Assessing enrollment of eligible infants in the national pediatric cardiology quality improvement collaborative (NPC-QIC) through linkage to the pediatric cardiac critical care consortium (PC4) registry.

BACKGROUND: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment. METHODS: We used the Pediatric Cardiac Critical Care Consortium (PC4) registry to assess enrollment rates in NPC-QIC for those centers participating in both registries using indirect identifiers (date of birth, date of admission, gender, and center) to match patient records. All infants born 1/1/2018-12/31/2020 and admitted 30 days of life were eligible. In PC4, all infants with a fundamental diagnosis of hypoplastic left heart or variant or who underwent a surgical or hybrid Norwood or variant were eligible. Standard descriptive statistics were used to describe the cohort and center match rates were plotted on a funnel chart. RESULTS: Of 898 eligible NPC-QIC patients, 841 were linked to 1,114 eligible PC4 patients (match rate 75.5%) in 32 centers. Match rates were lower in patients of Hispanic/Latino ethnicity (66.1%, p = 0.005), and those with any specified chromosomal abnormality (57.4%, p = 0.002), noncardiac abnormality (67.8%, p = 0.005), or any specified syndrome (66.5%, p = 0.001). Match rates were lower for patients who transferred to another hospital or died prior to discharge. Match rates varied from 0 to 100% across centers. CONCLUSIONS: It is feasible to match patients between the NPC-QIC and PC4 registries. Variation in match rates suggests opportunities for improvement in NPC-QIC patient enrollment.

Sustained radiation reduction following initial quality improvement intervention in a paediatric cardiac catheterisation laboratory.

BACKGROUND: As part of a quality improvement project beginning in October 2011, our centre introduced changes to reduce radiation exposure during paediatric cardiac catheterisations. This led to significant initial decreases in radiation to patients. Starting in April 2016, we sought to determine whether these initial reductions were sustained. METHODS: After a 30-day trial period, we implemented (1) weight-based reductions in preset frame rates for fluoroscopy and angiography, (2) increased use of collimators and safety shields, (3) utilisation of stored fluoroscopy and virtual magnification, and (4) hiring of a devoted radiation technician. We collected patient weight (kg), total fluoroscopy time (min), and procedure radiation dosage (cGy-cm2) for cardiac catheterisations between October, 2011 and September, 2019. RESULTS: A total of 1889 procedures were evaluated (196 pre-intervention, 303 in the post-intervention time period, and 1400 in the long-term group). Fluoroscopy times (18.3 ± 13.6 pre; 19.8 ± 14.1 post; 17.11 ± 15.06 long-term, p = 0.782) were not significantly different between the three groups. Patient mean radiation dose per kilogram decreased significantly after the initial quality improvement intervention (39.7% reduction, p = 0.039) and was sustained over the long term (p = 0.043). Provider radiation exposure was also significantly decreased from the onset of this project through the long-term period (overall decrease of 73%, p < 0.01) despite several changes in the interventional cardiologists who made up the team over this time period. CONCLUSION: Introduction of technical and clinical practice changes can result in a significant reduction in radiation exposure for patients and providers in a paediatric cardiac catheterisation laboratory. These reductions can be maintained over the long term.

A comparison of Kawasaki Disease and multisystem inflammatory syndrome in children.

Background: Due to the COVID-19 pandemic a novel disease has emerged, multisystem inflammatory syndrome in children (MIS-C). It presents post virally after a COVID-19 infection, and its clinical presentation and symptoms are very similar to Kawasaki Disease (KD). Aim of review: The objective of this review is to compare and contrast differences of Kawasaki Disease and MIS-C. Key scientific concepts of the review: Kawasaki Disease and MIS-C are very similar in clinical presentation and symptomatology. Understanding the diagnostic criteria is crucial to making an accurate diagnosis. Treatments in Kawasaki Disease are established, while in MIS-C treatment protocols are continuing to develop. Careful history taking and laboratory marker analysis should guide the clinician to accurate diagnosis.

Improved National Outcomes Achieved in a Cardiac Learning Health Collaborative Based on Early Performance Level.

OBJECTIVE: Within the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), a learning health network developed to improve outcomes for patients with hypoplastic left heart syndrome and variants, we assessed which centers contributed to reductions in mortality and growth failure. STUDY DESIGN: Centers within the NPC-QIC were divided into tertiles based on early performance for mortality and separately for growth failure. These groups were evaluated for improvement from the early to late time period and compared with the other groups in the late time period. RESULTS: Mortality was 3.8% for the high-performing, 7.6% for the medium-performing, and 14.4% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) from the early to late period. In the late period, there was no difference in mortality between the high- (5.7%), medium- (7%), and low- (4.6%) performing centers (P = .5). Growth failure occurred in 13.9% for the high-performing, 21.9% for the medium-performing, and 32.8% for the low-performing groups in the early time period. Only the low-performing group had a significant change (P < .001) over time. In the late period, there was no significant difference in growth failure between the high- (19.8%), medium- (21.5%), and low- (13.5%) performing groups (P = .054). CONCLUSIONS: Improvements in the NPC-QIC mortality and growth measures are primarily driven by improvement in those performing the worst in these areas initially without compromising the success of high-performing centers. Focus for improvement may vary by center based on performance.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). RESULTS: Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Risk Factors for Death or Transplant After Stage 2 Palliation for Single Ventricle Heart Disease.

Background: For infants with single ventricle heart disease, the time after stage 2 procedure (S2P) is believed to be a lower risk period compared with the interstage period; however, significant morbidity and mortality still occur. Objectives: This study aimed to identify risk factors for mortality or transplantation referral between S2P surgery and the first birthday. Methods: Retrospective cohort analysis of infants in the National Pediatric Cardiology Quality Improvement Collaborative who underwent staged single ventricle palliation from 2016 to 2022 and survived to S2P. Multivariable logistic regression and classification and regression trees were performed to identify risk factors for mortality and transplantation referral after S2P. Results: Of the 1,455 patients in the cohort who survived to S2P, 5.2% died and 2.3% were referred for transplant. Overall event rates at 30 and 100 days after S2P were 2% and 5%, respectively. Independent risk factors for mortality and transplantation referral included the presence of a known genetic syndrome, shunt type at stage 1 procedure (S1P), tricuspid valve repair at S1P, longer time to extubation and reintubation after S1P, ≥ moderate tricuspid regurgitation prior to S2P, younger age at S2P, and the risk groups identified in the classification and regression tree analysis (extracorporeal membrane oxygenation after S1P and longer S2P cardiopulmonary bypass time without extracorporeal membrane oxygenation). Conclusions: Mortality and transplantation referral rates after S2P to 1 year of age remain high ∼7%. Many of the identified risk factors after S2P are similar to those established for interstage factors around the S1P, whereas others may be unique to the period after S2P.

Cryo-balloon angioplasty for pulmonary vein stenosis in pediatric patients.

This study sought to determine the safety and effectiveness of cryo-balloon angioplasty (CbA) for pulmonary vein stenosis (PVS) in pediatric patients. Current therapy options for PVS are less than satisfactory due to recurrent progressive restenosis and neointimal proliferation. Catheterization database, hospital records, imaging studies, and pathologic specimens were reviewed for procedural-related and outcomes data in all patients who underwent pulmonary vein (PV) CbA using the Boston Scientific PolarCath Peripheral Dilation System between August 2006 and June 2009. Thirteen patients (19 PVs; median age 13 months [range 3.5 months to 18.5 years] and weight 7.9 kg [range 3.8 to 47.7]) underwent CbA. Mean PVS diameter after CbA increased from 2.19 (± 0.6) to 3.77 (± 1.1) mm (p < 0.001). Mean gradient decreased from 14 (± 7.4) to 4.89 (± 3.2) mm Hg (p < 0.001). Mean stenosis-to-normal vein diameter ratio increased from 0.52 (± 0.15) to 0.89 (± 0.33) (p < 0.001). Eight patients underwent repeat catheterization a mean of 5.6 months (± 3.66) later. Improved PVS diameter was maintained in 2 PVs. Four veins had restenosis but maintained diameters greater than that before initial CbA. In 11 PVs, the diameter decreased from 4.28 (± 1.14) to 2.53 (± 0.9) mm (p = 0.001). Mean gradient increased from 3.55 (± 3.0) to 14.63 (± 9.6) mm Hg (p = 0.011). All vessels underwent repeat intervention with acute relief of PVS. Stroke occurred within 24 h of CbA in 1 patient. CbA of PVS is safe and results in acute relief of stenosis. However, CbA appears minimally effective as the sole therapy in maintaining long-term relief of PVS.

Post-ligation cardiac syndrome is associated with increased morbidity in preterm infants.

OBJECTIVE: The influence of post-ligation cardiac syndrome (PLCS), a complication of patent ductus arteriosus (PDA) ligations, on neonatal outcomes is unknown. The purpose of this study was to determine the risks of PLCS on severe pulmonary morbidity and severe retinopathy of prematurity (ROP). STUDY DESIGN: Retrospective cohort study of infants who underwent a PDA ligation between 2006 and 2015. Data were collected on patients with and without PLCS. The primary outcome was the difference in severe bronchopulmonary dysplasia (BPD) between groups. Secondary outcomes included discharge with home oxygen and severe ROP. RESULT: A total of 100 infants that underwent PDA ligation during the study period were included in the study; 31 (31%) neonates developed PLCS. In adjusted analysis, PLCS was associated with increased risk for severe BPD (RR 1.67, 95% CI: 1.15-2.42) and home oxygen therapy (RR: 1.47, 95% CI: 1.09-1.99) only. No association with severe ROP was seen (RR: 1.48; 95% CI: 0.87-2.52). CONCLUSION: PLCS is associated with severe neonatal pulmonary morbidity, but not with severe ROP. Further investigation is warranted to validate these results.

Interstage Outcomes in Infants With Single Ventricle Heart Disease Comparing Home Monitoring Technology to Three-Ring Binder Documentation: A Randomized Crossover Study.

BACKGROUND: Interstage outcomes for infants with single ventricle remain suboptimal. We have previously described a tablet PC-based platform Cardiac High Acuity Monitoring Program (CHAMP) for remote monitoring which provides immediate access to data, videos, and instant alerts to our single ventricle care team. METHODS: This study compares traditional three-ring binder monitoring (Binder) to CHAMP using a randomized crossover design to evaluate mortality, resource utilization, and caregiver experience. At discharge, all single ventricle infants were monitored using Binder and randomized to receive CHAMP at either one or two months postdischarge. One month after randomization, caregivers could choose either Binder or CHAMP for the remainder of the interstage period. Caregivers experience was recorded using surveys. RESULTS: Enrollment included 31 single ventricle infants from May 2014 to June 2015. There was no interstage mortality over 4,911 total interstage days (median: 144/patient). Of 73 readmissions, 45 were unplanned. Of the initial 23 unplanned readmissions, 13 were found to have been based on data obtained exclusively through CHAMP (as instant alerts or based on data review) rather than caregiver concerns. Due to concerns regarding patient safety, additional enrollment was stopped. The CHAMP use was associated with significantly fewer unplanned intensive care unit days/100 interstage days, shorter delays in care, lower resource utilization at readmissions, and lower incidence of interstage growth failure and was preferred by a majority of caregivers. CONCLUSIONS: These findings suggest that CHAMP may offer benefits over Binder (improved interstage outcomes, delays in care, and caregiver experience). These findings should be tested across multiple centers in larger populations.

Rates of ICU Transfers After a Scheduled Night-Shift Interprofessional Huddle.

OBJECTIVES: To evaluate a scheduled interprofessional huddle among pediatric residents, nursing staff, and cardiologists on the number of high-risk transfers to the ICU. METHODS: A daily, night-shift huddle intervention was initiated between the in-house pediatric residents and nursing staff covering the cardiology ward patients with the at-home attending cardiologist. Retrospective cohort chart review identified high-risk transfers from the inpatient floor to the ICU over a 24-month period (eg, inotropic support, intubation, and/or respiratory support within 1 hour of ICU transfer). Satisfaction with the intervention and the impact of the intervention on team-based communication and resident education was collected using a retrospective pre-post survey. RESULTS: Ninety-three patients were identified as unscheduled transfers from the ward team to the ICU. Overall, 21 preintervention transfers were considered high risk, whereas only 8 patients were considered high risk after the intervention (P=.004). During the night shift, high risk transfers decreased from 8 of 17 (47%) to 3 of 21 patients (14%) (P=.03). Interprofessional communication improved with 12 of 14 nurses and 24 of 25 residents reporting effective communication after the intervention (P<.0001) compared with only 1 nurse and 15 residents reporting a positive experience before the intervention. Overall, all 3 provider groups stated an improved experience covering a high-risk cardiology patient population. CONCLUSIONS: Implementation of an interprofessional huddle may contribute to decreasing high-risk transfers to the ICU. Initiating a daily huddle was well received and allowed for open lines of communication across all provider groups.

Harnessing Teams and Technology to Improve Outcomes in Infants With Single Ventricle.

Infants with single ventricle require staged cardiac surgery, with stage I typically performed shortly after birth, stage II at 4 to 6 months of age, and stage III at 3 to 5 years of age. There is a high risk of interstage mortality and morbidity after infants are discharged from the hospital between stages I and II. Traditional home monitoring requires caregivers to record measurements of weight and oxygen saturation into a binder and requires families to assume a surveillance role. We have developed a tablet PC-based solution that provides secure and nearly instantaneous transfer of patient information to a cloud-based server, with the capacity for instant alerts to be sent to the caregiver team. The cloud-based IT infrastructure lends itself well to being able to be scaled to multiple sites while maintaining strict control over the privacy of each site. All transmitted data are transferred to the electronic medical record daily. The system conforms to recently released Food and Drug Administration regulation that pertains to mobile health technologies and devices. Since this platform was developed in March 2014, 30 patients have been monitored. There have been no interstage deaths. The experience of care providers has been unanimously positive. The addition of video has added to the use of the monitoring program. Of 30 families, 23 expressed a preference for the tablet PC over the notebook, 3 had no preference, and 4 preferred the notebook to the tablet PC.

Acute post-streptococcal glomerulonephritis in a 14-month-old boy: why is this uncommon?

Acute post-streptococcal glomerulonephritis (APSGN) is rare in children under 2 years of age. This is related in part to the disease patterns of group A streptococcus (GAS) and in part to impaired immunogenicity in infants. We report the case of a 14-month-old child with APSGN following GAS pharyngitis. This case illustrates that APSGN needs to be considered in the evaluation of both gross and microscopic hematuria in this age group. We review the literature of both GAS and APSGN and discuss the pathogenesis and epidemiologic reasons for this association.