Anatomic and physiologic diagnostic discrepancies in fetuses with single ventricle congenital heart disease in a contemporary cohort.

OBJECTIVE: Image quality of fetal echocardiography (FE) has improved in the recent era, but few recent studies have reported the accuracy of FE specifically in single ventricle congenital heart disease (SV). Our study aims to assess the ability of FE to correctly predict postnatal anatomy and physiology in SV in a contemporary cohort. METHODS: The contemporary, clinical reports of FE with SV performed from 7/2017 to 7/2021 were compared with postnatal echocardiograms in a formal quality assurance program. SV were grouped by anatomic subtype. Diagnostic errors were designated as major if the error would have resulted in significant alteration in fetal counseling or postnatal management. Remaining errors were classified as minor. Physiologic discrepancies, including prostaglandin dependency (PGE-D), atrioventricular valve regurgitation (AVVR), pulmonary venous obstruction, and atrial septal restriction were assessed by review of postnatal course. RESULTS: A total of 119 subjects were analyzed. SV subtypes in the cohort included hypoplastic left heart syndrome (HLHS) (n=68), tricuspid atresia (n=16), double inlet left ventricle (n=12), unbalanced atrioventricular canal (UAVC) (n=11), heterotaxy (n=9), and other (n=3). The rate of major anatomic and physiologic errors was low (n=6, 5.0%). A higher proportion of minor errors were noted in HLHS and tricuspid atresia but the differences were not statistically significant. Physiologic discrepancies were uncommon, with three major discrepancies including underestimation of degree of venous obstruction in one non-HLHS fetus with total anomalous pulmonary venous return, overestimation of atrial septal restriction in one HLHS fetus, and incorrect prediction of PGE-D with 1 false-negative for pulmonary blood flow. No discrepancy in degree of AVVR or atrial septal restriction affected postnatal care. Minor physiologic discrepancies included two false-positive predictions of PGE-D with 1 false-positive for ductal-dependent systemic flow, and 1 false-positive for pulmonary blood flow. CONCLUSIONS: In this contemporary review of FE in SV at our center, there was high accuracy in describing anatomic and physiologic findings in fetuses with SV. Major physiologic discrepancies were uncommon but included important cases of false-negative prediction of PGE-dependence and underestimation of obstruction of total anomalous pulmonary venous return. These data can both inform more accurate counseling of families with SV fetuses and guide diagnostic improvement efforts. This article is protected by copyright. All rights reserved.

The temporal burden of preparing catheters for re-use in adults with spinal cord injury: a cross-sectional study.

STUDY DESIGN: Cross-sectional OBJECTIVES: Neurogenic lower urinary tract dysfunction is common among people with spinal cord injury (SCI). Although single-use clean intermittent catheterization is recommended to facilitate routine bladder emptying, catheter re-use is common. Barriers associated with the preparation (i.e., cleaning) of catheters for re-use are unknown. This study examined barriers to catheter re-use in adult individuals with SCI by assessing (1) the time needed to clean a catheter, and (2) the perceived difficulty of the catheter cleaning routine. SETTING: Laboratory METHODS: Twenty individuals with chronic SCI ( ≥ 1 year since injury; Group 1 = 10 with tetraplegia; Group 2 = 10 with paraplegia) completed the study. Using a standardized cleaning procedure (i.e., Milton method), catheter cleaning was timed for each participant. Perceived difficulty was assessed using a 5-point Likert scale. Functional impairment was assessed with the Upper Extremity Motor Score (UEMS). RESULTS: Significant between-group differences were observed for total cleaning time (Group 1 = 1584.1 ± 179.8 s; Group 2 = 1321.0 ± 93.8 s, p = 0.004) and perceived difficulty [Group 1 = 2.6 (2, 3); Group 2 = 2 (1.7, 2.3), p = 0.028]. Total cleaning time was significantly correlated with UEMS (ρ = -0.709, p ≤ 0.001) and perceived difficulty (ρ = 0.468, p = 0.037). UEMS emerged as an independent predictor of total cleaning time (R2 = 0.745, ß = -0.833, p ≤ 0.001). CONCLUSIONS: Preparing catheters for re-use is time-intensive and difficult for people with higher SCI level, severity and more pronounced upper limb motor impairment, which was independently associated with total cleaning time. Performing this routine on a consistent basis would require a substantial time commitment and would have a profoundly negative impact on overall quality of life.

Myocarditis post-SARS-CoV-2 vaccination: a systematic review.

Variable clinical criteria taken by medical professionals across the world for myocarditis following coronavirus disease 2019 (COVID-19) vaccination along with wide variation in treatment necessitates understanding and reviewing the same. A systematic review was conducted to elucidate the clinical findings, laboratory parameters, treatment and outcomes of individuals with myocarditis after COVID-19 vaccination after registering with PROSPERO. Electronic databases including MEDLINE, EMBASE, PubMed, LitCovid, Scopus, ScienceDirect, Cochrane Library, Google Scholar and Web of Science were searched. A total of 85 articles encompassing 2184 patients were analysed. It was a predominantly male (73.4%) and young population (mean age: 25.5 ± 14.2 years) with most having taken an mRNA-based vaccine (99.4%). The mean duration from vaccination to symptom onset was 4.01 ± 6.99 days. Chest pain (90.1%), dyspnoea (25.7%) and fever (11.9%) were the most common symptoms. Only 2.3% had comorbidities. CRP was elevated in 83.3% and cardiac troponin in 97.6% patients. An abnormal ECG was reported in 979/1313 (74.6%) patients with ST-segment elevation being most common (34.9%). Echocardiographic data were available for 1243 patients (56.9%), of whom 288 (23.2%) had reduced left ventricular ejection fraction. Non-steroidal antiinflammatory drugs (76.5%), steroids (14.1%) followed by colchicine (7.3%) were used for treatment. Only 6 patients died among 1317 of whom data were available. Myocarditis following COVID-19 vaccination is often mild, seen more commonly in young healthy males and is followed by rapid recovery with conservative treatment. The emergence of this adverse event calls for harmonizing case definitions and definite treatment guidelines, which require wider research.

Evaluation of supraspinatus strengthening exercises based on fiber bundle architectural changes.

Supraspinatus strengthening is an integral part of rehabilitation programs. However, there is disparity regarding the best exercise. Thus, we investigated the effects of supraspinatus strengthening exercises on muscle fiber bundle architecture using real-time ultrasound. Participants were randomized into full-can (FC; n = 12), empty-can (EC; n = 11,) and prone horizontal abduction (PHA; n = 11) groups and performed three sessions/week for 8 weeks. Each session involved four sets of exercise for weeks 1-4 and six sets for weeks 5-8 with eight repetitions/set. Images of supraspinatus were captured in 0° (relaxed) and 60° (contracted) glenohumeral abduction. Fiber bundle length (FBL), pennation angle, and muscle thickness were measured at beginning, mid, and end of training. Maximum isometric abduction strength was measured in full-can, empty-can, and prone horizontal abduction positions using a hand-held dynamometer. FBL decreased after 4 weeks in FC [relaxed: Δ = -0.54 cm, P = 0.001, d = 0.92; contracted: Δ = -0.27 cm, P = 0.008, d = 0.27] and EC [relaxed: Δ = -0.59 cm, P = 0.008, d = 0.86; contracted: Δ = -0.36 cm, P = 0.013, d = 0.59], but not in PHA [relaxed: Δ = -0.11, P = 0.121, d = 0.31; contracted: Δ = -0.06 cm, P = 0.486, d = 0.13]. Strength increased significantly from week 4-8 only in PHA (Δ = +0.96 kg, P = 0.004, d = 0.47) when tested in prone horizontal abduction position. Maintenance of FBL with increased strength indicates prone horizontal abduction may be a better exercise to strengthen supraspinatus.

Innate and humoral recognition of the products of cell death: differential antigenicity and immunogenicity in lupus.

While apoptotic debris is believed to constitute the original antigenic insult in lupus (which is characterized by a time-dependent diversification of autoreactivity), whether such debris and autoantibodies specifically recognizing its constituents mediate differential effects on innate and humoral responses in lupus-prone mice is currently unknown. Apoptotic blebs (as opposed to cellular lysate) enhanced preferentially the maturation of dendritic cells (DCs) from bone marrow precursors drawn from lupus-prone mice. Murine, somatically mutated, apoptotic cell-reactive immunoglobulin (Ig)G monoclonal antibodies demonstrated enhanced recognition of DCs and also displayed a prominent lupus strain-specific bias in mediating DC maturation. Further, immunization of such antibodies specifically in lupus-prone mice resulted in widespread humoral autoreactivity; hypergammaglobulinaemia (a hallmark of systemic autoimmunity) was observed, accompanied by enhanced antibody titres to cellular moieties. Induced antibodies recognized antigens distinct from those recognized by the antibodies employed for immunization; in particular, nephritis-associated anti-double stranded (ds) DNA antibodies and neonatal lupus-associated anti-Ro60 antibodies were elicited by a non-dsDNA, non-Ro60 reactive antibody, and Sm was a favoured target. Further, only in lupus-prone mice did such immunization enhance the kinetics of humoral anti-self responses, resulting in the advanced onset of glomerulosclerosis. These studies reveal that preferential innate and humoral recognition of the products of cell death in a lupus milieu influence the indices associated with autoimmune pathology.

Elucidation of the potential disease-promoting influence of IgM apoptotic cell-reactive antibodies in lupus.

The undigested remnants of apoptosis are believed to stimulate the generation of autoantibodies in lupus. The biological properties of initiator, disease-specific IgM antibodies that specifically recognize apoptotic cells, readily detected in the sera of lupus patients, remain unclear. Apoptotic cell-reactive IgM monoclonal antibodies (generated from lupus-prone mice), as opposed to control IgM, preferentially stimulated maturation of bone marrow-derived dendritic cells (BMDCs) derived from such mice, relative to BMDCs derived from healthy mice. An influence of both antibody specificity and cell genotype was also apparent in the secretion of signature inflammatory cytokines. Immunization of such antibodies in lupus-prone animals induced increases in total serum IgG levels, with the elicited antibodies also preferentially recognizing moieties on dying cells. An expanded specificity was apparent both upon Western blot on cellular lysate and from the enhanced recognition of dsDNA, Ro60, RNP68 and Sm; the antibody most efficient in mediating autoreactive diversity, while being germline encoded, also induced the highest degree of phenotypic changes on BMDCs. Apoptotic cell-reactive IgM antibodies may therefore be potentially capable of influencing the course of systemic autoimmune disease by affecting both innate and adaptive immunity.

Evaluation of changes in tidal volume during mask ventilation following administration of neuromuscular blocking drugs.

The practice of checking the ability to mask ventilate before administering neuromuscular blocking drugs remains controversial. We prospectively evaluated the changes in the expired tidal volume during pressure-controlled ventilation (two-handed mask ventilation technique) as a surrogate marker to assess the ease of mask ventilation following administration of rocuronium. After informed consent, 125 patients were anaesthetised using a standard induction technique consisting of fentanyl, propofol and rocuronium, with anaesthesia then maintained with isoflurane in oxygen. The mean (SD) expired tidal volume before administration of rocuronium increased by 61 (13) ml at 2 min following onset of neuromuscular block (p < 0.001). This supports the concept that neuromuscular blockade induced by rocuronium facilitates mask ventilation.

Sternal tuberculosis.

Extra-pulmonary tuberculosis constitutes 15-20% of total tuberculosis (TB) case load in immuno-competent patients. Affliction of the skeletal system is rare with still rarer presentation of sternal osteomyelitis even in endemic countries. A patient with primary sternal TB presenting with multiple cutaneous sinuses over the anterior chest wall is being reported. A high element of suspicion is needed more so in resource limited setting for early diagnosis and treatment.

Evolution of interventions for complex coronary lesions.

The exponential growth of percutaneous coronary intervention (PCI) has in large part been due to expansion of the indications to include the procedure in patients with extensive coronary disease, multiple risk factors, older age and comorbidities. Improvement in PCI equipment, development of new interventional techniques, and availability of myocardial and systemic support techniques have all contributed to this growth. With these advances, patients once considered high risk for PCI are no longer considered high risk. This article reviews the complex coronary lesions challenges, various interventional and pharmacologic strategies for optimal results and novel technology.

Absence of histologic evidence of synovitis in patients with Gulf War veterans' illness with joint pain.

OBJECTIVE: An unexplained multisymptom illness, Gulf War veterans' illness (GWVI), has been described among allied force veterans of the first Gulf War (1990-1991). It has been proposed that some of its symptoms reflect an immune dysfunction, and rheumatologic symptoms including joint pain and stiffness are reported frequently. However, it is unknown whether synovial inflammation causes the articular symptoms. We examined synovial tissue from individuals with GWVI and joint pain for evidence of inflammation. METHODS: We compared synovial biopsy samples from 6 individuals with GWVI and joint pain with samples from 9 clinically asymptomatic controls (hematoxylin and eosin [H&E] stains only) and biopsy samples or surgically obtained specimens from 10 patients with rheumatoid arthritis (RA) and 12 with osteoarthritis (OA). Inflammatory changes were quantified in H&E stained sections with a modified synovitis score by immunostaining for CD3, CD20, CD38, CD68, Ki-67, and von Willebrand factor, and with a composite inflammation score based on these markers. RESULTS: Normal histology was seen in the GWVI specimens, except for mild focal lining hyperplasia and rare low-grade perivascular infiltrates in 1 specimen each. Mean +/- SEM synovitis scores were lowest and nearly identical in control (1.38 +/- 0.30) and GWVI specimens (1.41 +/- 0.29), intermediate in OA specimens (2.64 +/- 0.39), and highest in RA specimens (6.0 +/- 0.19). Likewise, inflammatory cells, cell division, vascular density, and composite inflammation score were lowest in the GWVI specimens. CONCLUSION: Despite significant joint pain, the GWVI synovia did not differ from normal controls. These results agree with other studies that have failed to document inflammatory or immunologic etiologies in GWVI.

Cost-effectiveness of Project ADAM: a project to prevent sudden cardiac death in high school students.

Public access defibrillation (PAD) in the adult population is thought to be both efficacious and cost-effective. Similar programs aimed at children and adolescents have not been evaluated for their cost-effectiveness. This study evaluates the potential cost-effectiveness of implementing Project ADAM, a program targeting children and adolescents in high schools in the Milwaukee Public School System. Project ADAM provides education about cardiopulmonary resuscitation (CPR) and the warning signs of sudden cardiac death (SCD) and training in the use and placement of automated external defibrillators (AEDs) in high schools. We developed decision analysis models to evaluate the cost-effectiveness of the decision to implement Project ADAM in public high schools in Milwaukee. We examined clinical model and public policy applications. Data on costs included estimates of hospital-based charges derived from a pediatric medical center where a series of patients were treated for SCD, educational programming, and the direct costs of one AED and training for 15 personnel per school. We performed sensitivity analyses to assess the variation in outputs with respect to changes to input data. The main outcome measures were Life years saved and incremental cost-effectiveness ratios. At an arbitrary societal willingness to pay $100,000 per life year saved, the policy to implement Project ADAM in schools is a cost-effective strategy at a threshold of approximately 5 patients over 5 years for the clinical model and approximately 8 patients over 5 years for the public policy model. Implementation of Project ADAM in high schools in the United States is potentially associated with an incremental cost-effectiveness ratio that is favorable.

Preliminary survey of leptospirosis amongst febrile patients from urban slums of East Delhi.

AIM: To determine the possibility of leptospirosis among patients from urban slums presenting with febrile illness during monsoon and post-monsoon season. METHODS: Evidence of leptospirosis in 180 patients with febrile illness was determined by looking for presence of immunoglobulin M (IgM) antibodies by leptospiral IgM enzyme linked immunosorbent assay (ELISA). The test was carried out on 160 Widal test negative and 20 Widal test positive sera received from febrile patients during June to September 2001. RESULTS: Twenty-seven out of 180 (15%) sera were positive for leptospiral IgM antibodies. CONCLUSIONS: This preliminary survey indicates that leptospirosis could be an important cause of febrile illness in patients from urban slums during monsoon and post-monsoon season.

Measuring the impact of new technology: an outcomes-based approach.

In the current era of decreasing resources, many healthcare organizations are under increasing pressure to evaluate new technology. Advances and innovations in healthcare technology are instrumental in reshaping the healthcare system and in impacting the practice of medicine, including critical care. Healthcare organizations are faced with the following four questions related to adopting a new technology: 1) Should the organization invest in this technology? 2) What are the associated benefits and risks of the capital investment? 3) What is the impact on patient outcomes as a result of adopting the technology? 4) What is the return on investment to the organization? These issues represent challenging areas for the researcher, clinician, manager, and policy maker. New techniques from the business and outcomes research arenas allow for the development of quantitative and qualitative models to facilitate answering these questions. By integrating business and clinical considerations, these models can impact policy at two levels: 1) by identifying optimal capital expenditure decisions for individual healthcare organizations, it can provide a significant competitive advantage for an organization; and 2) by identifying optimal health policies related to technology at a national and international health level, it can impact the healthcare systems and patient outcomes in a favorable manner.

Outcome in children receiving continuous venovenous hemofiltration.

OBJECTIVE: Continuous venovenous hemofiltration (CVVH) alone or with dialysis (D) has become an important supportive therapy for critically ill children with acute renal failure. Previous reports of pediatric patient outcome either mix CVVH/D with other renal replacement modalities or do not examine severity of illness. The current study examines only outcomes of children receiving CVVH/D using Pediatric Risk of Mortality (PRISM) scores to control for severity of illness. PATIENTS: Twenty-one patients (mean age: 8.8 +/- 6.3 years; mean weight: 28.3 +/- 20.8 kg) received 22 courses of CVVH/D. OUTCOMES: Nine (42.8%) of 21 patients survived. Nine (75%) of 12 deaths occurred within 25 days of pediatric intensive care unit (PICU) admission. Mean PRISM score at PICU admission and CVVH initiation were 13.1 +/- 5.8 and 15.4 +/- 8.9, respectively. Mean patient weight, age, PRISM score at PICU admission and at CVVH/D initiation, maximum pressor number, estimated glomerular filtration rate at CVVH/D initiation and change in mean airway pressure did not differ between survivors and nonsurvivors. The degree of fluid overload at CVVH/D initiation was significantly lower in survivors (16.4% +/- 13.8%) compared with nonsurvivors (34.0% +/- 21.0%), even when controlled for severity of illness by PRISM score. Mean cost of providing CVVH/D accounted for only 1% of total PICU cost per patient. CONCLUSIONS: The pattern of early multiorgan system failure and death, minimal relative cost of CVVH/D provision, and potential for improved outcome with initiation of CVVH/D at lesser degrees of fluid overload are factors that may support early initiation of CVVH/D in critically ill children with acute renal failure.

Intensive care--a cost effective option for developing countries?

The estimation of cost-effectiveness of pediatric critical care services is an extremely relevant issue for both developing and industrialized nations. Pediatric critical care is expensive and the long outcomes are still relatively unclear. From the perspective of patients who receive the benefits of these services, there may be little controversy regarding cost-effectiveness. However, the issue becomes very complex when attempts are made to identify which patients will benefit most. This also needs to be considered while developing public policy when decisions for allocation of limited resources need to be made within health care systems, and choices need to be made between the provision of health care and other public services. This article addresses the complex issue of economic evaluations and describes various type of cost analyses. The difference between charges and costs is defined, and a discussion of the measurement of costs and benefits, and their relationship to outcomes research is provided. Although there is not a unique answer to the issue of cost-effectiveness for pediatric critical care services, the available literature particularly from the United States is summarized, and the ethical implications explored.

Multiple aortic thrombi associated with protein C and S deficiency.

We describe a woman with an unusual case of thromboembolism of the mesenteric artery in whom multiple thrombi were subsequently found in the aorta and right heart chambers on transesophageal echocardiography. Further evaluation revealed a deficiency of protein C and S plasma proteins, inhibitors of the clotting system. The patient was treated successfully with systemic anticoagulation. Aortic thrombus is common in the setting of underlying atherosclerosis. However, the association of aortic thrombus with a deficiency of protein C and S is rare. To our knowledge, this is the first reported case of mural thrombus of the thoracic aorta associated with combined protein C and S deficiency. Our report underscores the important role of transesophageal echocardiography in the evaluation of patients with arterial thromboembolism.

Factors affecting longevity of homograft valves used in right ventricular outflow tract reconstruction for congenital heart disease.

BACKGROUND: Few studies have explored the long-term function of cryopreserved homograft valves used for reconstruction of the right ventricular tract (RVOT) in patients with congenital heart disease. METHODS AND RESULTS: Among 205 patients receiving cryopreserved homografts for reconstruction of the RVOT between November 1985 and April 1999, the outcome of 220 homografts in 183 operative survivors was analyzed. There were 150 pulmonary and 70 aortic homografts used. Median age at implantation was 4.4 years (mean 6.9+/-7.6 years, range 3 days to 48 years). End points included (1) patient survival, (2) homograft failure (valve explant or late death), and (3) homograft dysfunction (homograft insufficiency or homograft stenosis). Survival was 88% at 10 years. Freedom from homograft failure was 74+/-4% at 5 years and 54+/-7% at 10 years. Univariable analysis identified younger age, longer donor warm ischemic time, valve Z: value <2, and previous procedure as risk factors for homograft failure and dysfunction. Aortic homograft type and extracardiac operative technique predicted homograft failure but not dysfunction. For patients