Variation exists in the timing of surgery for balanced complete atrioventricular septal defect repair. We sought to explore associations between timing of repair and resource utilisation and clinical outcomes in the first year of life.

In this retrospective single-centre cohort study, we included patients who underwent complete atrioventricular septal defect repair between 2005 and 2019. Patients with left or right ventricular outflow tract obstruction and major non-cardiac comorbidities (except trisomy 21) were excluded. The primary outcome was days alive and out of the hospital in the first year of life.

Included were 79 infants, divided into tertiles based on age at surgery (1st = 46 to 137 days, 2nd = 140 – 176 days, 3rd = 178 – 316 days). There were no significant differences among age tertiles for days alive and out of the hospital in the first year of life by univariable analysis (tertile 1, median 351 days; tertile 2, 348 days; tertile 3, 354 days; p = 0.22). No patients died. Fewer post-operative ICU days were used in the oldest tertile relative to the youngest, but days of mechanical ventilation and hospitalisation were similar. Clinical outcomes after repair and resource utilisation in the first year of life were similar for unplanned cardiac reinterventions, outpatient cardiology clinic visits, and weight-for-age z-score at 1 year.

Age at complete atrioventricular septal defect repair is not associated with important differences in clinical outcomes or resource utilisation in the first year of life.

Data regarding preoperative mortality in neonates with critical CHD are sparse and would aid patient care and family counselling. The objective of this study was to utilise a multicentre administrative dataset to report the rate of and identify risk factors for preoperative in-hospital mortality in neonates with critical CHD across US centres.

The Pediatric Health Information System database was utilised to search for newborns ≤30 days old, born 1 January 2009 to 30 June 2018, with an ICD-9/10 code for d-transposition of the great arteries, truncus arteriosus, interrupted aortic arch, or hypoplastic left heart syndrome. Preoperative in-hospital mortality was defined as patients who died prior to discharge without an ICD code for cardiac surgery or interventional catheterisation.

Overall preoperative mortality rate was at least 5.4% (690/12,739) and varied across diagnoses (d-TGA 2.9%, TA 8.3%, IAA 5.5%, and HLHS 7.3%) and centres (0–20.5%). In multivariable analysis, risk factors associated with preoperative mortality included preterm delivery (<37 weeks) (OR 2.3, 95% CI: 1.8–2.9; p < 0.01), low birth weight (<2.5 kg) (OR 3.8, 95% CI: 3.0–4.7; p < 0.01), and genetic abnormality (OR 1.6, 95% CI: 1.2–2.2; p < 0.01). Centre average surgical volume was not a significant risk factor.

Approximately 1 in 20 neonates with critical CHD suffered preoperative in-hospital mortality, and rates varied across diagnoses and centres. Better understanding of the factors that drive the variation (e.g. patient factors, preoperative care models, surgical timing) could help identify patient care improvement opportunities and inform conversations with families.

The efficacy of a specialized pediatric cardiac rapid response team is unknown. We hypothesized that a specialized cardiac rapid response team would facilitate team-wide communication between the cardiac stepdown unit and cardiac intensive care unit (ICU) teams and improve patient care.

A specialized pediatric cardiac rapid response team was implemented in June 2015. All pediatric cardiac rapid response team activations and outcomes from implementation through December 2018 were reviewed. Cardiac arrests and unplanned transfers to the cardiac ICU were indexed to 1000 patient-days to account for inpatient volume trends and evaluated over time.

There were 202 cardiac rapid response team activations in 108 unique patients during the study period. After implementation of the pediatric cardiac rapid response team, unplanned transfers from the cardiac stepdown unit to the cardiac ICU decreased from 16.8 to 7.1 transfers per 1000 patient days (p = 0.012). The stepdown unit cardiac arrest rate decreased from 1.2 to 0.0 arrests per 1000 patient-days (p = 0.015). There was one death on the cardiac stepdown unit in the 5 years since the implementation of the cardiac rapid response team, compared to four deaths in the previous 5 years.

A reduction in unplanned cardiac ICU transfers, cardiac arrests, and mortality on the cardiac stepdown unit has been observed since the implementation of a specialized pediatric cardiac rapid response team. A specialized cardiac rapid response team may improve communication and empower the interdisciplinary care team to escalate care for patients experiencing clinical decline.

Children with congenital heart disease (CHD) require lifelong cardiology follow-up. Many experience gaps in care around the age of transition to adult-oriented care with associated comorbidity. We describe the impact of a clinic-based intervention on follow-up rates in this high-risk population.

Patients ≥11 years seen in a paediatric outpatient CHD Transition Clinic completed self-assessment questionnaires, underwent focused teaching, and were followed on a clinic registry with assessment of care continuation. The cohort “lost to follow-up” rate, defined as absence from care at least 6 months beyond the recommended timeframe, was compared with a control group. Secondary outcomes included questionnaire scores and adult cardiology referral trends.

Over 26 months, 53 participants completed an initial Transition Clinic visit; 43% (23/53) underwent a second visit. Median participant age was 18.0 years (interquartile range 16.0, 22.0). The cohort’s “lost to follow-up” rate was 7.3%, which was significantly lower than the control rate (25.9%, p < 0.01). Multivariable regression analyses demonstrated clinic participation as the only factor independently associated with follow-up rates (p = 0.048). Transition readiness was associated with older age (p = 0.01) but not sex, univentricular heart, interventional history, or surgical complexity. One-third of adult participants transferred to adult care.

A CHD Transition Clinic intervention can improve follow-up rates in adolescents and young adults. Age is an important factor in transition readiness, and retention of adults in paediatric care appears multi-factorial. We postulate that serial assessments of self-management, focused education, and registry utilisation may improve patient outcomes by reducing lapses in care.

Indexing left ventricular mass to body surface area or height2.7 leads to inaccuracies in diagnosing left ventricular hypertrophy in obese children. Lean body mass predictive equations provide the opportunity to determine the utility of lean body mass in indexing left ventricular mass. Our objectives were to compare the diagnostic accuracy of predicted lean body mass, body surface area, and height in detecting abnormal left ventricle mass in obese children.

Obese non-hypertensive patients aged 4–21 years were recruited prospectively. Dual-energy X-ray absorptiometry was used to measure lean body mass. Height, weight, sex, race, and body mass index z-score were used to calculate predicted lean body mass.

We enrolled 328 patients. Average age was 12.6 ± 3.8 years. Measured lean body mass had the strongest relationship with left ventricular mass (R2 = 0.84, p < 0.01) compared to predicted lean body mass (R2 = 0.82, p < 0.01), body surface area (R2 = 0.80, p < 0.01), and height2.7 (R2 = 0.65, p < 0.01). Of the clinically derived variables, predicted lean body mass was the only measure to have an independent association with left ventricular mass (β = 0.90, p < 0.01). Predicted lean body mass was the most accurate scaling variable in detecting left ventricular hypertrophy (positive predictive value = 88%, negative predictive value = 99%).

Lean body mass is the strongest predictor of left ventricular mass in obese children. Predicted lean body mass is the most accurate anthropometric scaling variable for left ventricular mass in left ventricular hypertrophy detection. Predicted lean body mass should be considered for clinical use as the body size correcting variable for left ventricular mass in obese children.