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Congenital genitourinary anomalies are among the most frequent types of birth defects in neonates. Some anomalies can be a significant cause of morbidity in infancy, while others remain asymptomatic even until adulthood and can be at times the only manifestation of a complex systemic disease. The spectrum of these anomalies results from the developmental insults that can occur at various embryologic stages, and an understanding of the formation of the genitourinary system is helpful in the evaluation and treatment of a child with a congenital genitourinary anomaly. Imaging plays an essential role in the diagnosis of congenital genitourinary anomalies and treatment planning. In this article, we highlight the embryologic and characteristic imaging features of various congenital genitourinary anomalies, demonstrate the utility of different imaging modalities in management, and review specific imaging modalities and protocols for image optimization.

BACKGROUND When head injured children undergo head computed tomography (CT), radiation dosing can vary considerably between institutions, potentially exposing children to excess radiation, increasing risk for malignancies later in life. We compared radiation delivery from head CTs at a level 1 pediatric trauma center (PTC) versus scans performed at referring adult general hospitals (AGHs). We hypothesized that children at our PTC receive a significantly lower radiation dose than children who underwent CT at AGHs for similar injury profiles. METHODS We retrospectively reviewed the charts of all patients younger than 18 years who underwent CT for head injury at our PTC or at an AGH before transfer between January 1 and December 31, 2019. We analyzed demographic and clinical data. Our primary outcome was head CT radiation dose, as calculated by volumetric CT dose index (CTDIvol) and dose-length product (DLP; the product of CTDIvol and scan length). We used unadjusted bivariate and multivariable linear regression (adjusting for age, weight, sex) to compare doses between Children's Hospital Los Angeles and AGHs. RESULTS Of 429 scans reviewed, 193 were performed at our PTC, while 236 were performed at AGHs. Mean radiation dose administered was significantly lower at our PTC compared with AGHs (CTDIvol 20.3/DLP 408.7 vs. CTDIvol 30.6/DLP 533, p < 0.0001). This was true whether the AGH was a trauma center or not. After adjusting for covariates, findings were similar for both CTDIvol and DLP. Patients who underwent initial CT at an AGH and then underwent a second CT at our PTC received less radiation for the second CT (CTDIvol 25.6 vs. 36.5, p < 0.0001). CONCLUSIONS Head-injured children consistently receive a lower radiation dose when undergoing initial head CT at a PTC compared with AGHs. This provides a basis for programs aimed at establishing protocols to deliver only as much radiation as necessary to children undergoing head CT. LEVEL OF EVIDENCE Care Management/Therapeutic, level IV.

OBJECTIVEAnterior approaches to the lumbar spine provide wide exposure that facilitates placement of large grafts with high fusion rates. There are limited data on the effects of obesity on perioperative complications.METHODSData from consecutive patients undergoing anterior lumbar interbody fusion (ALIF) from 2007 to 2016 at a single academic center were analyzed. The primary outcome was any perioperative complication. Complications were divided into those occurring intraoperatively and those occurring postoperatively. Multivariate logistic regression was used to assess the association of obesity and other variables with these complications. An estimation table was used to identify a body mass index (BMI) threshold associated with increased risk of postoperative complication.RESULTSA total of 938 patients were identified, and the mean age was 57 years; 511 were females (54.5%). The mean BMI was 28.7 kg/m2, with 354 (37.7%) patients classified as obese (BMI ≥ 30 kg/m2). Forty patients (4.3%) underwent a lateral transthoracic approach, while the remaining 898 (95.7%) underwent a transabdominal retroperitoneal approach. Among patients undergoing transabdominal retroperitoneal ALIF, complication rates were higher for obese patients than for nonobese patients (37.0% vs 28.7%, p = 0.010), a difference that was driven primarily by postoperative complications (36.1% vs 26.0%, p = 0.001) rather than intraoperative complications (3.2% vs 4.3%, p = 0.416). Obese patients had higher rates of ileus (11.7% vs 7.2%, p = 0.020), wound complications (11.4% vs 3.4%, p < 0.001), and urinary tract infections (UTI) (5.0% vs 2.5%, p = 0.049). In a multivariate model, age, obesity, and number of ALIF levels fused were associated with an increased risk of postoperative complication. An estimation table including 19 candidate cut-points, odds ratios, and adjusted p values found a BMI ≥ 31 kg/m2 to have the highest association with postoperative complication (p = 0.012).CONCLUSIONSObesity is associated with increased postoperative complications in ALIF, including ileus, wound complications, and UTI. ALIF is a safe and effective procedure. However, patients with a BMI ≥ 31 kg/m2 should be counseled on their increased risks and warrant careful preoperative medical optimization and close monitoring in the postoperative setting.

Summer camps for children living with heart disease can have a profound impact on well-being. However, specialized camps often require extensive resources (i.e., 24-h medical staff supervision) and may be located in far remote settings. Integrating children with heart disease into mainstream day camps may address these barriers. The purpose of this study is to describe the experience of attending an integrated day camp from the perspectives of children with heart disease and their parents.This study used a qualitative descriptive design. Among 25 eligible families, 9 participated in interviews which were held 3 months to 2 years after attending an integrated camp (mean age of children at camp was 7.3 ± 2.25 years). Interviews were audio-recorded and transcribed verbatim for an inductive thematic analysis.Many parents chose the integrated camp as their child's first summer camp experience, citing trust in the local division of Cardiology's approval of the camp activities as an important reason for enrolling. All participants agreed the integrated camp was a valued opportunity which should continue, although not all described positive camp experiences. Participants' descriptions of the integrated camp were organized into two main themes: 1) overall expectations of the camp and 2) important opportunities afforded by the camp experience. Partaking in a typical camp experience, connecting to local children with heart disease, adequate safety precautions and activity adaptations were specific expectations held by participants. Important opportunities included greater independence and confidence, navigating disclosure of their diagnosis to peers on their own terms, and more diverse social connections. Improving communication with parents to ensure expectations match camp objectives would have enhanced the experience.Practitioners looking for an alternative to specialized camps for their patients with heart disease may use these results to guide the design and promotion of an integrated camp.

Penile changes (most prominently shortening) after radical prostatectomy (RP) can negatively influence body image and quality of life. Here, we review the relevance of penile length to sexual satisfaction, the etiology of penile shortening after RP, and interventions that may preserve penile length. Most studies measure flaccid stretched penile length from the penopubic skin junction to the glans tip; however, the technique reportedly underestimates erect length by 23%. There is evidence that oral pharmacotherapy and mechanical devices may provide some benefit for length preservation, but the evidence basis remains marginal. Surgical augmentation in the setting of penile shortening may be efficacious but carries risks including potential for failure and/or worsening deformity. Penile length loss (perceived or objective) can have a major impact on quality of life after radical prostatectomy. Additional research is required to understand optimal means to help men preserve penile length after RP.

BACKGROUND: Cutaneous lumbosacral findings in neonates are common in the newborn nursery but may also be associated with occult spinal dysraphism. Variation in management of lumbosacral findings by neonatal clinicians has not been previously described. METHODS: Clinicians in the Better Outcomes through Research for Newborns (BORN) Network were invited to participate in an electronic survey. Participants reviewed 18 photographs of lumbosacral findings in asymptomatic neonates and selected 1 or more initial management step(s): routine care, watchful waiting, imaging, and/or subspecialty consultation. Additional data collected include ease of access to imaging and subspecialty consultants and characteristics of respondents. RESULTS: Of 407 BORN Network clinicians, 206 (51%) completed the survey. Respondents were in >90% agreement in initial management approach of 8 of 18 cases. The most common initial actions were spinal ultrasound (53%), neurosurgery evaluation (18%), and MRI (13%). Anomalies of the gluteal crease had the lowest proportion of agreement. In 2 cases, there were differences in respondents’ choice to image or consult a subspecialist depending on their percent clinical full time equivalent spent taking care of neonates CONCLUSIONS: Variation in initial management of neonatal lumbosacral findings by clinicians in the BORN Network was seen most often for deviations of the gluteal crease, flat vascular macules, and coccygeal hair.

OBJECTIVEAnterior lumbar interbody fusion (ALIF) is a powerful technique that provides wide access to the disc space and allows for large lordotic grafts. When used with posterior spinal fusion (PSF), the procedures are often staged within the same hospital admission. There are limited data on the perioperative risk profile of ALIF-first versus PSF-first circumferential fusions performed within the same hospital admission. In an effort to understand whether these procedures are associated with different perioperative complication profiles, the authors performed a retrospective review of their institutional experience in adult patients who had undergone circumferential lumbar fusions.METHODSThe electronic medicals records of patients who had undergone ALIF and PSF on separate days within the same hospital admission at a single academic center were retrospectively analyzed. Patients carrying a diagnosis of tumor, infection, or traumatic fracture were excluded. Demographics, surgical characteristics, and perioperative complications were collected and assessed.RESULTSA total of 373 patients, 217 of them women (58.2%), met the inclusion criteria. The mean age of the study cohort was 60 years. Surgical indications were as follows: degenerative disease or spondylolisthesis, 171 (45.8%); adult deformity, 168 (45.0%); and pseudarthrosis, 34 (9.1%). The majority of patients underwent ALIF first (321 [86.1%]) with a mean time of 2.5 days between stages. The mean number of levels fused was 2.1 for ALIF and 6.8 for PSF. In a comparison of ALIF-first to PSF-first cases, there were no major differences in demographics or surgical characteristics. Rates of intraoperative complications including venous injury were not significantly different between the two groups. The rates of postoperative ileus (11.8% vs 5.8%, p = 0.194) and ALIF-related wound complications (9.0% vs 3.8%, p = 0.283) were slightly higher in the ALIF-first group, although the differences did not reach statistical significance. Rates of other perioperative complications were no different.CONCLUSIONSIn patients undergoing staged circumferential fusion with ALIF and PSF, there was no statistically significant difference in the rate of perioperative complications when comparing ALIF-first to PSF-first surgeries.

Objective To evaluate practice variation in pharmacologic management in the International Kawasaki Disease Registry (IKDR). Study design Practice variation in intravenous immunoglobulin (IVIG) therapy, anti-inflammatory agents, statins, beta-blockers, antiplatelet therapy, and anticoagulation was described. Results We included 1627 patients from 30 IKDR centers with maximum coronary artery aneurysm (CAA) z scores 2.5-4.99 in 848, 5.0-9.99 in 349, and ≥10.0 (large/giant) in 430 patients. All centers reported IVIG and acetylsalicylic acid (ASA) as primary therapy and use of additional IVIG or steroids as needed. In 23 out of 30 centers, (77%) infliximab was also used; 11 of these 23 centers reported using it in 20% of patients. Nonsteroidal anti-inflammatory agents were used in >10% of patients in only nine centers. Beta-blocker (8.8%, all patients) and abciximab (3.6%, all patients) were mainly prescribed in patients with large/giant CAAs. Statins (2.7%, all patients) were mostly used in one center and only in patients with large/giant CAAs. ASA was the primary antiplatelet modality for 99% of patients, used in all centers. Clopidogrel (18%, all patients) was used in 24 centers, 11 of which used it in >50% of their patients with large/giant CAAs. Conclusions In the IKDR, IVIG and ASA therapy as primary therapy is universal with common use of a second dose of IVIG for persistent fever. There is practice variation among centers for adjunctive therapies and anticoagulation strategies, likely reflecting ongoing knowledge gaps. Randomized controlled trials nested in a high-quality collaborative registry may be an efficient strategy to reduce practice variation.

Psychosocial health issues are common among children with cardiac diagnoses. Understanding parent and child perceptions is important because parents are the primary health information source. Significant discrepancies have been documented between parent/child quality-of-life data but have not been examined among psychosocial diagnostic instruments. This study examined agreement and discrepancies between parent and child reports of psychosocial health and quality of life in the paediatric cardiology population. Children (n=50, 6–14 years) with diagnoses of CHDs (n=38), arrhythmia (n=5), cardiomyopathy (n=4), or infectious disease affecting the heart (n=3) were enrolled, completing one or more outcome measures. Children and their parents completed self-reports and parent proxy reports of quality of life – Pediatric Quality of Life Inventory – and psychosocial health – Behavioral Assessment Scale for Children (Version 2). Patients also completed the Multidimensional Anxiety Scale for Children. Associations (Pearson’s correlations, Intraclass Correlation Coefficients) and differences (Student’s t-tests) between parent proxy reports and child self-reports were evaluated. Moderate parent–child correlations were found for physical (R=0.33, p=0.03), school (R=0.43, p

Objective Computer-aided auscultation in the differentiation of pathologic (AHA class I) from no or innocent murmurs (AHA class III) would be of great value to the general practitioner. This would allow objective screening for structural heart disease, standardized documentation of auscultation findings, and may avoid unnecessary referrals to pediatric cardiologists. Our goal was to assess the quality of a novel computerized algorithm that automatically classifies murmurs in phonocardiograms (PCGs) acquired in a pediatric population. Design This is a pilot study testing the ability of a novel computerized algorithm to accurately diagnose PCGs compared with interpreted echocardiograms as a gold standard. Setting This study was performed in pediatric cardiology clinics at a tertiary care hospital. Patients All incoming patients were recruited, including patients with no murmurs, innocent murmurs, and pathologic murmurs (106 patients). Intervention Using an electronic stethoscope, PCGs were acquired by the pediatric cardiologist from each patient. The PCGs were analyzed by the algorithm and diagnoses were compared with findings by echocardiograms interpreted by pediatric cardiologists which were used as the gold standard. Outcome Measures Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were calculated. Results When compared with echocardiography as a gold standard in diagnosing murmurs, the computerized algorithm tested on N=34 PCGs, yielded a sensitivity of 87% and specificity of 100%, a positive predictive value of 100%, negative predictive value of 90% and an accuracy of 94%. Conclusion With echocardiogram as a gold standard, this computerized algorithm can detect pathologic murmurs with high sensitivity, specificity and accuracy, comparable to if not better than published results of pediatric cardiologists and neonatologists. This study confirms the high quality and “real-world” robustness of a novel computational algorithm in the assessment of pediatric murmurs.

Background: Electronic consultations (eConsult) allow for communication between primary care providers and specialists in an asynchronous manner. This study examined provider satisfaction, topics of interest, and efficiency of eConsult in pediatric hematology/oncology in Ottawa, Canada. Methods: We conducted a cross-sectional assessment of all eConsult cases directed to pediatric hematology/oncology specialists using the Champlain BASE (Building Access to Specialists through eConsultation) eConsult service from June 1, 2014 to May 31, 2016. Results: There were 1064 eConsults to pediatrics during the study timeperiod and pediatric hematology/oncology consults accounted for 8% (85). During the same study timeperiod, 524 consults were seen in the pediatric hematology/oncology clinic. The majority of the eConsults were for hematology (90.5%) in contrast to oncology topics (9.5%). The most common topics were anemia, hemoglobinopathy, bleeding disorder, and thrombotic state. Primary care providers rated the eConsult service very highly, and their comments were very positive. The eConsult service resulted in deferral of 40% of consults originally contemplated to require a face-to-face specialist visit. Conclusions: This study showed successful implementation and use of the eConsult service for pediatric hematology/oncology and resulted in avoidance of a large number of face-to-face consultation. The common topics identified areas for continuing medical education.

Physical activity is promoted for optimal health but may carry risks for children who require medically necessary activity restrictions. The sensitivity, specificity, and reliability of the Get Active Questionnaire (GAQ) for identifying children needing special considerations during physical activity was evaluated among parents of 207 children aged 3 to 14 years (97 (47%) female, mean age of 8.4 ± 3.7 years). GAQ responses were compared with reports obtained directly from the treating physician (n = 192/207) and information in the medical chart (clinic notes/physician letter, n = 111/207). Parent GAQ responses (either “No to all questions” or “Yes to 1 or more questions”) agreed with physician (κ = 0.16, p = 0.003) and medical record (κ = 0.15, p = 0.003) reports regarding the need for special consideration during physical activity (Yes/No). Sensitivity was 71% (20/28) and specificity was 59% (96/164), with few false-negative responses. The GAQ was most effective for rheumatology and cardiology patients. False positives were 29% to 46%, except among chronic pain (80%) and rehabilitation (75%) patients. Test–retest reliability was moderate (Cronbach’s α = 0.70) among 57 parents who repeated the GAQ 1 week later. The GAQ effectively identified children not requiring physical activity restrictions and those with medical conditions similar to those of concern among adults. Additional questions from a qualified exercise professional, as recommended for a “Yes” response on the GAQ, should reduce the false-positive burden. Indicating the timeframe of reference for each question and including an option to describe other special considerations (e.g., medication, supervision) are recommended.

Background We have previously documented an increase in the incidence of Kawasaki disease (KD) in Ontario followed by a stabilization from 1995 to 2006. We sought to validate the estimation of incidence of KD using administrative data and to describe the epidemiology of KD across Canada from 2004 to 2014. Methods We queried the Canadian Hospital Discharge Database for hospital admissions associated with a discharge diagnosis of KD. The data set was manually curated and estimates of incidence were compared with those obtained from the retrospective triennial surveillances of KD performed in 2007 and 2010. Results The average number of cases per year identified through administrative data was 245 ± 45 vs 229 ± 33 from retrospective surveillance. This overestimation, representing 7 ± 6%, is similar to the historical percentage of patients originally diagnosed with KD in whom the diagnosis is subsequently excluded. The annual incidence of KD in Canada was 19.6, 6.4, and 1.3 cases per 100,000 children younger than 5 years, 5-9 years, and 10-14 years old, respectively, with important regional and seasonal differences. The incidence remained stable over the study period in the youngest age group but increased in both older age categories. Coronary artery aneurysms affected 3.5% of all patients, and 0.8% experienced associated major cardiac complications. Conclusions Reliance on administrative data to determine incidence of KD is feasible and accurate with manual curation of the data. The incidence of KD in Canada seems to have plateaued for younger children. Differences in annual incidence observed between provinces remain to be explained, and might reflect genetic or environmental differences.

Background Champlain BASE™ (Building Access to Specialists through eConsultation) is a web-based asynchronous electronic communication service that allows primary-care- practitioners (PCPs) to submit “elective” clinical questions to a specialist. For adults, PCPs have reported improved access and timeliness to specialist advice, averted face-to-face specialist referrals in up to 40% of cases and high provider satisfaction. Objective To determine whether the expansion of eConsult to a pediatric setting would result in similar measures of improved healthcare system process and high provider acceptance reported in adults. Design Prospective observational cohort study. Setting Single Canadian tertiary-care academic pediatric hospital (June 2014–16) servicing 1.2 million people. Participants 1. PCPs already using eConsult. 2.Volunteer pediatric specialists provided services in addition to their regular workload. 3.Pediatric patients (< 18 years-old) referred for none-acute care conditions. Main outcomes and measures Specialty service utilization and access, impact on PCP course-of-action and referral-patterns and survey-based provider satisfaction data were collected. Results 1064 eConsult requests from 367 PCPs were answered by 23 pediatric specialists representing 14 specialty-services. The top three specialties represented were: General Pediatrics 393 cases (36.9%), Orthopedics 162 (15.2%) and Psychiatry 123 (11.6%). Median specialist response time was 0.9 days (range 93.3%) of PCPs rated eConsult as very good/excellent value for both patients and themselves. All specialist survey-respondents indicated eConsult should be a continued service. Conclusions and relevance Similar to adults, eConsult improves PCP access and timeliness to elective pediatric specialist advice and influences their care decisions, while reporting high end-user satisfaction. Further study is warranted to assess impact on resource utilization and clinical outcomes.

The relationship between parameters of brain functional networks and clinical indexes is unclear so far in patients with diabetic retinopathy (DR). This paper is to investigate this. Twenty-one patients with different grades of DR and 21 age- and sex-matched healthy controls were enrolled from August 2012 to September 2014. The clinical indexes recorded included DR grade, duration of diabetes, HbA1c, diabetic foot screen, fasting plasma glucose, insulin, Homa-β, Homa-IR, insulin sensitive index (ISI), Mini-Mental State Examination (MMSE), and patient sex and age. Subjects were scanned using 3-T MR with blood-oxygen-level-dependent and 3D–FSPGR sequences. MR data was analyzed via preprocessing and functional network construction, and quantified indexes of network (clustering coefficient, characteristic path length, global efficiency, degree distribution, and small worldness) were evaluated. Statistics consisted of ANOVA and correlation. There were significant differences between patients and controls among clustering coefficient, characteristic path length, degree distribution, and small worldness parameters (P

Background: Rising incidences of Kawasaki disease (KD) have been reported worldwide. Reported herein are the results of 4 triennial KD surveillances conducted in Ontario. Methods: Between 1995 and 2006 all hospitals in Ontario were asked on 4 occasions to identify all patients with discharge diagnoses of KD and report incident cases. Results: The latest surveillance identified 697 new KD patients (100% response rate) for a total of 2378 KD patients through all 4 surveillances. Yearly incidence was 26.2/100 000 for

Background: An electrocardiogram has been proposed to screen for prolonged QT interval that may predispose infants to sudden death in the first year of life. Understanding the reliability of QT interval measurement will inform the design of a screening program. Methods: Three pediatric cardiologists measured the QT/RR intervals on 60 infant electrocardiograms (median age 46 days), from leads II, V5 and V6 on three separate occasions, 7 days apart, according to a standard protocol. The QTc was corrected by Bazett's (QTcB), Fridericia's (QTCFrid), and Hodges' (QTcH) formulae. Intraobserver and interobserver reliability were assessed by intraclass correlation coefficients (ICC), limits of agreement and repeatability coefficients for single, average of two and average of three measures. Agreement for QTc prolongation (> 440 msec) was assessed by kappa coefficients. Results: QT interval intraobserver ICC was 0.86 and repeatability coefficient was 25.9 msec; interobserver ICC increased from 0.88 for single observations to 0.94 for the average of 3 measurements and repeatability coefficients decreased from 22.5 to 16.7 msec. For QTcB, intraobserver ICC was 0.67, and repeatability was 39.6 msec. Best interobserver reliability for QTcB was for the average of three measurements (ICC 0.83, reproducibility coefficient 25.8 msec), with further improvement for QTcH (ICC 0.92, reproducibility coefficient 16.69 msec). Maximum interobserver kappa for prolonged QTc was 0.77. Misclassification around specific cut points occurs because of the repeatability coefficients. Conclusions: Uncorrected QT measures are more reliable than QTcB and QTCFrid. An average of three independent measures provides the most reliable QT and QTc measurements, with QTcH better than QTcB.

Objective Previous publications using nonconcurrent series of patients indicate improved survival for patients with hypoplastic left heart syndrome (HLHS) undergoing stage I palliation with a right ventricle to pulmonary artery conduit (NW-RVPA) vs. a modified Blalock-Taussig shunt (NW-BT). We compared postoperative outcomes in a concurrent series of patients with HLHS undergoing an NW-BT procedure vs. NW-RVPA procedure. Design Perioperative data from 66 consecutive patients who underwent NW-BT (n = 37) or NW-RVPA (n = 29) procedures were retrospectively analyzed. Setting Cardiac intensive care unit in a tertiary pediatric hospital. Patients Charts were reviewed for all patients with the diagnosis of HLHS undergoing the NW-BT or NW-RVPA procedure between January 2002 and December 2003. Results Cardiopulmonary bypass time was longer in the NW-BT group than in the NW-RVPA group (152.5 +/- 52.0 vs. 134.5 +/- 36.1 mins; p = .04). Postoperative diastolic pressures were higher and the Pao2 to Fio2 ratio profiles were lower for the NW-RVPA group over the first 72 hrs. Time to sternal closure (2 [1-6] vs. 4 [2-41] days; p = .01), duration of mechanical ventilation (113 [49-386] vs. 136 [84-764] hrs; p = .01), time to establish enteral feeds (4 [2-8] vs. 5 [3-22] days; p = .01), length of intensive care unit stay (11 [7-55] vs. 15 [8-90] days; p = .04), and length of hospital stay (16 [11-67] vs. 27 [12-126] days; p = .01) were shorter in the NW-RVPA group. Postoperative mortality was not significantly different between the NW-RVPA group (7%) and NW-BT group (11%). Conclusion At an experienced institution with low stage I palliation mortality for HLHS, there were no differences in early morbidity and mortality between the NW-RVPA and NW-BT procedures. The primary advantage of the NW-RVPA procedure may be faster recovery following surgery and earlier discharge from the hospital.

Background Modification of the Norwood procedure has been reported to improve immediate postoperative mortality compared with the classic Norwood. Interstage mortality has not been shown to be improved with the modified Norwood probably because of the small number of patients from each institution. The goal of this study was to determine if meta-analysis would provide sufficient data to prove statistical difference in interstage mortality for the modified Norwood procedure. Methods PubMed was searched using six different terms individually for articles from January 2003 to October 2004. Manuscripts that compared the classic to modified Norwood were reviewed. Mantel-Haenszel analysis was used to evaluate the relationship between treatment method and mortality stratified across hospitals. The Breslow-Day procedure tested homogeneity of odds ratio across hospitals. Separate analyses were performed for inpatient and interstage periods. Results A total of 4,545 citations was screened. Five manuscripts met the criteria. Seventy-two patients undergoing classic Norwood and 84 patients undergoing modified Norwood survived to initial hospital discharge. The Breslow-Day statistic supported homogeneity of odds ratios for survival across hospitals (χ 2 = 2.09, df=4, p = 0.72). Odds of interstage death was 11.6 times greater (2.2 to 62.1, 95% CI) for the classic Norwood compared with the modified Norwood procedure. This difference was statistically significant by the Mantel-Haenszel χ 2 (11.0, p = 0.001). The Breslow-Day statistic supported homogeneity of the odds ratios across hospitals (χ 2 = 3.1, df=4, p = 0.53). Conclusions The modified Norwood procedure has a significantly lower interstage mortality compared with the classic Norwood procedure. A large randomized study is needed to determine whether these results remain consistent.

The measurement of hepatitis B virus (HBV) DNA, is important for monitoring and evaluating the efficacy of anti-viral agents in the treatment of patients with chronic hepatitis B. Three different hybridization assays for quantitative measurement of HBV DNA: direct membrane (dot-blot) hybridization, liquid hybridization (Abbott HBV DNA assay) and branched DNA signal amplification assay (Quantiplex, Chiron), were applied to 114 serial serum samples obtained from 13 patients with chronic active hepatitis B who had received ribavirin 600 mg daily for four weeks. Among the three assays, the correlation was found to be highest between Quantiplex and Abbott HBV DNA assay (r = 0.71, p0.01), moderate between Quantiplex and dot-blot hybridization (r = 0.58, p0.01) and lowest between dot-blot hybridization and Abbott HBV DNA assay (r = 0.27, p0.01). Quantiplex detected 107 (94%) of 114 specimens and was the most sensitive assay. All specimens positive by dot-blot hybridization and Abbott HBV DNA assays were detected positive by Quantiplex. The Dot-blot hybridization assay detected all 89 (100%) specimens with a high HBV DNA level (or = 10 million genome equivalent (Meq)/ml by Quantiplex), but detected only 7 (50%) of 14 specimens with a low HBV DNA level (10 Meq/ml). The Abbott HBV DNA assay detected 85 (95%) of 89 specimens with a high HBV DNA level, but detected only 3 (17%) of 18 specimens with a low HBV DNA level. Among 7 negative specimens in the Quantiplex assay, 2 were detected positive by polymerase chain reaction. In conclusion, Quantiplex assay was more sensitive than Abbott HBV DNA assay and dot-blot hybridization assay for quantitative measurement of serum HBV DNA and can be used in the evaluation of the therapeutic drug effect on chronic hepatitis B patients.

Objective To investigate the reporting of the analysis of interobserver and intra-observer variability within clinical research studies from five high-impact cardiology journals published in 2005. Study Design and Setting A cross-sectional study using a combined electronic and manual search identified 180 of 511 eligible articles that reported the assessment of observer variability. Sixty of these were randomly selected for detailed review. Results The proportion of the 60 studies reporting interobserver variability, intra-observer variability, or both were 27%, 17%, and 53%, respectively. The reported methodological design of interobserver and intra-observer analyses included a specific protocol in 42% and 33%, identified observers as independent in 31% and 17%, as blinded in 50% and 31%, and identified a prior statistical plan in only 33% and 36%, respectively. Pearson correlation was the most reported measure for continuous variables, and the methods of Bland and Altman were reported in 15% of interobserver and 14% of intra-observer studies, respectively. For categorical variables, a kappa statistic was reported in 82% and 80%, respectively. Conclusion Reliability assessment is hampered by unclear and incomplete reporting of interobserver and intra-observer analysis. For continuous variables, inappropriate methods were most frequently reported as being done.

Background There are distinct physiologic differences between patients with single-ventricle lesions who have undergone the Norwood procedure with a right ventricle–to–pulmonary artery conduit (NW-RVPA) compared with those patients who have undergone the Norwood operation with a Blalock-Taussig shunt (NW-BTS). We evaluated bidirectional Glenn operation outcomes and compared the two groups to assess whether the type of Norwood operation influenced outcomes. Methods A retrospective chart review compared bidirectional Glenn operation outcomes for children undergoing the Norwood operation with NW-RVPA or NW-BTS at Children's Hospital Boston from January 1, 2002, to December 31, 2003. Results Of 80 patients undergoing the Norwood operation, 56 (NW-BTS, 27 versus NW-RVPA, 29) returned for the bidirectional Glenn operation at our institution. The NW-RVPA group had a lower median age at presentation for bidirectional Glenn (4.5 months versus 5.8 months; p = 0.01), but had better weight gain (20.6 g/day versus 16.5 g/day; p = 0.03) than the NW-BTS group. No interstage deaths occurred in the NW-RVPA group. There were no differences in morbidity or mortality after the BDG between the two groups. Conclusions There were no differences in morbidity and mortality outcomes after the bidirectional Glenn operation between the NW-RVPA and NW-BTS groups. Despite younger age at presentation, the NW-RVPA patients had better growth rate, which may have contributed to the similar postoperative outcomes.

Background: Hemorrhagic shock secondary to trauma is associated with poor survival. The impedance threshold device (ITD) has been shown to improve blood pressure and survival rates in an adult porcine model of hemorrhagic hypovolemia. Pediatric hemodynamics, anatomy, and physiology differ from adults. Evaluation of the ITD has not been previously assessed in a pediatric porcine model of hypovolemia induced by hemorrhage. Objective: To determine whether ITD-assisted breathing, with and without positive end-expiratory pressure, will improve key hemodynamic parameters following hypovolemia induced by hemorrhage in a pediatric porcine model. Methods: Intubated, anesthetized, hemodynamically stable, spontaneously breathing piglets were rapidly bled 40% of their calculated blood volume. Piglets' hemodynamic and intrathoracic pressures were continuously monitored during 10-min normovolemic baseline, bleed to hypotensive baseline, 10-min ITD-assisted breathing, 10 mins without ITD, 10-min ITD-assisted breathing randomized with or without positive end-expiratory pressure (3 cm H 2 O), 10 mins without ITD, reinfusion of shed blood, 10-min baseline following retum to normovolemia. The ITD had an inspiratory cracking pressure of -7 cm H 2 O. Transthoracic echocardiographic parameters were measured at the end of each 10-min period. Results: There was no significant difference in baseline assessments between groups. Systolic blood pressure, cardiac index, and stroke volume index were significantly greater during ITD-assisted breathing. There was a trend toward increased left ventricular end-diastolic dimension during ITD use. Heart rate, systemic vascular resistance index, left ventricular end-systolic dimension, and shortening fraction did not change significantly during ITD-assisted breathing. There was equivalent improvement in systolic blood pressure, cardiac index, and stroke volume index, when the ITD alone and ITD plus positive end-expiratory pressure were used. Conclusions: ITD-assisted breathing significantly augmented systolic blood pressure, cardiac index, and stroke volume index in this pediatric porcine model of hemorrhagic hypovolemia. These effects appear related to increased left ventricular preload and not by increased systemic vascular resistance or heart rate. These beneficial effects of ITD-assisted breathing are not changed by the addition of positive end-expiratory pressures of 3 cm H 2 O.

OBJECTIVES To determine variations in neonatology practice regarding the diagnosis and management of patent ductus arteriosus (PDA) in premature infants. DESIGN Standardized telephone interview of preferences and practices. SUBJECTS Canadian neonatologists in active clinical practice. RESULTS Of 136 eligible Canadian neonatologists, 100 respondents (74%) estimated the proportion of infants with suspected PDA who have echocardiography to be a median of 80% (range 0% to 100%), with considerable variation both within and between centres. Only two centres had written guidelines. More recent medical school graduates were significantly more likely to use echocardiography. Increased use of echocardiography was also significantly related to increased availability. Fluid restriction and indomethacin was used as initial therapy by 89% of respondents, with the indomethacin dose standardized for 83%; surgical ligation was used when indomethacin therapy was contraindicated or had failed. Personal guidelines directed decisions regarding therapy for the majority of neonatologists. CONCLUSIONS Among Canadian neonatologists, there is considerable variation regarding practices related to the diagnosis and management of PDA in premature infants. This variation may potentially affect the cost effectiveness of care for these patients.

Isolated ventricular inversion is a very rare anomaly in which there is atrioventricular (AV) discordance and ventriculararterial (VA) concordance (AVD-VAC). Thus although the aorta arises from the left ventricle and the pulmonary artery (PA) originates from the right ventricle, the systemic and pulmonary circulations are in parallel and not in series. The physiology is that of complete transposition of the great arteries (TGA), but an arterial switch is contraindicated because it would leave the morphologically right ventricle to support systemic circulation. Six different anatomic types of AVD-VAC have been identified (Figure 1). All such patients rely on adequate mixing of pulmonary and systemic circulation through either a patent ductus arteriosus or an intracardiac shunt to survive. These anomalies are one of the few indications for the atrial switch operation in the modern era. Herein we describe the anatomy, corrective operation, and long-term follow-up in these rare patients.