Longitudinal cohort study investigating neurodevelopmental and socioemotional outcomes in school-entry aged children after open heart surgery in Australia and New Zealand: the NITRIC follow-up study protocol.

INTRODUCTION: Despite growing awareness of neurodevelopmental impairments in children with congenital heart disease (CHD), there is a lack of large, longitudinal, population-based cohorts. Little is known about the contemporary neurodevelopmental profile and the emergence of specific impairments in children with CHD entering school. The performance of standardised screening tools to predict neurodevelopmental outcomes at school age in this high-risk population remains poorly understood. The NITric oxide during cardiopulmonary bypass to improve Recovery in Infants with Congenital heart defects (NITRIC) trial randomised 1371 children <2 years of age, investigating the effect of gaseous nitric oxide applied into the cardiopulmonary bypass oxygenator during heart surgery. The NITRIC follow-up study will follow this cohort annually until 5 years of age to assess outcomes related to cognition and socioemotional behaviour at school entry, identify risk factors for adverse outcomes and evaluate the performance of screening tools. METHODS AND ANALYSIS: Approximately 1150 children from the NITRIC trial across five sites in Australia and New Zealand will be eligible. Follow-up assessments will occur in two stages: (1) annual online screening of global neurodevelopment, socioemotional and executive functioning, health-related quality of life and parenting stress at ages 2-5 years; and (2) face-to-face assessment at age 5 years assessing intellectual ability, attention, memory and processing speed; fine motor skills; language and communication; and socioemotional outcomes. Cognitive and socioemotional outcomes and trajectories of neurodevelopment will be described and demographic, clinical, genetic and environmental predictors of these outcomes will be explored. ETHICS AND DISSEMINATION: Ethical approval has been obtained from the Children's Health Queensland (HREC/20/QCHQ/70626) and New Zealand Health and Disability (21/NTA/83) Research Ethics Committees. The findings will inform the development of clinical decision tools and improve preventative and intervention strategies in children with CHD. Dissemination of the outcomes of the study is expected via publications in peer-reviewed journals, presentation at conferences, via social media, podcast presentations and medical education resources, and through CHD family partners. TRIAL REGISTRATION NUMBER: The trial was prospectively registered with the Australian New Zealand Clinical Trials Registry as 'Gene Expression to Predict Long-Term Neurodevelopmental Outcome in Infants from the NITric oxide during cardiopulmonary bypass to improve Recovery in Infants with Congenital heart defects (NITRIC) Study - A Multicentre Prospective Trial'. TRIAL REGISTRATION: ACTRN12621000904875.

Balloon dilatation versus surgical valvotomy for congenital aortic stenosis: a propensity score matched study.

BACKGROUND: Balloon valvuloplasty and surgical aortic valvotomy have been the treatment mainstays for congenital aortic stenosis in children. Choice of intervention often differs depending upon centre bias with limited relevant, comparative literature. OBJECTIVES: This study aims to provide an unbiased, contemporary matched comparison of these balloon and surgical approaches. METHODS: Retrospective analysis of patients with congenital aortic valve stenosis who underwent balloon valvuloplasty (Queensland Children's Hospital, Brisbane) or surgical valvotomy (Royal Children's Hospital, Melbourne) between 2005 and 2016. Patients were excluded if pre-intervention assessment indicated ineligibility to either group. Propensity score matching was performed based on age, weight, and valve morphology. RESULTS: Sixty-five balloon patients and seventy-seven surgical patients were included. Overall, the groups were well matched with 18 neonates/25 infants in the balloon group and 17 neonates/28 infants in the surgical group. Median age at balloon was 92 days (range 2 days - 18.8 years) compared to 167 days (range 0 days - 18.1 years) for surgery (rank-sum p = 0.08). Mean follow-up was 5.3 years. There was one late balloon death and two early surgical deaths due to left ventricular failure. There was no significant difference in freedom from reintervention at latest follow-up (69% in the balloon group and 70% in the surgical group, p = 1.0). CONCLUSIONS: Contemporary analysis of balloon aortic valvuloplasty and surgical aortic valvotomy shows no difference in overall reintervention rates in the medium term. Balloon valvuloplasty performs well across all age groups, achieving delay or avoidance of surgical intervention.

Transcatheter closure of the patent ductus arteriosus: an intention to treat analysis.

BACKGROUND: In patients with patent ductus arteriosus (PDA), transcatheter closure is the current procedure of choice. There are multiple devices available with limited current comparative data and varied recommendations for device selection. OBJECTIVE: To assess the efficacy and safety of the Flipper coil (FC) and the Amplatzer Duct Occluder (ADO). METHODS: An intention to treat analysis of all children admitted to the catheter laboratory at a single institution for occlusion of PDA from 2003 to 2011 was performed. Patient and device selection were determined by the treating physician. Standard techniques for FC and ADO implantation were used. RESULTS: Two hundred and twenty eight children with median weight of 14.2 kg (range; 5.5-68 kg) underwent cardiac catheterisation, with successful occlusion in 96.2% of patients when attempted. In 16 patients, median angiographic PDA diameter of 0.8 mm (range; 0.4-1.2 mm), was considered too small to warrant closure. Eight patients with large PDA's underwent surgical ligation. FC was successfully used in 70 (34.3%) and ADO in 134 (66.7%) patients. ADO patients were smaller (P=0.004) with larger PDA's (P<0.0001) than the FC group. Median fluoroscopy time was longer for ADO patients (10.1 min vs 8.0 min; P<0.0001). ADO had a lower embolisation rate (0% vs 6.6%; P=0.005) and a higher complete occlusion rate at follow-up (100% vs 73.4%; P<0.0001). Length of hospital admission decreased with time in both groups. CONCLUSION: Transcatheter closure of the PDA has a high degree of safety and efficacy. This study suggests that the ADO may be the device of first choice in the current era.