ABSTRACT
BACKGROUND: The Pediatric Cardiac Critical Care Consortium (PC4) is a multi-institutional quality improvement registry focused on the care delivered in the cardiac ICU for patients with CHD and acquired heart disease. To assess data quality, a rigorous procedure of data auditing has been in place since the inception of the consortium. MATERIALS AND METHODS: This report describes the data auditing process and quantifies the audit results for the initial 39 audits that took place after the transition from version one to version two of the registry's database. RESULTS: In total, 2219 total encounters were audited for an average of 57 encounters per site. The overall data accuracy rate across all sites was 99.4%, with a major discrepancy rate of 0.52%. A passing score is based on an overall accuracy of >97% (achieved by all sites) and a major discrepancy rate of <1.5% (achieved by 38 of 39 sites, with 35 of 39 sites having a major discrepancy rate of <1%). Fields with the highest discrepancy rates included arrhythmia type, cardiac arrest count, and current surgical status. CONCLUSIONS: The extensive PC4 auditing process, including initial and routinely scheduled follow-up audits of every participating site, demonstrates an extremely high level of accuracy across a broad array of audited fields and supports the continued use of consortium data to identify best practices in paediatric cardiac critical care.
Subject(s)
Data Accuracy , Quality Improvement , Child , Humans , Registries , Critical Care , Databases, FactualABSTRACT
OBJECTIVES: In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care. DATA SOURCES: The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine." STUDY SELECTION: This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification. DATA EXTRACTION: A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs. DATA SYNTHESIS: Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed. CONCLUSIONS: In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.
Subject(s)
Pediatrics , Physicians , Child , Critical Care , Curriculum , Education, Medical, Graduate , Humans , Infant, Newborn , United StatesABSTRACT
OBJECTIVES: Characterize transport medical control education in Pediatric Critical Care Medicine fellowship. DESIGN: Cross-sectional survey study. SETTING: Pediatric Critical Care Medicine fellowship programs in the United States. SUBJECTS: Pediatric Critical Care Medicine fellowship program directors. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We achieved a 74% (53/72) response rate. A majority of programs (85%) require fellows to serve as transport medical control, usually while carrying out other clinical responsibilities and sometimes without supervision. Fellows at most programs (80%) also accompany the transport team on patient retrievals. Most respondents (72%) reported formalized transport medical control teaching, primarily in a didactic format (76%). Few programs (25%) use a standardized assessment tool. Transport medical control was identified as requiring all six Accreditation Council for Graduate Medical Education competencies, with emphasis on professionalism and interpersonal and communication skills. CONCLUSIONS: Transport medical control responsibilities are common for Pediatric Critical Care Medicine fellows, but training is inconsistent, assessment is not standardized, and supervision may be lacking. Fellow performance in transport medical control may help inform assessment in multiple domains of competencies. Further study is needed to identify effective methods for transport medical control education.
Subject(s)
Curriculum , Fellowships and Scholarships , Child , Critical Care , Cross-Sectional Studies , Humans , Needs Assessment , United StatesABSTRACT
OBJECTIVE: To describe the similarities and differences in the evaluation and treatment of multisystem inflammatory syndrome in children (MIS-C) at hospitals in the US. STUDY DESIGN: We conducted a cross-sectional survey from June 16 to July 16, 2020, of US children's hospitals regarding protocols for management of patients with MIS-C. Elements included characteristics of the hospital, clinical definition of MIS-C, evaluation, treatment, and follow-up. We summarized key findings and compared results from centers in which >5 patients had been treated vs those in which ≤5 patients had been treated. RESULTS: In all, 40 centers of varying size and experience with MIS-C participated in this protocol survey. Overall, 21 of 40 centers required only 1 day of fever for MIS-C to be considered. In the evaluation of patients, there was often a tiered approach. Intravenous immunoglobulin was the most widely recommended medication to treat MIS-C (98% of centers). Corticosteroids were listed in 93% of protocols primarily for moderate or severe cases. Aspirin was commonly recommended for mild cases, whereas heparin or low molecular weight heparin were to be used primarily in severe cases. In severe cases, anakinra and vasopressors frequently were recommended; 39 of 40 centers recommended follow-up with cardiology. There were similar findings between centers in which >5 patients vs ≤5 patients had been managed. Supplemental materials containing hospital protocols are provided. CONCLUSIONS: There are many similarities yet key differences between hospital protocols for MIS-C. These findings can help healthcare providers learn from others regarding options for managing MIS-C.
Subject(s)
COVID-19/therapy , Clinical Protocols , Practice Patterns, Physicians'/statistics & numerical data , Systemic Inflammatory Response Syndrome/therapy , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Anticoagulants/therapeutic use , Antirheumatic Agents/therapeutic use , Aspirin/therapeutic use , COVID-19/diagnosis , Child , Cross-Sectional Studies , Glucocorticoids/therapeutic use , Heparin/therapeutic use , Hospitals , Humans , Immunoglobulins, Intravenous , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Surveys and Questionnaires , Systemic Inflammatory Response Syndrome/diagnosis , United States/epidemiology , Vasoconstrictor Agents/therapeutic useABSTRACT
BACKGROUND: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes coronavirus disease 2019 (COVID-19), an entity in children initially characterized by milder case presentations and better prognoses as compared with adults. Recent reports, however, raise concern for a new hyperinflammatory entity in a subset of pediatric COVID-19 patients. METHODS: We report a fatal case of confirmed COVID-19 with hyperinflammatory features concerning for both multi-inflammatory syndrome in children (MIS-C) and primary COVID-19. RESULTS: This case highlights the ambiguity in distinguishing between these two entities in a subset of pediatric patients with COVID-19-related disease and the rapid decompensation these patients may experience. CONCLUSIONS: Appropriate clinical suspicion is necessary for both acute disease and MIS-C. SARS-CoV-2 serologic tests obtained early in the diagnostic process may help to narrow down the differential but does not distinguish between acute COVID-19 and MIS-C. Better understanding of the hyperinflammatory changes associated with MIS-C and acute COVID-19 in children will help delineate the roles for therapies, particularly if there is a hybrid phenotype occurring in adolescents.
Subject(s)
COVID-19/complications , COVID-19/physiopathology , Myocarditis/complications , Myocarditis/physiopathology , Adolescent , Black or African American , COVID-19/diagnosis , COVID-19/pathology , Female , Humans , Intensive Care Units , Myocarditis/diagnosis , Myocarditis/pathology , SARS-CoV-2/isolation & purification , Systemic Inflammatory Response SyndromeABSTRACT
OBJECTIVES: To describe the practice analysis undertaken by a task force convened by the American Board of Pediatrics Pediatric Critical Care Medicine Sub-board to create a comprehensive document to guide learning and assessment within Pediatric Critical Care Medicine. DESIGN: An in-depth practice analysis with a mixed-methods design involving a descriptive review of practice, a modified Delphi process, and a survey. SETTING: Not applicable. SUBJECTS: Seventy-five Pediatric Critical Care Medicine program directors and 2,535 American Board of Pediatrics Pediatric Critical Care Medicine diplomates. INTERVENTIONS: A practice analysis document, which identifies the full breadth of knowledge and skill required for the practice of Pediatric Critical Care Medicine, was developed by a task force made up of seven pediatric intensivists and a psychometrician. The document was circulated to all 75 Pediatric Critical Care Medicine fellowship program directors for review and comment and their feedback informed modifications to the draft document. Concurrently, data from creation of the practice analysis draft document were also used to update the Pediatric Critical Care Medicine, was developed by a task force made up of seven pediatric intensivists and a psychometrician. The document was circulated to all 75 Pediatrics Pediatric Critical Care Medicine fellowship program directors for review and comment and their feedback informed modifications to the draft document. Concurrently, data from creation of the practice analysis draft document were also used to update the Pediatric Critical Care Medicine content outline, which was sent to all 2,535 American Board of Pediatrics Pediatric Critical Care Medicine diplomates for review during an open-comment period between January 2019 and February 2019, and diplomate feedback was used to make updates to both the content outline and the practice analysis document. MEASUREMENTS AND MAIN RESULTS: After review and comment by 25 Pediatric Critical Care Medicine program directors (33.3%) and 619 board-certified diplomates (24.4%), a comprehensive practice analysis document was created through a two-stage process. The final practice analysis includes 10 performance domains which parallel previously published Entrustable Professional Activities in Pediatric Critical Care Medicine. These performance domains are made up of between three and eight specific tasks, with each task including the critical knowledge and skills that are necessary for successful completion. The final practice analysis document was also used by the American Board of Pediatrics Pediatric Critical Care Medicine Sub-board to update the Pediatric Critical Care Medicine content outline. CONCLUSIONS: A systematic approach to practice analysis, with stakeholder engagement, is essential for an accurate definition of Pediatric Critical Care Medicine practice in its totality. This collaborative process resulted in a dynamic document useful in guiding curriculum development for training programs, maintenance of certification, and lifetime professional development to enable safe and efficient patient care.
Subject(s)
Fellowships and Scholarships , Medicine , Certification , Child , Critical Care , Humans , Surveys and Questionnaires , United StatesABSTRACT
OBJECTIVES: To ascertain the national experience regarding which physician trainees are allowed to participate in pediatric interfacility transports and what is considered adequate education and training for physician trainees prior to participating in the transport of children. DESIGN: Self-administered electronic survey. SETTING: Pediatric transport teams listed with the American Academy of Pediatrics Section on Transport Medicine. SUBJECTS: Leaders of U.S. pediatric transport teams. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Forty-four of the 90 U.S. teams surveyed (49%) responded. Thirty-nine (89%) were university hospital-affiliated. Most programs (26/43, 60%) allowed trainees to participate in pediatric transport in some capacity. Mandatory transport rotations were reported for pediatric critical care (PICU) fellows (9/42, 21%), neonatology (neonatal ICU) fellows (6/42, 14%), pediatric emergency medicine fellows (4/41, 10%), emergency medicine residents (3/43, 7%), and pediatric residents (2/43, 5%). Fellow participation was reported by 19 of 28 programs (68%) with PICU fellowships, 12 of 25 programs (48%) with pediatric emergency medicine fellowships, and 10 of 34 programs (29%) with neonatal ICU fellowships. Transport programs with greater than or equal to 1,000 annual incoming transports were more likely to include PICU and pediatric emergency medicine fellows as providers (p = 0.04; 95% CI, 1.04-25.71 and p = 0.02; 95% CI, 1.31-53.75). Most commonly, trainees functioned as medical control physicians (86%), provided minute-to-minute medical direction for critically ill patients (62%), performed intubations (52%), and were code leaders for patients undergoing cardiopulmonary resuscitation during transport (52%). Most transport programs required pediatric residents, PICU, and pediatric emergency medicine fellows to complete a PICU rotation prior to participating in pediatric transports. The majority of transport programs did not use any metrics to determine airway proficiency of physician trainees. CONCLUSIONS: There is heterogeneity with regard to the types of physician trainees allowed to participate in pediatric interfacility transports, the roles played by physician trainees during pediatric transport, and the training (or lack thereof) provided to physician trainees prior to their participating in pediatric transports.
Subject(s)
Education, Medical, Graduate , Pediatrics , Transportation of Patients , Cardiopulmonary Resuscitation , Child , Fellowships and Scholarships , Humans , Infant , Intensive Care Units, Pediatric , Internship and Residency , Physicians , Surveys and QuestionnairesABSTRACT
OBJECTIVES: To describe the current approach to initial training, ongoing skill maintenance, and assessment of competence in central venous catheter placement by pediatric critical care medicine fellows, a subset of trainees in whom this skill is required. DESIGN: Cross-sectional internet-based survey with deliberate sampling. SETTING: United States pediatric critical care medicine fellowship programs. SUBJECTS: Pediatric critical care medicine program directors of Accreditation Council for Graduate Medical Education-accredited fellowship programs. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A working group of the Education in Pediatric Intensive Care Investigators research collaborative conducted a national study to assess the degree of standardization of training and competence assessment of central venous catheter placement across pediatric critical care medicine fellowship programs. After piloting, the survey was sent to all program directors (n = 67) of Accreditation Council for Graduate Medical Education-accredited pediatric critical care medicine programs between July 2017 and September 2017. The response rate was 85% (57/67). Although 98% of programs provide formalized central venous catheter placement training for first-year fellows, only 42% of programs provide ongoing maintenance training as part of fellowship. Over half (55%) of programs use a global assessment tool and 33% use a checklist-based tool when evaluating fellow central venous catheter placement competence under direct supervision. Only two programs (4%) currently use an assessment tool previously published and validated by the Education in Pediatric Intensive Care group. A majority (82%) of responding program directors believe that a standardized approach to assessment of central venous catheter competency across programs is important. CONCLUSIONS: Despite national mandates for skill competence by many accrediting bodies, no standardized system currently exists across programs for assessing central venous catheter placement. Most pediatric critical care medicine programs use a global assessment and decisions around the ability of a fellow to place a central venous catheter under indirect supervision are largely based upon subjective assessment of performance. Further investigation is needed to determine if this finding is consistent in other specialties/subspecialties, if utilization of standardized assessment methods can improve program directors' abilities to ensure trainee competence in central venous catheter insertion in the setting of variable training approaches, and if these findings are consistent with other procedures across critical care medicine training programs, adult and pediatric.
Subject(s)
Catheterization, Central Venous/methods , Central Venous Catheters , Fellowships and Scholarships/organization & administration , Pulmonary Medicine/education , Attitude of Health Personnel , Child , Clinical Competence , Cross-Sectional Studies , Curriculum , Humans , United StatesABSTRACT
OBJECTIVE: To develop a postoperative mortality case-mix adjustment model to facilitate assessment of cardiac ICU quality of care, and to describe variation in adjusted cardiac ICU mortality across hospitals within the Pediatric Cardiac Critical Care Consortium. DESIGN: Observational analysis. SETTING: Multicenter Pediatric Cardiac Critical Care Consortium clinical registry. PARTICIPANTS: All surgical cardiac ICU admissions between August 2014 and May 2016. The analysis included 8,543 admissions from 23 dedicated cardiac ICUs. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We developed a novel case-mix adjustment model to measure postoperative cardiac ICU mortality after congenital heart surgery. Multivariable logistic regression was performed to assess preoperative, intraoperative, and immediate postoperative severity of illness variables as candidate predictors. We used generalized estimating equations to account for clustering of patients within hospital and obtain robust SEs. Bootstrap resampling (1,000 samples) was used to derive bias-corrected 95% CIs around each predictor and validate the model. The final model was used to calculate expected mortality at each hospital. We calculated a standardized mortality ratio (observed-to-expected mortality) for each hospital and derived 95% CIs around the standardized mortality ratio estimate. Hospital standardized mortality ratio was considered a statistically significant outlier if the 95% CI did not include 1. Significant preoperative predictors of mortality in the final model included age, chromosomal abnormality/syndrome, previous cardiac surgeries, preoperative mechanical ventilation, and surgical complexity. Significant early postoperative risk factors included open sternum, mechanical ventilation, maximum vasoactive inotropic score, and extracorporeal membrane oxygenation. The model demonstrated excellent discrimination (C statistic, 0.92) and adequate calibration. Comparison across Pediatric Cardiac Critical Care Consortium hospitals revealed five-fold difference in standardized mortality ratio (0.4-1.9). Two hospitals had significantly better-than-expected and two had significantly worse-than-expected mortality. CONCLUSIONS: For the first time, we have demonstrated that variation in mortality as a quality metric exists across dedicated cardiac ICUs. These findings can guide efforts to reduce mortality after cardiac surgery.
Subject(s)
Cardiac Surgical Procedures/mortality , Heart Defects, Congenital/surgery , Hospital Mortality , Intensive Care Units, Pediatric/statistics & numerical data , Risk Adjustment/methods , Age Factors , Child, Preschool , Critical Care , Extracorporeal Membrane Oxygenation/statistics & numerical data , Female , Humans , Infant , Infant, Newborn , Logistic Models , Male , Respiration, Artificial/statistics & numerical data , Retrospective Studies , Risk Factors , Severity of Illness IndexABSTRACT
As the acuity and complexity of pediatric patients with congenital cardiac disease have increased, there are many noncardiac issues that may be present in these patients. These noncardiac problems may affect clinical outcomes in the cardiac intensive care unit and must be recognized and managed. The Pediatric Cardiac Intensive Care Society sought to provide an expert review of some of the most common challenges of the respiratory, gastrointestinal, hematological, renal, and endocrine systems in pediatric cardiac patients. This review provides a brief overview of literature available and common practices.
Subject(s)
Coronary Care Units/organization & administration , Disease Management , Heart Defects, Congenital/therapy , Intensive Care Units, Pediatric/organization & administration , Child , HumansABSTRACT
The determination of fluid responsiveness in the critically ill child is of vital importance, more so as fluid overload becomes increasingly associated with worse outcomes. Dynamic markers of volume responsiveness have shown some promise in the pediatric population, but more research is needed before they can be adopted for widespread use. Our aim was to investigate effectiveness of respiratory variation in peak aortic velocity and pulse pressure variation to predict fluid responsiveness, and determine their optimal cutoff values. We performed a prospective, observational study at a single tertiary care pediatric center. Twenty-one children with normal cardiorespiratory status undergoing general anesthesia for neurosurgery were enrolled. Respiratory variation in peak aortic velocity (ΔVpeak ao) was measured both before and after volume expansion using a bedside ultrasound device. Pulse pressure variation (PPV) value was obtained from the bedside monitor. All patients received a 10 ml/kg fluid bolus as volume expansion, and were qualified as responders if stroke volume increased >15% as a result. Utility of ΔVpeak ao and PPV and to predict responsiveness to volume expansion was investigated. A baseline ΔVpeak ao value of greater than or equal to 12.3% best predicted a positive response to volume expansion, with a sensitivity of 77%, specificity of 89% and area under receiver operating characteristic curve of 0.90. PPV failed to demonstrate utility in this patient population. Respiratory variation in peak aortic velocity is a promising marker for optimization of perioperative fluid therapy in the pediatric population and can be accurately measured using bedside ultrasonography. More research is needed to evaluate the lack of effectiveness of pulse pressure variation for this purpose.
Subject(s)
Anesthesia, General/methods , Aorta/physiology , Blood Flow Velocity , Neurosurgical Procedures , Respiration, Artificial , Adolescent , Blood Pressure , Child , Child, Preschool , Critical Illness , Echocardiography , Hemodynamics , Humans , Infant , Monitoring, Intraoperative/methods , Pediatrics/methods , Predictive Value of Tests , Prospective Studies , ROC Curve , Respiration , Sensitivity and Specificity , Signal Processing, Computer-Assisted , Tertiary Care CentersABSTRACT
OBJECTIVE: To understand sustainability and assure long-term gains in multidisciplinary performance improvement using an operating room to cardiac ICU handoff process focused on creation of a shared mental model. DESIGN: Performance improvement cohort project with pre- and postintervention assessments spanning a 4-year period. SETTING: Twenty-six bed pediatric cardiac ICU in a tertiary care children's hospital. PATIENTS: Cardiac surgery patients admitted to cardiac ICU from the operating room following cardiac surgery. INTERVENTIONS: An interdisciplinary workgroup overhauled our handoff process in 2010. The new algorithm emphasized role delineation, standardized communication, and creation of a shared mental model. Our "I-5" mnemonic allowed validation and verification of a shared mental model between multidisciplinary teams. Staff orientation and practice guidelines were revised to incorporate the new process, visual aids were distributed and posted at each patient's bedside, and lapses/audit data were discussed in multidisciplinary forum. MEASUREMENTS AND MAIN RESULTS: Audits assessing equipment and information transfer during handoff were performed 8 weeks following implementation (n = 29), repeated at 1 year (n = 37), 3 years (n = 15), and 4 years (n = 50). Staff surveys prior to implementation, at 8 weeks, and 4 years postintervention assessed satisfaction. Comprehensiveness of information transfer improved in the 4 years following implementation, and staff satisfaction was maintained. At 4 years, discussion of all elements of information transfer was 94%, increased from 85% 8 weeks following implementation and discussion of four or more information elements was 100% increased from 93%. Of the 73% of staff who completed the survey at 4 years, 91% agreed that they received all necessary information, and 87% agreed that the handoff resulted in a shared mental model. CONCLUSIONS: Our methods were effective in creating and sustaining high levels of staff communication and adherence to the new process, thus achieving sustainable gains. Performance improvement initiatives require proactive interdisciplinary maintenance to be successful long term.
Subject(s)
Intensive Care Units, Pediatric , Models, Psychological , Operating Rooms , Patient Care Team/standards , Patient Handoff/standards , Quality Improvement/organization & administration , Algorithms , Communication , Hospitals, Pediatric , Humans , Interprofessional Relations , Outcome and Process Assessment, Health Care , Patient Care Team/organization & administration , Patient Handoff/organization & administration , Program Evaluation , Quality Assurance, Health Care/organization & administration , Tertiary Care CentersABSTRACT
BACKGROUND: Pediatric critical care medicine requires the acquisition of procedural skills, but to date no criteria exist for assessing trainee competence in central venous catheter (CVC) insertion. OBJECTIVE: The goal of this study was to create and demonstrate validity evidence for a direct observation tool for assessing CVC insertion. METHODS: Ten experts used the modified Delphi technique to create a 15-item direct observation tool to assess 5 scripted and filmed simulated scenarios of CVC placement. The scenarios were hosted on a dedicated website from March to May 2013, and respondents recruited by e-mail completed the observation tool in real time while watching the scenarios. The goal was to obtain 50 respondents and a total of 250 scenario ratings. RESULTS: A total of 49 pediatrics intensive care faculty physicians (6.3% of 780 potential subjects) responded and generated 188 scenario observations. Of these, 150 (79.8%) were recorded from participants who scored 4 or more on the 5 scenarios. The tool correctly identified the expected reference standard in 96.8% of assessments with an interrater agreement kappa (standard error) = 0.94 (0.07) and receiver operating characteristic = 0.97 (95% CI 0.94-0.99). CONCLUSIONS: This direct observation assessment tool for central venous catheterization demonstrates excellent performance in identifying the reference standard with a high degree of interrater reliability. These assessments support a validity construct for a pediatric critical care medicine faculty member to assess a provider placing a CVC in a pediatrics patient.
Subject(s)
Catheterization, Central Venous/standards , Clinical Competence/standards , Adolescent , Child , Child, Preschool , Critical Care , Delphi Technique , Educational Measurement/methods , Humans , Infant , Pediatrics/education , Reproducibility of Results , Video RecordingABSTRACT
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is a lethal lung developmental disorder caused by heterozygous point mutations or genomic deletion copy-number variants (CNVs) of FOXF1 or its upstream enhancer involving fetal lung-expressed long noncoding RNA genes LINC01081 and LINC01082. Using custom-designed array comparative genomic hybridization, Sanger sequencing, whole exome sequencing (WES), and bioinformatic analyses, we studied 22 new unrelated families (20 postnatal and two prenatal) with clinically diagnosed ACDMPV. We describe novel deletion CNVs at the FOXF1 locus in 13 unrelated ACDMPV patients. Together with the previously reported cases, all 31 genomic deletions in 16q24.1, pathogenic for ACDMPV, for which parental origin was determined, arose de novo with 30 of them occurring on the maternally inherited chromosome 16, strongly implicating genomic imprinting of the FOXF1 locus in human lungs. Surprisingly, we have also identified four ACDMPV families with the pathogenic variants in the FOXF1 locus that arose on paternal chromosome 16. Interestingly, a combination of the severe cardiac defects, including hypoplastic left heart, and single umbilical artery were observed only in children with deletion CNVs involving FOXF1 and its upstream enhancer. Our data demonstrate that genomic imprinting at 16q24.1 plays an important role in variable ACDMPV manifestation likely through long-range regulation of FOXF1 expression, and may be also responsible for key phenotypic features of maternal uniparental disomy 16. Moreover, in one family, WES revealed a de novo missense variant in ESRP1, potentially implicating FGF signaling in the etiology of ACDMPV.
Subject(s)
Genome, Human , Genomic Imprinting , Persistent Fetal Circulation Syndrome/pathology , Pulmonary Alveoli/abnormalities , Pulmonary Veins/pathology , Chromosomes, Human, Pair 16/genetics , Comparative Genomic Hybridization , Female , Forkhead Transcription Factors/genetics , Genes, Lethal , High-Throughput Nucleotide Sequencing , Humans , Infant, Newborn , Male , Pedigree , Persistent Fetal Circulation Syndrome/genetics , Pulmonary Alveoli/pathology , Sequence DeletionABSTRACT
BACKGROUND: The development of low cardiac output syndrome (LCOS) after cardiopulmonary bypass (CPB) occurs in up to 25% of neonates and is associated with increased morbidity. Invasive cardiac output monitors such as pulmonary artery catheters have limited availability and are costly. Near-infrared spectroscopy (NIRS) is a noninvasive tool for monitoring regional oxygenation in neonates in the cardiac intensive care unit (CICU). We hypothesize that anterior abdominal NIRS may aid in the early identification of LCOS after cardiac surgery. METHODS: Prospective observational study from October 2013 to October 2014 of all neonates with congenital heart disease admitted to the CICU following CPB. Abdominal NIRS values were continuously recorded upon CICU admission and for the subsequent 24-hour period. The primary outcome was the development of LCOS. Low cardiac output syndrome was defined as the presence of metabolic lactic acidosis (pH < 7.3 and lactate > 4) or addition of a new vasoactive agent or a vasoactive inotropic score > 15. Autoregressive time series models were constructed for each patient based on the continuously recorded NIRS values, and patients were stratified by development of LCOS. RESULTS: Twenty-seven neonates met inclusion criteria, of whom 11 developed LCOS. Neonates who developed LCOS had lower constant NIRS values (49% vs. 66%, P < .001). Constant NIRS values less than 58% best predicted development of LCOS with a sensitivity of 100% and specificity of 69%. CONCLUSION: Lower constant anterior abdominal NIRS values in the early postoperative period may allow early identification of neonates at risk for LCOS.
Subject(s)
Abdomen/blood supply , Cardiac Output, Low/diagnosis , Cardiac Surgical Procedures , Cardiopulmonary Bypass , Heart Defects, Congenital/surgery , Postoperative Complications/diagnosis , Regional Blood Flow , Spectroscopy, Near-Infrared/methods , Acidosis, Lactic/complications , Cardiac Output, Low/complications , Female , Humans , Infant, Newborn , Intensive Care Units , Male , Oxygen , Prospective Studies , ROC CurveABSTRACT
Introduction. Human metapneumovirus (HMPV) is a paramyxovirus from the same subfamily as respiratory syncytial virus (RSV) and causes similar acute lower respiratory tract infection. Albuterol in the setting of acute RSV infection is controversial and has not yet been studied in HMPV. We sought to determine the frequency of albuterol use in HMPV infection and the association between albuterol administration and patient outcomes. Methods. We conducted a retrospective cohort study identifying all patients hospitalized in a tertiary care children's hospital with laboratory-confirmed HMPV infection between January 2010 and December 2010. Results. There were 207 patients included in the study; 57% had a chronic medical condition. The median hospital length of stay was 3 days. Only 31% of patients in the study had a documented wheezing history, while 69% of patients received at least one albuterol treatment. There was no difference in length of stay between patients who received albuterol and those who did not. Conclusion. There is a high frequency of albuterol use in children hospitalized with HMPV infection. As with RSV, evidence may not support routine use of bronchodilators in patients with acute HMPV respiratory infection. Research involving additional patient outcomes and illness severity indicators would be useful in future studies.
ABSTRACT
BACKGROUND: Clinical databases in congenital and paediatric cardiac care provide a foundation for quality improvement, research, policy evaluations and public reporting. Structured audits verifying data integrity allow database users to be confident in these endeavours. We report on the initial audit of the Pediatric Cardiac Critical Care Consortium (PC4) clinical registry. Materials and methods Participants reviewed the entire registry to determine key fields for audit, and defined major and minor discrepancies for the audited variables. In-person audits at the eight initial participating centres were conducted during a 12-month period. The data coordinating centre randomly selected intensive care encounters for review at each site. The audit consisted of source data verification and blinded chart abstraction, comparing findings by the auditors with those entered in the database. We also assessed completeness and timeliness of case submission. Quantitative evaluation of completeness, accuracy, and timeliness of case submission is reported. RESULTS: We audited 434 encounters and 29,476 data fields. The aggregate overall accuracy was 99.1%, and the major discrepancy rate was 0.62%. Across hospitals, the overall accuracy ranged from 96.3 to 99.5%, and the major discrepancy rate ranged from 0.3 to 0.9%; seven of the eight hospitals submitted >90% of cases within 1 month of hospital discharge. There was no evidence for selective case omission. CONCLUSIONS: Based on a rigorous audit process, data submitted to the PC4 clinical registry appear complete, accurate, and timely. The collaborative will maintain ongoing efforts to verify the integrity of the data to promote science that advances quality improvement efforts.
Subject(s)
Cardiology , Clinical Audit , Data Accuracy , Databases, Factual/standards , Pediatrics , Registries/standards , Cooperative Behavior , Critical Care/standards , Hospitals/statistics & numerical data , Quality Improvement , United StatesABSTRACT
OBJECTIVES: To describe the teaching and evaluation modalities used by pediatric critical care medicine training programs in the areas of professionalism and communication. DESIGN: Cross-sectional national survey. SETTING: Pediatric critical care medicine fellowship programs. SUBJECTS: Pediatric critical care medicine program directors. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Survey response rate was 67% of program directors in the United States, representing educators for 73% of current pediatric critical care medicine fellows. Respondents had a median of 4 years experience, with a median of seven fellows and 12 teaching faculty in their program. Faculty role modeling or direct observation with feedback were the most common modalities used to teach communication. However, six of the eight (75%) required elements of communication evaluated were not specifically taught by all programs. Faculty role modeling was the most commonly used technique to teach professionalism in 44% of the content areas evaluated, and didactics was the technique used in 44% of other professionalism content areas. Thirteen of the 16 required elements of professionalism (81%) were not taught by all programs. Evaluations by members of the healthcare team were used for assessment for both competencies. The use of a specific teaching technique was not related to program size, program director experience, or training in medical education. CONCLUSIONS: A wide range of techniques are currently used within pediatric critical care medicine to teach communication and professionalism, but there are a number of required elements that are not specifically taught by fellowship programs. These areas of deficiency represent opportunities for future investigation and improved education in the important competencies of communication and professionalism.