19. Quantitative resident datasets on these variables were collated and analysed from 2018 to 2021. Using the Vensim® software (Ventana Systems, Inc., Harvard, MA), historical resident data and pandemic progression delays were used to create a novel simulation model to predict future progression delay. Various durations of delay were also programmed into the software to simulate restrictions of varying severity that would impact resident progression. Results Using the model with scenarios simulating varying pandemic length, we found that the estimated average delay for residents in each accredited year ranged from an increase of one month for year 2 residents to more than three months for year 4 residents. Movement restrictions lasting a year would require up to six years before the program returned to a pre-pandemic equilibrium. Conclusion Systems dynamic modelling can be used to predict delays in residency training programs during a pandemic. The impact on the workforce can thus be projected, allowing residency programs to institute mitigating measures to avoid progression delay.

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INTRODUCTION: This study aims to find out the optimal timing for herniotomy for premature infants with inguinal hernia (IH): early during hospitalisation or delayed after hospital discharge. METHOD: A retrospective cohort study was conducted on premature infants diagnosed with IH during their initial hospitalization between 2015 and 2020. Demographic data and clinical outcomes were compared between infants undergoing herniotomy before discharge ("early") and those who were discharged without herniotomy ("delayed"). Student's t-test or Mann-Whitney U test and Fisher's exact test were used for statistical analysis. RESULTS: Of 219 premature infants, 189 (86.3%) underwent early herniotomy, while 30 were discharged with unoperated IH. In the delayed group, 15 (50%) underwent planned delayed herniotomy, and the remaining 15 experienced spontaneous resolution (absence of inguinal bulge over at least 1-year follow-up). The gestational age and birth weight of both groups were similar. At surgery, the delayed group median (interquartile range) was significantly older (42.1[38-49] vs 37.7 [36-40] weeks, p < 0.001) and heavier (3.27 [2.21-4.60] vs 2.22 [2.00-2.70] kg, p < 0.001). Two infants (1%) in the early group presented with incarcerated IH requiring urgent operation. In the delayed group, no infant developed incarcerated IH while awaiting elective operation (time from diagnosis to operation 44 [21-85] days). There was no statistically significant difference in respiratory and surgical complications between the two groups, although the delayed group had lesser surgical complications (0% vs 9.5%). CONCLUSION: Deferring herniotomy after discharge for premature infants is safe with close monitoring and associated with a chance of spontaneous resolution. LEVEL OF EVIDENCE: Level III, treatment study.

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BACKGROUND: Three-dimensional (3D) transesophageal echocardiogram (TEE) is the gold standard for the diagnosis of degenerative mitral regurgitation (dMR) and preoperative planning for transcatheter mitral valve repair (TMVr). TEE is an invasive modality requiring anesthesia and esophageal intubation. The severe acute respiratory syndrome coronavirus 2 pandemic has limited the number of elective invasive procedures. Multi-detector computed tomographic angiography (MDCT) provides high-resolution images and 3D reconstructions to assess complex mitral anatomy. We hypothesized that MDCT would reveal similar information to TEE relevant to TMVr, thus deferring the need for a preoperative TEE in certain situations like during a pandemic. METHODS: We retrospectively analyzed data on patients who underwent or were evaluated for TMVr for dMR with preoperative MDCT and TEE between 2017 and 2019. Two TEE and 2 MDCT readers, blinded to patient outcome, analyzed: leaflet pathology (flail, degenerative, mixed), leaflet location, mitral valve area (MVA), flail width/gap, anterior-posterior (AP) and commissural diameters, posterior leaflet length, leaflet thickness, presence of mitral valve cleft and degree of mitral annular calcification (MAC). RESULTS: A total of 22 (out of 87) patients had preoperative MDCT. MDCT correctly identified the leaflet pathology in 77% (17/22), flail leaflet in 91% (10/11), MAC degree in 91% (10/11) and the dysfunctional leaflet location in 95% (21/22) of patients. There were no differences in the measurements for MVA, flail width, commissural or AP diameter, posterior leaflet length, and leaflet thickness. MDCT overestimated the measurements of flail gap. CONCLUSIONS: For preoperative TMVr planning, MDCT provided similar measurements to TEE in our study.

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Background: Severe tricuspid regurgitation (TR) may persist after a mitral transcatheter edge-to-edge repair (M-TEER) and is associated with worsened clinical outcomes and survival. It is unclear which patients with concomitant mitral regurgitation (MR) and TR will have TR reduction after M-TEER. The aim of this study was to identify the predictors of residual TR after transcatheter edge-to-edge repair (TEER). Methods: Data were collected from the Northwell TEER registry, a prospectively maintained mandatory database including 4 high-volume transcatheter aortic valve replacement/TEER centers. Transthoracic echocardiograms, both pre-TEER and post-TEER, were evaluated. Univariate and multivariate logistic regression analyses were performed to identify predictors of severe TR after M-TEER. Significant TR reduction was defined as a reduction in TR grade by at least 1+ with moderate (2+) or less TR at 1 month. Results: Of the 479 patients who underwent M-TEER, 107 patients with concomitant severe MR/TR were included. Successful MR reduction occurred in 89 patients (84%) and a significant TR reduction in 45 (42%). On the univariate analysis, the only predictors of severe residual TR were right atrial area and unsuccessful M-TEER. On the multivariate logistic regression model, the only predictor variable for patients with a reduction in TR was MR reduction of ≥3+ with M-TEER. Conclusions: In patients with concomitant severe MR and TR, TR reduction after isolated M-TEER occurs in only ∼40% of patients. MR grade reduction ≥3+ was the only independent predictor for TR reduction. Other clinical and echocardiographic variables (including pulmonary hypertension, right ventricular function, tricuspid annular dilation, atrial fibrillation, and presence of a cardiac implantable electrical device) were not associated with residual TR. Inability to predict TR reduction after M-TEER highlights the importance of establishing transcatheter tricuspid valve therapies and should factor in heart-team discussions.

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BACKGROUND: Our study aimed to compare the clinical outcomes and cost-efficiency of antibiotic management versus laparoscopic appendectomy for acute uncomplicated appendicitis (AUA) in children during the COVID-19 pandemic when resources were limited and transmission risks uncertain. METHOD: In this prospective comparative cohort study, we analyzed the data of 139 children diagnosed with AUA meeting the following inclusion criteria: 5-18 years of age, symptoms duration of ≤ 48 h, appendix diameter ≤ 11 mm and no appendicolith. Treatment outcomes between non-operative management group (78/139) and upfront laparoscopic appendectomy group (61/139) were compared. Antibiotic regimes were intravenous ceftriaxone/metronidazole or amoxicillin/clavulanic acid for 48 h, followed by oral antibiotics to complete total 10-days course. RESULTS: 8/78 (10.3%) children had early failure (within 48 h) requiring appendectomy. 17/70 (24.3%) patients experienced late recurrence within mean follow-up time of 16.2 ± 4.7 months. There were no statistical differences in peri-operative complications, negative appendicectomy rate, and incidence of perforation and hospitalization duration between antibiotic and surgical treatment groups. Cost per patient in upfront surgical group was significantly higher ($6208.5 ± 5284.0) than antibiotic group ($3588.6 ± 3829.8; p = 0.001). CONCLUSION: Despite 24.3% risk of recurrence of appendicitis in 16.2 ± 4.7 months, antibiotic therapy for AUA appears to be a safe and cost-effective alternative to upfront appendectomy.

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Purpose: This study aims to compare the outcomes of extravesical (EVUR) and intravesical (IVUR) ureteric reimplantation for primary vesicoureteral reflux (VUR) via systematic review and meta-analysis. Methods: Literature review from Medline, Embase, and Cochrane since inception to March 2022 was performed. Meta-analysis was conducted on eligible randomized controlled trials (RCT) and observational cohort studies (OCS) comparing outcomes between EVUR and IVUR. Results: Twelve studies were included, comprising 577 patients (778 ureters) operated by EVUR and 395 patients (635 ureters) by IVUR. Pre-operative VUR grade, postoperative VUR persistence and hydronephrosis was not statistically significant. EVUR had shorter operative time [mean differences (MD) -22.91 min; 95% confidence interval (CI), -44.53 to -1.30, P = 0.04] and hospital stay (MD -2.09 days; 95% CI, -2.82 to -1.36, P < 0.00001) compared to IVUR. Bilateral EVUR had higher risk of postoperative acute urinary retention (ARU) (8.1%) compared to bilateral IVUR (1.7%) (OR = 4.40; 95% CI, 1.33-14.58, P = 0.02). No patient undergoing unilateral EVUR or IVUR experienced ARU. Conclusion: Both EVUR and IVUR are equally effective in correcting primary VUR. Operative time and hospital stay are shorter after EVUR compared to IVUR. However, bilateral EVUR is associated with higher risk of postoperative ARU.

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PURPOSE OF REVIEW: Transgender individuals represent a growing part of our population with current trends indicating that clinicians will be treating more transgender patients in both the inpatient and outpatient setting. Current cardiovascular guidelines lack recommendations for transgender care secondary to limited data in this population. As we await future guideline recommendations, we provide a comprehensive review of the literature and practical management strategies related to transgender cardiovascular health. RECENT FINDINGS: Transgender individuals are at higher risk for some cardiovascular diseases compared to their cisgender counterparts. Gender-affirming hormone therapy, concomitant health conditions, lifestyle habits, access to services, and quality of care all contribute to this finding. While it is likely both safe and appropriate to apply current CVD guidelines to the care of transgender men and women, clinicians should consider additional factors in risk assessment and address unique aspects of care at every visit.

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INTRODUCTION: Relatively little is known about faculty development (FD) activities that help participants achieve sustainable behavioral change. This qualitative study evaluated the medium- to long-term impact of a FD workshop informed by transformative learning (TL) theory. It aimed to discover which aspects of FD prompted healthcare professionals (HPs) to adopt effective teaching and learning practices. METHODS: Seventeen participants were interviewed between January and July 2020, 7 to 30 months after the workshop. Purposeful sampling strategies were used to collect data and analysis was performed using reflexive thematic analysis. RESULTS: Four themes were identified: perspectival shift in educational practice, re-affirmation of current practices, becoming an educator, and valuing FD that accommodates HPs' multiple communities of practice (CoPs). Workshop activities foregrounding critical discourse and reflection helped participants gain new knowledge and deeper understanding of education. TL was likely when participants already identified as an educator in addition to their HP identity. Additionally, a workplace CoP determined the type and level of support affecting HPs' development as educators. DISCUSSION: Aspects of FD that prompted HPs to adopt effective teaching and learning practices included initiatives that catalyzed critical discourse and reflection. Readiness for TL is promoted when HPs have a strong educator identity because of workplace educator CoPs. Future research could explore effecting sustainable post-workshop behavioral change in HPs through the strengthening of workplace educator CoPs. To do this, institutions could send co-located HPs from different disciplines to the same FD program.

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Background: The International Takotsubo Registry (InterTAK) score was developed to assess the probability of takotsubo syndrome (TTS) and to distinguish it from acute coronary syndrome, which has a similar clinical presentation. A European Society of Cardiology (ESC) consensus statement suggests invasive coronary angiography may be deferred in patients with non-ST segment elevation myocardial infarction (NSTEMI) and high probability of TTS. We aimed to determine the predictive performance of the InterTAK score in a real-world population of NSTEMIs. Materials and Methods: The InterTAK score was retrospectively calculated for consecutive female patients with NSTEMI who underwent invasive coronary angiography at two academic medical centers in New York City from March 2016 to August 2018. Results: Among 375 women with NSTEMI, 15 (4%) had InterTAK score >70, indicating high probability of TTS. Ten (67%) met ESC TTS criteria for a noninvasive strategy, among whom seven had TTS, two had spontaneous coronary artery dissection (SCAD), and one had coronary artery disease requiring revascularization. A total of 48 women (13% of cohort) had a final diagnosis of TTS. The InterTAK score had an area under the receiver operating characteristics curve (AUC) of 0.82 (95% confidence interval, 0.75-0.88) for TTS, with 21% sensitivity and 98% specificity at score >70. Conclusions: In this real-world sample of consecutive female NSTEMI patients, the InterTAK score was predictive of TTS but a high score was rare, and use of the score to obviate invasive angiography could have resulted in delay of revascularization and missed diagnosis of SCAD for a small number of patients. The InterTAK score should be used in conjunction with other clinical variables for angiographic referral.

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Inclusion and equal representation of women in cardiovascular imaging trials are essential to provide insight into the factors impacting women's heart health and outcomes. Despite heart disease being the leading cause of mortality for women in the United States, women have been underrepresented in cardiovascular clinical trials, including imaging trials. Research demonstrates that women have key sex-specific differences in the pathophysiology of cardiovascular disease, the evolution of disease state, and disease manifestation (Solimene, 2010; Nevsky et al., 2011 [1,2]). This understanding and acknowledgment come decades after clinical providers have extrapolated data from cardiovascular disease clinical trials conducted primarily on Caucasian men, assuming the data were generalizable to sex, race, and ethnicity. The current cardiology society guidelines, which recommend optimal medical therapies for various cardiovascular diseases, are based on trials predominantly focused on men rather than women. Sex-based research, governmental and institutional task forces, and policies on gender equity have made inroads into the disproportionate number of women's enrollment in clinical research. The National Institutes of Health in the 1990s set forth requirements on incorporating women and minorities in research, including clinical trials (Mastroianni et al., 1994; Mieres et al., 2014 [3,4]). Continued progress is imperative to improve the gap in the number of women enrolled in clinical research trials.

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We present the case of a symptomatic young woman with mitral stenosis and regurgitation due to a congenital mitral arcade. Multimodality imaging with echocardiography and computed tomography were used for diagnosis and surgical planning. The patient underwent successful bioprosthetic valve replacement. (Level of Difficulty: Intermediate.).

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The objective of this study was to monitor the extracellular brain chemistry dynamics at baseline and in relation to spontaneous seizures in human patients with refractory epilepsy. Thirty patients with drug-resistant focal epilepsy underwent intracranial electroencephalography and concurrent brain microdialysis for up to 8 continuous days. Extracellular brain glutamate, glutamine, and the branched-chain amino acids (BCAAs) valine, leucine, and isoleucine were quantified in the dialysis samples by liquid chromatography-tandem mass spectrometry. Extracellular BCAAs and glutamate were chronically elevated at baseline by approximately 1.5-3-fold in brain regions of seizure onset and propagation versus regions not involved by seizures. Moreover, isoleucine increased significantly above baseline as early as 3 h before a spontaneous seizure. BCAAs play important roles in glutamatergic neurotransmission, mitochondrial function, neurodegeneration, and mammalian target of rapamycin signaling. Because all of these processes have been implicated in epilepsy, the results suggest a novel role of BCAAs in the pathogenesis of spontaneous seizures.

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Hepatic encephalopathy secondary to hyperammonaemia is a known complication of chronic liver disease. In contrast, non-cirrhotic hyperammonaemia is a lesser-known entity that should be considered in a patient with acute encephalopathy as part of the diagnostic workup as prompt identification can help to avoid complications such as seizures and cerebral oedema. We present a case of a middle-aged woman who presented electively for a total pancreatectomy-duodenectomy with splenectomy, hepatico-jejunostomy, gastro-jejunostomy and developed encephalopathy on postoperative day 10 due to non-cirrhotic hyperammonaemia.

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Meier-Gorlin syndrome (MGS) is a rare autosomal recessive disorder characterized by the triad of short stature, microtia and absent or small patellae. We report on a patient with MGS secondary to biallelic mutations in CDC45 detected on whole exome sequencing (WES). Patients with MGS caused by mutations in CDC45 display a distinct phenotype characterized by craniosynostosis and anorectal malformation. Our patient had craniosynostosis, anorectal malformation and short stature, but did not have the microtia or patella hypoplasia. Our report also highlights the value of WES in aiding diagnosis of patients with rare genetic diseases. In conclusion, our case report and review of the literature illustrates the unique features of CDC45-related MGS as well as the benefits of WES in reducing the diagnostic odyssey for patients with rare genetic disorders.

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INTRODUCTION: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-diagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients. METHODS: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date. RESULTS: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina. CONCLUSION: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment. STUDY DESIGN: Case series, level IV evidence.

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A 55-day-old boy was transferred to our unit with intestinal obstruction and obstructive jaundice after two neonatal operations for duodenal atresia and intestinal malrotation. Abdominal ultrasound showed dilated intrahepatic and extrahepatic ducts with cut-off at the distal common bile duct (CBD). He underwent emergency laparotomy for adhesive intestinal obstruction with a contained abscess from mid-jejunal perforation. Biliary dissection was not attempted due to poor preoperative nutritional status. Tube cholecystostomy was created for biliary decompression. Postoperative magnetic resonance cholangiopancreatography showed dilated CBD with cut-off at the ampulla but did not demonstrate pancreaticobiliary maljunction (PBMJ). The diagnostic dilemma was whether our patient had congenital PBMJ or had developed biliary stricture from perioperative ischaemic scarring. He underwent definitive surgery at 7 months: excision of dilated CBD with Roux-en-Y hepaticojejeunal reconstruction, excisional tapering duodenoplasty and jejunostomy creation. Intraoperative finding was type I choledochal cyst and subsequently confirmed on histology. Postoperative recovery was uneventful and bilirubin levels normalised.

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BACKGROUND/AIMS: Posterior retroperitoneoscoic adrenalectomy has been reported as an option for adrenal tumor resection but is not commonly performed in children owing to the extreme semikneeling position advocated to flatten the lumbar lordosis in order to achieve adequate retroperitoneal space. As children have smaller lordosis angles, flattening of the lordosis and creation of optimal retroperitoneal space may be achieved with less hip flexion. We used pediatric lumbar lordosis measurements to develop a modified prone jackknife position and report our experiences with this setup for posterior retroperitoneoscopic adrenalectomy for adrenal tumors. METHODS: Lordosis angles were measured on sagittal computed tomography (CT) and magnetic resonance imaging (MRI) studies of patients with adrenal tumors and compared to normal references. The data were used to develop our modified prone jackknife position. Selected patients with adrenal tumors underwent posterior retroperitoneoscopic adrenalectomy in this position. Patient demographics, diagnoses, operative times, complications, postop analgesia requirements, and length of hospitalization were analyzed. RESULTS: CT and MRI studies were analyzed for 20 patients with adrenal tumors diagnosed in our institution from 2012 to 2017; median lordosis angle was 27.84° (range: 15.50°-36.48°) - less than reference lordosis angles of respective age groups, and flexion angles of common operating tables. Five patients underwent retroperitoneoscopic adrenalectomy between June 2016 and June 2018. Histological diagnoses were neuroblastoma, adrenal hyperplasia, pheochromocytoma, and adrenal angiomatoid fibrous histiocytoma. Median age was 4 years [range: 1-11]. Median operating time was 137 min [range 111-181 min]. No conversions to open surgery were required. One patient had intraoperative bleeding from the adrenal vein. Only 1 patient required postoperative opioids for analgesia. Median length of hospitalization after surgery was 2 days (range: 2-3 days). CONCLUSIONS: Pediatric patients can achieve flattening of lumbar lordosis with less extreme positioning. Posterior retroperitoneoscopic adrenalectomy in a modified prone jackknife position is a feasible operation for pediatric patients with small adrenal masses. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: Level III.

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OBJECTIVE: Middle ear extension of vestibular schwannomas is not a common occurrence, and only a few cases have been described so far in past publications. We report three new cases of vestibular schwannomas extending to the middle ear and reviewed the literature to specify the patterns of such an extension. MATERIALS AND METHODS: We analysed databases of previously published articles to search for additional cases of middle ear extension of vestibular schwannomas and compared them to the cases we have documented. Extension patterns of the tumours were analysed, especially focusing on the extension through the round and oval windows. RESULTS AND CONCLUSION: Middle ear vestibular schwannomas are uncommon tumours and only 13 cases have been published so far. The vestibular schwannoma (internal auditory canal or intralabyrinthine) has to invade the labyrinth first (complete invasion in 88% of the cases, n = 14), before reaching the middle ear. In the majority of cases (69%, n = 11/16), internal auditory canal vestibular schwannomas or intralabyrinthine schwannomas extended in the middle ear though the round window.

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