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.

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.

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.

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.

Registry-based trials have emerged as a potentially cost-saving study methodology. Early estimates of cost savings, however, conflated the benefits associated with registry utilisation and those associated with other aspects of pragmatic trial designs, which might not all be as broadly applicable. In this study, we sought to build a practical tool that investigators could use across disciplines to estimate the ranges of potential cost differences associated with implementing registry-based trials versus standard clinical trials.

We built simulation Markov models to compare unique costs associated with data acquisition, cleaning, and linkage under a registry-based trial design versus a standard clinical trial. We conducted one-way, two-way, and probabilistic sensitivity analyses, varying study characteristics over broad ranges, to determine thresholds at which investigators might optimally select each trial design.

Registry-based trials were more cost effective than standard clinical trials 98.6% of the time. Data-related cost savings ranged from $4300 to $600,000 with variation in study characteristics. Cost differences were most reactive to the number of patients in a study, the number of data elements per patient available in a registry, and the speed with which research coordinators could manually abstract data. Registry incorporation resulted in cost savings when as few as 3768 independent data elements were available and when manual data abstraction took as little as 3.4 seconds per data field.

Registries offer important resources for investigators. When available, their broad incorporation may help the scientific community reduce the costs of clinical investigation. We offer here a practical tool for investigators to assess potential costs savings.

Patients with hypoplastic left heart syndrome and its variants following palliation surgery are at risk for thrombosis. This study examines variability of antithrombotic practice, the incidence of interstage shunt thrombosis, and other adverse events following Stage I and Stage II palliation within the National Pediatric Cardiology Quality Improvement Collaborative registry.

We carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites. Antithrombotic medications used at Stage I and Stage II discharge were evaluated. Variability of antithrombotics use at the individual patient level and intersite variability, incidence of shunt thrombosis, and other adverse events such as cardiac arrest, seizure, stroke, and need for cardiac catheterisation intervention in the interstage period were identified. Antithrombotic strategies for hybrid Stage I patients were evaluated but they were excluded from the variability and outcomes analysis.

A total of 932 Stage I and 923 Stage II patients were included in the study: 93.8% of Stage I patients were discharged on aspirin and 4% were discharged on no antithrombotics, and 77% of Stage II patients were discharged on aspirin and 17.5% were discharged on no antithrombotics. Only three patients (0.2%) presented with interstage shunt thrombosis. The majority of patients who died during interstage or required shunt dilation and/or stenting were discharged home on aspirin.

Aspirin is the most commonly used antithrombotic following Stage I and Stage II palliation. There is more variability in the choice of antithrombotics following Stage II compared with Stage I. The incidence of interstage shunt thrombosis and associated adverse events was rare.

Syncope is common in children and adolescents and most commonly represents neurocardiogenic syncope. No information has been reported regarding the effect of syncope on health-related quality of life in children.

This was a retrospective cohort study of patients seen in the Heart Institute Syncope Clinic at Cincinnati Children's Hospital Medical Center between July, 2009 and June, 2010. Health-related quality of life was assessed using the PedsQL™ tool. PedsQL™ scores were compared with both healthy historical controls and historical controls with chronic illnesses.

A total of 106 patients were included for analysis. In all, 90% were Caucasian and 63% were girls. The median age was 15.1 years (8.2–21.6). Compared with healthy controls, patients had lower PedsQL™ scores: Total score (75.2 versus 83.8, p < 0.0001); Physical Health Summary (78.8 versus 87.5, p < 0.0001); Psychosocial Health Summary (73.9 versus 81.9, p < 0.001), Emotional Functioning (68.9 versus 79.3, p < 0.001); and School Functioning (66.4 versus 81.1, p < 0.001). No difference was seen in Social Functioning (86.2 versus 85.2, p = 0.81). Patients also had lower PedsQL™ Total scores than patients with diabetes mellitus (p < 0.0001) and similar scores to patients with asthma, end-stage renal disease, obesity, and structural heart disease.

Children with syncope, although typically benign in aetiology, can have low health-related quality of life.

Adequate nutritional support is essential for normal infant growth and development. Infants with congenital cardiac disease are known to be at risk for growth failure. We sought to describe perioperative growth in infants undergoing surgical repair of two-ventricle congenital cardiac disease and assess for predictors of their pattern of growth.

Full-term infants who underwent surgical repair of two-ventricle congenital cardiac disease at a single institution were enrolled in a retrospective cohort study performed following a larger prospective study. Infants with facial, gastrointestinal, or neurologic anomalies, trisomy chromosomal abnormality, birth weight less than 2500 grams, or those transferred to another institution before discharge home were excluded. The primary outcome was change in weight-for-age z score from surgery to discharge. Our secondary outcome variable was post-operative hospital length of stay.

A total of 76 infants met the inclusion criteria. Medain age at surgery was 5 days with a range from 1 to 44. The median weight-for-age z score at surgery was −0.2 with a range from −2.9 to 2.8 and by discharge had dropped to −1.2 with a range from −3.4 to 1.8. The median change in weight-for-age z score from surgery to discharge was −1.0 with a range from −2.3 to 0.2. Delayed post-operative nutrition (p < 0.001) and reintubation following initial post-operative extubation (p = 0.001) were associated with decrease in weight-for-age z score.

Infants undergoing repair of two-ventricle congenital cardiac disease had poor growth in the post-operative period. This may be mitigated by early initiation of post-operative nutrition.