Comparison of Ductal Stent Versus Surgical Shunt as Initial Intervention for Neonates with Pulmonary Atresia with Intact Ventricular Septum.

Data comparing surgical systemic-to-pulmonary artery shunt and patent ductus arteriosus (PDA) stent as the initial palliation procedure for patients with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. We sought to compare characteristics and outcomes in a multicenter cohort of patients with PA-IVS undergoing surgical shunts versus PDA stents. We retrospectively reviewed neonates with PA-IVS from 2009 to 2019 in 19 United States centers. Bivariate comparisons and multivariable logistic regression analysis were performed to determine the relationship between initial palliation strategy and outcomes including major adverse cardiovascular events (MACE): stroke, mechanical circulatory support, cardiac arrest, or death. 187 patients were included: 38 PDA stents and 149 surgical shunts. Baseline characteristics did not differ statistically between groups. Post-procedural MACE occurred in 4 patients (11%) with PDA stents versus 38 (26%) with surgical shunts, p = 0.079. Overall, the initial palliation strategy was not significantly associated with MACE (aOR:0.37; 95% CI,0.13-1.02). In patients with moderate-to-severe right ventricle hypoplasia, PDA stents were significantly associated with decreased odds of MACE (aOR:0.36; 95% CI,0.13-0.99). PDA stents were associated with lower vasoactive inotrope scores (median 0 versus 5, p < 0.001), greater likelihood to be extubated at the end of their procedure (37% versus 4%, p < 0.001), and shorter duration of mechanical ventilation (median 24 versus 96 h, p < 0.001). PDA stents were associated with significantly more unplanned reinterventions for hypoxemia compared to surgical shunts (42% vs. 20%, p = 0.009). In this multicenter study, neonates with PA-IVS who underwent PDA stenting received less vasoactive and ventilatory support postoperatively compared to those who had surgical shunts. Furthermore, patients with the most severe morphology had decreased odds of MACE.

Clinical Outcomes After Tracheostomy in Children With Single Ventricle Physiology: Collaborative Research From the Pediatric Cardiac Intensive Care Society Multicenter Cohort, 2010-2021.

OBJECTIVES: Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle physiology. We aimed to describe clinical characteristics and outcomes in a multicenter cohort of patients with single ventricle physiology who underwent tracheostomy before Fontan operation. DESIGN: Multicenter retrospective cohort study.SETTING: Twenty-one tertiary care pediatric institutions participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society. PATIENTS: We reviewed 99 children with single ventricle physiology who underwent tracheostomy before the Fontan operation at 21 institutions participating in Collaborative Research from the Pediatric Cardiac Intensive Care Society between January 2010 and December 2020, with follow-up through December 31, 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Death occurred in 51 of 99 patients (52%). Cox proportional hazard analysis was performed to determine factors associated with death after tracheostomy. Results are presented as hazard ratio (HR) with 95% CIs. Nonrespiratory indication(s) for tracheostomy (HR, 2.21; 95% CI, 1.14-4.32) and number of weeks receiving mechanical ventilation before tracheostomy (HR, 1.06; 95% CI, 1.02-1.11) were independently associated with greater hazard of death. In contrast, diagnosis of tricuspid atresia or Ebstein's anomaly was associated with less hazard of death (HR, 0.16; 95% CI, 0.04-0.69). Favorable outcome, defined as survival to Fontan operation or decannulation while awaiting Fontan operation with viable cardiopulmonary physiology, occurred in 29 of 99 patients (29%). Median duration of mechanical ventilation before tracheostomy was shorter in patients who survived to favorable outcome (6.1 vs. 12.1 wk; p < 0.001), and only one of 16 patients with neurologic indications for tracheostomy and 0 of ten patients with cardiac indications for tracheostomy survived to favorable outcome. CONCLUSIONS: For children with single ventricle physiology who undergo tracheostomy, mortality risk is high and should be carefully considered when discussing tracheostomy as an option for these children. Favorable outcomes are possible, although thoughtful attention to patient selection and tracheostomy timing are likely necessary to achieve this goal.

Remaining Useful Life Estimation of Hollow Worn Railway Vehicle Wheels via On-Board Random Vibration-Based Wheel Tread Depth Estimation.

The problem of remaining useful life estimation (RULE) of hollow worn railway vehicle wheels in terms of remaining mileage via wheel tread depth estimation using on-board vibration signals from a single accelerometer on the bogie frame is presently investigated. This is achieved based on the introduction of a statistical time series method that employs: (i) advanced data-driven stochastic Functionally Pooled models for the modeling of the vehicle dynamics under different wheel tread depths in a range of interest until a critical limit, as well as tread depth estimation through a proper optimization procedure, and (ii) a wheel tread depth evolution function with respect to the vehicle running mileage that interconnects the estimated hollow wear with the remaining useful mileage. The method's RULE performance is investigated via hundreds of Simpack-based Monte Carlo simulations with an Attiko Metro S.A. vehicle and many hollow worn wheels scenarios which are not used for the method's training. The obtained results indicate the accurate estimation of the wheels tread depth with a mean absolute error of ∼0.07 mm that leads to a corresponding small error of ∼3% with respect to the wheels remaining useful mileage. In addition, the comparison with a recently introduced Multiple Model (MM)-based multi-health state classification method for RULE, demonstrates the better performance of the postulated method that achieves 81.17% True Positive Rate (TPR) which is significantly higher than the 45.44% of the MM method.

The 'Holy Grail' of shoulder dislocations: a systematic review on traumatic bilateral luxatio erecta; is it in reality a once-in-a-lifetime experience for an orthopaedic surgeon?

INTRODUCTION: Even though shoulder dislocation is thought to be the most common dislocation treated in the Emergency Department, inferior ones, known as Luxatio Erecta, comprise only 0.5% of them. Taking into consideration the rareness of unilateral Luxatio Erecta, bilateral cases should be even fewer. The purpose of this paper is to identify the reported number of cases of Traumatic Bilateral Luxatio Erecta in the literature over the last 100 years and to summarize the mechanism of injury, the initial management, and the complications of these patients. MATERIALS AND METHODS: We performed a systematic review of the literature regarding Traumatic Bilateral Luxatio Erecta. All articles published until 31st of December 2022 in PubMed and Google Scholar databases were searched using the terms "luxatio erecta", 'inferior dislocation", and "bilateral". RESULTS: Eighty-two articles were retrieved from PubMed and Google Scholar search. Forty-four of them were initially included in our review. Six additional articles meeting the inclusion criteria were found from cross-references. CONCLUSION: The presence of this injury is extremely rare with only 51 cases in the literature. The incidence of concomitant injuries and complications seems to be extremely high and neurological deficits were detected on 42.8% of patients with Bilateral Luxatio Erecta. To our knowledge, this is the first systematic review of the literature regarding Traumatic Bilateral Luxatio Erecta that includes articles not only in English, a fact that provides more reliability on the estimation of the real number of cases of this rare injury compared to any other review on this subject to date.

Bone mineral density, vitamin D and osseous metabolism indices in neurofibromatosis type 1: A systematic review and meta-analysis.

BACKGROUND: Neurofibromatosis type 1 (NF1) is a genetic autosomal neurocutaneous syndrome correlated with skeletal dysplasia and defects in the osseous microarchitecture. The physiological mechanism for the development of NF1-related bone abnormal turnover is still unclear. OBJECTIVES: A meta-analysis was performed to investigate the effects of NF1 on bone mineral density (BMD) and osseous metabolic indices in order to provide clinical evidence for the pathogenesis of the associated skeletal deformities. METHODS: A systematic literature review search was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines in the PubMed/Medline and Web of Science databases from the date of inception of each database through to 10 September 2023. Specific inclusion and exclusion criteria were applied for the identification of studies examining the effects of NF1 on bone strength and metabolism. The Newcastle-Ottawa and Jadad scales were applied to assess the quality of the included studies. RevMan 5.3 software was used for the analysis of the data, and MedCalc was applied to examine publication bias. RESULTS: Overall, 13 studies met the inclusion criteria comprised of 5 cross-sectional, 6 case-control and 2 retrospective studies. 703 patients and 973 healthy subjects formed the NF1 and control group, respectively. The results of the meta-analysis displayed that lumbar (SMD = -3.85, 95%CI = -7.53 to -0.18, Z = 2.05, p = 0.04) and femoral (SMD = -4.78, 95%CI = -8.86 to -0.69, Z = 2.29, p = 0.02) BMD was reduced in the NF1 group. Both in children and adults the serum levels of 25 hydroxyvitamin D3 were also decreased in NF1 group, but without any statistical significance (SMD = -0.62, 95%CI = -1.34 to -0.11, Z = 1.66, p = 0.10). Serum Parathyroid hormone (PTH) (SMD = 0.73, 95%CI = 0.31 to 1.15, Z = 3.43, p = 0.0006) and C-telopeptide of type 1 collagen (CTX) (SMD = 0.82, 95%CI = 0.33 to 1.30, Z = 3.29, p = 0.001) were elevated in NF1 patients, while serum calcium (SMD = -0.10, 95%CI = -0.74 to 0.53, Z = 0.32, p = 0.75) phosphorous (SMD = 0.33, 95%CI = -0.38 to 1.05, Z = 0.92, p = 0.36), alkaline phosphatase (ALP) (SMD = -0.36, 95%CI = -0.77 to 0.05, Z = 1.71, p = 0.09), osteocalcin (SMD = 1.81, 95%CI = -0.37 to -3.98, Z = 1.63, p = 0.10) and bone formation markers (SMD = 0.28, 95%CI = -0.37 to -0.94, Z = 0.85, p = 0.39) were not. CONCLUSION: NF1 is associated with decreased BMD at the lumbar spine and femur. Taking into account that the serum levels of PTH, CTX were increased whereas the concentrations of vitamin D, calcium, phosphorous, ALP, osteocalcin and bone formation markers were not altered significantly in the NF1 patients compared with the healthy subjects, a vitamin D independent dysregulated bone cellular activity could be considered. STUDY REGISTRATION: Registered on PROSPERO (CRD42023424751).

Static Baropodometry for Assessing Short-Term Functional Outcome after Unilateral Total Knee Arthroplasty: Exploring Correlation between Static Plantar Pressure Measurements and Self-Reported Outcomes.

This study aimed to investigate the association between objective baropodometric and radiological measurements and patient self-reported functional outcomes, assessed through the Knee Injury and Osteoarthritis Outcome Score (KOOS). Additionally, it sought to evaluate the effectiveness of static baropodometry in predicting short-term KOOS results following unilateral total knee arthroplasty (TKA). We conducted a prospective single-center study involving 32 patients who underwent unilateral TKA for knee osteoarthritis (KOA). Patients were evaluated both preoperatively and six months postoperatively, utilizing objective measurements derived from static baropodometric analysis in a normal, relaxed, bipedal standing position using a multi-platform Plantar Pressure Analysis System (PPAS) and radiographic measurements of the femorotibial angle (FTA) and subjective assessments through the national validated version of the KOOS. The study found an insignificant average correction of -0.69° ± 4.12° in the preoperative FTA at the sixth month after TKA. Moreover, there were no significant differences in the KOOS based on different types of knee alignment (KA) both pre- and postoperatively (p > 0.05). No significant correlations were observed between the KOOS, and total average affected and unaffected plantar pressures (TAAPP and TAUPP) pre- and postoperatively, as well as KA pre- and postoperatively. However, significant changes were observed in TAAPP and TAUPP measurements after unilateral TKA. TAAPP demonstrated a significant increase postoperatively (mean change (SD) = 18.60 (47.71); p = 0.035). In conclusion, this study found no significant correlation between KA, static baropodometric measurements, including pre- and postoperative differences, and KOOS outcomes. Therefore, static plantar pressure measurements alone might not serve as a reliable predictor of short-term clinical outcomes after unilateral TKA, as reported by patients.

Atrial arrhythmias following lung transplant: a single pediatric center experience.

Background: Outcomes after lung transplant (LTx) in children have slowly improved. Although atrial arrhythmia (AA) is a common and adverse complication following LTx among adults, there is limited data on pediatric recipients. We detail our pediatric single-center experience while providing further insights on occurrence and management of AA following LTx. Methods: A retrospective analysis of LTx recipients at a pediatric LTx program from 2014 to 2022 was performed. We investigated timing of occurrence and management of AA following LTx, and its effect on post-LTx outcome. Results: Three out of nineteen (15%) pediatric LTx recipients developed AA. The timing of occurrence was 9-10 days following LTx. Those patients in the older age group (age >12 years old) were the only ones who developed AA. Developing AA did not have a negative effect on hospital stay duration or short-term mortality. All LTx recipients with AA were discharged home on therapy that was discontinued at 6 months for those who was on mono-therapy without recurrence of AA. Conclusions: AA is an early post-operative complication in older children and younger adults undergoing LTx at a pediatric center. Early recognition and aggressive management can mitigate any morbidity or mortality. Future investigations should explore factors that place this population at risk for AA in order to prevent this complication post-operatively.

Impact of COVID-19 Pandemic on Fragility Fractures of the Hip: An Interrupted Time-Series Analysis of the Lockdown Periods in Western Greece and Review of the Literature.

Since December 2019, the COVID-19 pandemic has had a significant impact on healthcare systems worldwide, prompting policymakers to implement measures of isolation and eventually adopt strict national lockdowns, which affected mobility, healthcare-seeking behavior, and services, in an unprecedented manner. This study aimed to analyze the effects of these lockdowns on hip-fracture epidemiology and care services, compared to nonpandemic periods in previous years. We retrospectively collected data from electronic patient records of two major hospitals in Western Greece and included patients who suffered a fragility hip fracture and were admitted during the two 5-week lockdown periods in 2020, compared to time-matched patients from 2017-2019. The results showed a drop in hip-fracture incidence, which varied among hospitals and lockdown periods, and conflicting impacts on time to surgery, time to discharge after surgery, and total hospitalization time. The study also found that differences between the two differently organized units were exaggerated during the COVID-19 lockdown periods, highlighting the impact of compliance with social-distancing measures and the reallocation of resources on the quality of healthcare services. Further research is needed to fully understand the specific variations and patterns of geriatric hip-fracture care during emergency health crises characterized by limited resources and behavioral changes.

Subchondral Bone Cyst Development in Osteoarthritis: From Pathophysiology to Bone Microarchitecture Changes and Clinical Implementations.

Osteoarthritis is a degenerative joint disease affecting middle-aged and elderly patients. It mainly involves weight-bearing joints such as the hip, knee and spine as well as the basilar joint of the thumb, causing dysfunction and painful symptoms. Often, joint arthritis is accompanied by cartilage defects, joint space narrowing, osteophytes, bone sclerosis and subchondral bone cysts (SBC). The aim of the present study was to explore the pathophysiology responsible for the development of SBCs as well as the association between SBCs and disease progress, the level of clinical symptoms and their impact on postoperative outcomes and risk of possible complications following joint replacements if left untreated. A literature review on PubMed articles was conducted to retrieve and evaluate all available evidence related to the main objective mentioned above. A few theories have been put forth to explain the formation process of SBCs. These involve MMPs secretion, angiogenesis, and enhanced bone turnover as a biological response to abnormal mechanical loads causing repeated injuries on cartilage and subchondral tissue during the development of arthritis. However, the application of novel therapeutics, celecoxib-coated microspheres, local administration of IGF-1 and activated chondrocytes following surgical debridement of SBCs hinders the expansion of SBCs and prevents the progression of osteoarthritis.

Absolute Versus Relative Near-Infrared Spectroscopy in Pediatric Cardiac Patients.

OBJECTIVES: Near-infrared spectroscopy (NIRS) has been increasingly accepted as a noninvasive marker of regional tissue oxygenation despite concerns of imprecision and wide limits of agreement (LOA) with invasive oximetry. New generation absolute monitors may have improved accuracy compared with trend monitors. We sought to compare the concordance with invasive venous oximetry of a new generation absolute NIRS-oximeter (FORESIGHT ELITE; CASMED, Branford, CT) with a modern widely used trend monitor (INVOS 5100C; Medtronic, Minneapolis, MN). DESIGN: Prospective single-center study. SETTING: Tertiary pediatric heart center. PATIENTS: Children undergoing elective cardiac catheterization under general anesthesia. Time-paired venous oximetry samples (jugular and renal) were compared with NIRS-derived oximetry by two monitors using regression and Bland-Altman analysis. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We enrolled 36 children (19 female, 10 cyanotic) with median age 4.1 years (25-75%, 2.5-7.8 yr) and weight 16.7 kg (12.3-29.1 kg). The absolute difference between NIRS-derived and invasive jugular oximetry was less than 10% in 67% of occasions for both monitors. Correlation was fair (Spearman rs = 0.40; p = 0.001) for the FORESIGHT ELITE and poor ( rs = 0.06; p = 0.71) for the INVOS 5100C. Bias and LOA were +6.7% (+22%, -9%) versus +1.3% (LOA = +24%, -21%), respectively. The absolute difference between NIRS-derived and invasive renal oximetry was less than 10% in 80% of occasions with moderate correlation ( rs = 0.57; p < 0.001) for the FORESIGHT ELITE and in 61% of occasions with moderate correlation ( rs = 0.58; p < 0.001) for the INVOS 5100C; bias and LOA were +3.6% (+19%, -12%) and -1.4 % (+27%, -30%), respectively. NIRS correlation with renal venous oximetry was worse for cyanotic versus noncyanotic patients ( p = 0.02). CONCLUSIONS: Concordance and LOA of NIRS-derived oximetry with invasive venous oximetry in the cerebral and renal vascular beds was suboptimal for clinical decision-making. Cyanosis adversely affected NIRS performance in the renal site.

Procedural Outcomes of Pulmonary Atresia With Intact Ventricular Septum in Neonates: A Multicenter Study.

BACKGROUND: Multicenter contemporary data describing short-term outcomes after initial interventions of neonates with pulmonary atresia with intact ventricular septum (PA-IVS) are limited. This multicenter study describes characteristics and outcomes of PA-IVS neonates after their initial catheter or surgical intervention and identifies factors associated with major adverse cardiac events (MACE). METHODS: Neonates with PA-IVS who underwent surgical or catheter intervention between 2009 and 2019 in 19 centers were reviewed. Risk factors for MACE, defined as cardiopulmonary resuscitation, mechanical circulatory support, stroke, or in-hospital mortality, were analyzed using multivariable logistic regression models. RESULTS: We reviewed 279 neonates: 79 (28%) underwent right ventricular decompression, 151 (54%) underwent systemic-to-pulmonary shunt or ductal stent placement only, 36 (13%) underwent right ventricular decompression with shunt or ductal stent placement, and 11 (4%) underwent transplantation. MACE occurred in 57 patients (20%): 26 (9%) received mechanical circulatory support, 37 (13%) received cardiopulmonary resuscitation, stroke occurred in 16 (6%), and 23 (8%) died. The presence of 2 major coronary artery stenoses (adjusted odds ratio, 4.99; 95% CI, 1.16-21.39) and lower weight at first intervention (adjusted odds ratio, 1.52; 95% CI, 1.01-2.27) were significantly associated with MACE. Coronary ischemia was the most frequent presumed mechanism of death (n = 10). CONCLUSIONS: In a multicenter cohort, 1 in 5 neonates with PA-IVS experienced MACE after their initial intervention. Patients with 2 major coronary artery stenoses or lower weight at the time of the initial procedure were most likely to experience MACE and warrant vigilance during preintervention planning and postintervention management.

Implementation of a 24-hour infection diagnosis protocol in the pediatric cardiac intensive care unit (CICU).

OBJECTIVES: To reduce unnecessary antibiotic exposure in a pediatric cardiac intensive care unit (CICU). DESIGN: Single-center, quality improvement initiative. Monthly antibiotic utilization rates were compared between 12-month baseline and 18-month intervention periods. SETTING: A 25-bed pediatric CICU. PATIENTS: Clinically stable patients undergoing infection diagnosis were included. Patients with immunodeficiency, mechanical circulatory support, open sternum, and recent culture-positive infection were excluded. INTERVENTIONS: The key drivers for improvement were standardizing the infection diagnosis process, order-set creation, limitation of initial antibiotic prescription to 24 hours, discouraging indiscriminate vancomycin use, and improving bedside communication and situational awareness regarding the infection diagnosis protocol. RESULTS: In total, 109 patients received the protocol; antibiotics were discontinued in 24 hours in 72 cases (66%). The most common reasons for continuing antibiotics beyond 24 hours were positive culture (n = 13) and provider preference (n = 13). A statistical process control analysis showed only a trend in monthly mean antibiotic utilization rate in the intervention period compared to the baseline period: 32.6% (SD, 6.1%) antibiotic utilization rate during the intervention period versus 36.6% (SD, 5.4%) during the baseline period (mean difference, 4%; 95% CI, -0.5% to -8.5%; P = .07). However, a special-cause variation represented a 26% reduction in mean monthly vancomycin use during the intervention period. In the patients who had antibiotics discontinued at 24 hours, delayed culture positivity was rare. CONCLUSIONS: Implementation of a protocol limiting empiric antibiotic courses to 24 hours in clinically stable, standard-risk, pediatric CICU patients with negative cultures is feasible. This practice appears safe and may reduce harm by decreasing unnecessary antibiotic exposure.

Contemporary Multicenter Outcomes for Truncus Arteriosus With Interrupted Aortic Arch.

BACKGROUND: Truncus arteriosus with interrupted aortic arch (TA-IAA) is a rare congenital heart defect with historically poor outcomes. Contemporary multicenter data are limited. METHODS: A retrospective cohort study of children who underwent repair of TA-IAA between 2009 and 2016 at 12 tertiary care referral centers within the United States was performed. Major adverse cardiac events (MACE) were defined as postoperative extracorporeal membrane oxygenation, cardiopulmonary resuscitation, or operative mortality. TA-IAA patients were compared with TA patients who underwent repair during the study period from the same institutions. RESULTS: We reviewed 35 patients with TA-IAA. MACE occurred in 12 patients (34%). Improvement over time was observed during the study period with 11 events (92%) occurring in the first half of the study period (P = .03). Factors associated with MACE included moderate or severe truncal valve insufficiency (P < .01), concomitant truncal valve repair (P = .04), and longer cardiopulmonary bypass duration (P = .02). In comparison with 216 patients who underwent TA repair, patients with TA-IAA had a higher rate of MACE, but this finding was not statistically significant (34% vs 20%, respectively; P = .07). Additionally no differences between TA-IAA and TA groups were observed for unplanned reoperations (14% vs 22%, respectively; P = .3), hospital length of stay (24 vs 23 days, P = .65), or late deaths (7% vs 7%, P = 1.00). CONCLUSIONS: In this contemporary, multicenter cohort the rate of MACE after repair of TA-IAA was high but improved during the study period. Early childhood outcomes of patients with TA-IAA were similar to those with TA.

Hypophosphatemic osteomalacia in neurofibromatosis 1 associated with intracranial gliomas and congenital renal agenesis: A rare case report and review of the literature.

Introduction: Neurofibromatosis Type 1 (Nf1), also termed von Recklinghausen disease, is a rare autosomal dominant genetic disorder accompanied by several osseous and skeletal manifestations. In NF, hypophosphatemia linked to secondary hyperparathyroidism due to Vitamin D deficiency and low calcium intake has been reported as a risk factor for low bone mass density (BMD), but reports of NF1 associated oncogenic hypophosphatemic osteomalacia (HO) are extremely rare. Case Report: We report a patient with NF1 associated with intracranial low-grade gliomas and congenital renal agenesis suffering from HO. Bone defects and deformities such as generalized bone pains located in feet, ankles and lower limbs, thoracic scoliosis, mild bowing of long bones of lower limbs, stress fractures, and old fractures as well as with altered bone metabolic serum markers were present. After 8 weeks of follow-up, it was observed that the combination of oral administration of phosphate and Vitamin D improved her medical symptoms without significant changes in phosphate levels or BMD. Conclusion: Although renal agenesis is not correlated with hypophosphatemia, the coexistence of NF1, renal congenital deformities, and low-grade gliomas may contribute to disease severity. Conventional treatment with high doses of oral calcitriol associated with phosphate is efficient to improve the clinical and laboratory symptoms of the disease.

Surgical Repair of Complete Common Atrioventricular Canal in a Conjoined Twin Following Separation.

Congenital heart disease (CHD) is common in conjoined twins but reports of surgical repair in this population are rare. We present the case of a conjoined twin diagnosed prenatally with a complete common atrioventricular canal defect (CAVC). Due to wound complications from twin separation, definitive repair was deferred and pulmonary arterial banding was performed initially. At 6 months, a complete CAVC repair was performed. The present case adds to the literature on successful cardiac surgical repairs in conjoined twins.

Pulmonary Atresia with Intact Ventricular Septum: Midterm Outcomes from a Multicenter Cohort.

Contemporary multicenter data regarding midterm outcomes for neonates with pulmonary atresia with intact ventricular septum are lacking. We sought to describe outcomes in a contemporary multicenter cohort, determine factors associated with end-states, and evaluate the effect of right ventricular coronary dependency and coronary atresia on transplant-free survival. Neonates treated during 2009-2019 in 19 United States centers were reviewed. Competing risks analysis was performed to determine cumulative risk of each end-state, and multivariable regression analyses were performed to identify factors associated with each end-state and transplant-free survival. We reviewed 295 patients. Median tricuspid valve Z-score was - 3.06 (25%, 75%: - 4.00, - 1.52). Final end-state was biventricular repair for 45 patients (15.2%), one-and-a half ventricle for 16 (5.4%), Fontan for 75 (25.4%), cardiac transplantation for 29 (9.8%), and death for 54 (18.3%). Seventy-six patients (25.7%) remained in mixed circulation. Cumulative risk estimate of death was 10.9%, 16.1%, 16.9%, and 18.8% at 1, 6 months, 1 year, and 5 years, respectively. Tricuspid valve Z-score was inversely, and coronary atresia positively associated with death or transplantation [odds ratio (OR) = 0.46, (95% confidence interval (CI) = 0.29-0.75, p < 0.001) and OR = 3.75 (95% CI 1.46-9.61, p = 0.011), respectively]. Right ventricular coronary dependency and left coronary atresia had a significant effect on transplant-free survival (log-rank p < 0.001). In a contemporary multicenter cohort of patients with PAIVS, consisting predominantly of patients with moderate-to-severe right ventricular hypoplasia, we observed favorable survival outcomes. Right ventricular coronary dependency and left, but not right, coronary atresia significantly worsens transplant-free survival.

Sex, but not age and bone mass index positively impact on the development of osteochondral micro-defects and the accompanying cellular alterations during osteoarthritis progression.

Background: Osteoarthritis (ΟΑ) is characterized by cartilage breakdown and subchondral sclerosis. Micro-fractures of the calcified tissues have been, also, detected, but their exact role has not been elucidated yet. This study was to examine the frequency of cracks during OA progression and to correlate them with the underlying cellular modifications and matrix metalloproteinase-2 (MMP-2) expression using histological/immunohistological methods. Methods: Overall, 20 patients and 3 controls (9 specimens per patient), aged 60-89 years, diagnosed with hip/knee OA were included. The development of cracks was examined in 138 sections, whereas the expression of MMP-2 was examined in 69 additional sections. Results: Based on Mankin score, three groups of OA severity were analyzed: Group I (mild) was constituted of sections with score 1-5 while Groups II (moderate) and III (severe) with score 6-7 and greater or equal to 8, respectively. Demographic characteristics did not reveal any association between the number of microdefects and age or body mass index (BMI). Cartilage micro-cracks were increased during moderate and severe OA, while bone cracks were increased during mild and severe OA. In knee OA, cartilage cracks were not correlated with Mankin score, whereas in hip OA they appeared association with severity score. Bone cracks were positively correlated with matrix apoptotic osteocytes and osteoblastic cells, but not with osteoclasts. MMP-2 immunostaining was increasing by OA severity in the osteochondral unit. Similarly, MMP-2 was expressed on the microcracks' wall mainly in Group III. Conclusion: Our data displayed that bone cracks during primary OA stages, represent an early adaptative mechanism aiming to maintain cartilage integrity. Accumulation of bone defects and concomitant increase of apoptotic osteocytes activated an abnormal remodeling due to osteoblastic activity, in which MMP-2 played a pivotal role, leading to subchondral sclerosis promoting further osteochondral deformities.

Measurement of Cardiac Output Using an Ultrasonic Cardiac Output Monitor (USCOM) in Patients with Single-Ventricle Physiology.

We evaluate the validity of cardiac index (CI) measurements utilizing the Ultrasonic Cardiac Output Monitor (USCOM), a non-invasive Doppler ultrasound device, by comparing measurements to cardiac catheterization-derived CI measurements in patients with single-ventricle physiology. USCOM measurements were repeated three times for each patient at the beginning of a cardiac catheterization procedure for twenty-six patients undergoing elective pre-Glenn or pre-Fontan catheterization. CI was measured by USCOM and was calculated from cardiac catheterization data using Fick's method. Bland-Altman analysis for CI showed bias of 0.95 L/min/m2 with the 95% limits of agreement of - 1.85 and 3.75. Pearson's correlation coefficient was 0.89 (p < 0.001) indicating a strong positive relationship between USCOM and cardiac catheterization CI measurements. When excluding two patients with significant dilation of the neo-aortic valve (z-score > + 5), the bias improved to 0.66 L/min/m2 with the 95% limits of agreement of - 1.38 and 2.70. Percent error of limits of agreement was 34%. There was excellent intra-operator reproducibility of USCOM CI measurements with an intra-class coefficient of 0.96. We demonstrate the use of USCOM to measure CI in patients with single-ventricle physiology for the first time, showing acceptable agreement of the CI measurements between USCOM and cardiac catheterization with a high intra-operator reproducibility.

Current Aspects on the Pathophysiology of Bone Metabolic Defects during Progression of Scoliosis in Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF1), which is the most common phacomatoses, is an autosomal dominant disorder characterized by clinical presentations in various tissues and organs, such as the skin, eyes and nervous and skeletal systems. The musculoskeletal implications of NF1 include a variety of deformities, including scoliosis, kyphoscoliosis, spondylolistheses, congenital bony bowing, pseudarthrosis and bone dysplasia. Scoliosis is the most common skeletal problem, affecting 10-30% of NF1 patients. Although the pathophysiology of spinal deformities has not been elucidated yet, defects in bone metabolism have been implicated in the progression of scoliotic curves. Measurements of Bone Mineral Density (BMD) in the lumbar spine by using dual energy absorptiometry (DXA) and quantitative computer tomography (QCT) have demonstrated a marked reduction in Z-score and osteoporosis. Additionally, serum bone metabolic markers, such as vitamin D, calcium, phosphorus, osteocalcin and alkaline phosphatase, have been found to be abnormal. Intraoperative and histological vertebral analysis confirmed that alterations of the trabecular microarchitecture are associated with inadequate bone turnover, indicating generalized bone metabolic defects. At the molecular level, loss of function of neurofibromin dysregulates Ras and Transforming Growth factor-ß1 (TGF-ß1) signaling and leads to altered osteoclastic proliferation, osteoblastic activity and collagen production. Correlation between clinical characteristics and molecular pathways may provide targets for novel therapeutic approaches in NF1.

Double dynamic hydrogels formed by wormlike surfactant micelles and cross-linked polymer.

HYPOTHESIS: Interpenetrating networks consisting of a polymer network with dynamic cross-links and a supramolecular network allow obtaining hydrogels with significantly enhanced mechanical properties. EXPERIMENTS: Binary hydrogels composed of a dynamically cross-linked poly(vinyl alcohol) (PVA) network and a transient network of entangled highly charged mixed wormlike micelles (WLMs) of surfactants (potassium oleate and n-octyltrimethylammonium bromide) were prepared and studied by rheometry, SANS, USANS, cryo-TEM, and NMR spectroscopy. FINDINGS: Binary hydrogels show significantly enhanced rheological properties (a 3400-fold higher viscosity and 27-fold higher plateau modulus) as compared to their components taken separately. This is due to the microphase separation leading to local concentrating of PVA and WLMs providing larger number of polymer-polymer contacts for cross-linking and longer WLMs with more entanglements. Such materials are very promising for the application in many areas, ranging from enhanced oil recovery to biomedical uses.