Geographical challenges and inequity of healthcare access for high-risk paediatric heart disease.

BACKGROUND: Geographical context is an important consideration for health system design to promote equality in access to care for patients with childhood heart disease (CHD), particularly those living in regional, rural, and remote areas. To help inform future policy and practice recommendations, this study aimed to (i) describe the geographic distribution of high-risk CHD patients accessing an Australian state-wide specialist service and (ii) estimate travel time for accessing healthcare via general practitioners (primary), nearest paediatric centre (secondary) and specialist paediatric cardiac centre (tertiary). METHODS: Participants included a cohort of children (0-18 year) who accessed state-wide specialist CHD services over a 3-year period (2019-2021) in Queensland, Australia. Locations for patient residence, general practitioner, closest paediatric centre and tertiary cardiac centre were mapped using geographical information system (GIS) software (ArcGIS Online). Travel distance and times were estimated using a Google Maps Application Programming Interface (API). RESULTS: 1019 patients (median age 3.8 years) had cardiac intervention and were included in the sample. Of this cohort, 30.2% lived outside the heavily urbanised South East Queensland (SEQ) area where the tertiary centre is located. These patients travel substantially further and longer to access tertiary level care (but not secondary or primary level care) compared to those in SEQ. Median distance for patients residing outside SEQ to access tertiary care was 953 km with a travel time of 10 h 43 min. This compares to 5.5 km to the general practitioner and 20.6 km to a paediatric service (8.9 and 54 min respectively). CONCLUSION: This geographical mapping of CHD services has demonstrated a key challenge inherent in providing specialist cardiac care to children in a large state-based healthcare system. A significant proportion of high-risk patients live large distances from tertiary level care. The greater accessibility of primary care services highlights the importance of supporting primary care physicians outside metropolitan areas to acquire or build the ability and capacity to care for children with CHD. Strengthening local primary and secondary services not only has the potential to improve the outcomes of high-risk patients, but also to reduce costs and burden associated with potentially avoidable travel from regional, rural, or remote areas to access specialist CHD services.

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.

Nitric Oxide on Extracorporeal Membrane Oxygenation in Neonates and Children (NECTAR Trial): Protocol for a Randomized Controlled Trial.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) provides support for the pulmonary or cardiovascular function of children in whom the predicted mortality risk remains very high. The inevitable host inflammatory response and activation of the coagulation cascade due to the extracorporeal circuit contribute to additional morbidity and mortality in these patients. Mixing nitric oxide (NO) into the sweep gas of ECMO circuits may reduce the inflammatory and coagulation cascade activation during ECMO support. OBJECTIVE: The purpose of this study is to test the feasibility and safety of mixing NO into the sweep gas of ECMO systems and assess its effect on inflammation and coagulation system activation through a pilot randomized controlled trial. METHODS: The Nitric Oxide on Extracorporeal Membrane Oxygenation in Neonates and Children (NECTAR) trial is an open-label, parallel-group, pilot randomized controlled trial to be conducted at a single center. Fifty patients who require ECMO support will be randomly assigned to receive either NO mixed into the sweep gas of the ECMO system at 20 ppm for the duration of ECMO or standard care (no NO) in a 1:1 ratio, with stratification by support type (veno-venous vs veno-arterial ECMO). RESULTS: Outcome measures will focus on feasibility (recruitment rate and consent rate, and successful inflammatory marker measurements), the safety of the intervention (oxygenation and carbon dioxide control within defined parameters and methemoglobin levels), and proxy markers of efficacy (assessment of cytokines, chemokines, and coagulation factors to assess the impact of NO on host inflammation and coagulation cascade activation, clotting of ECMO components, including computer tomography scanning of oxygenators for clot assessments), bleeding complications, as well as total blood product use. Survival without ECMO and the length of stay in the pediatric intensive care unit (PICU) are clinically relevant efficacy outcomes. Long-term outcomes include neurodevelopmental assessments (Ages and Stages Questionnaire, Strength and Difficulties Questionnaire, and others) and quality of life (Pediatric Quality of Life Inventory and others) measured at 6 and 12 months post ECMO cannulation. Analyses will be conducted on an intention-to-treat basis. CONCLUSIONS: The NECTAR study investigates the safety and feasibility of NO as a drug intervention during extracorporeal life support and explores its efficacy. The study will investigate whether morbidity and mortality in patients treated with ECMO can be improved with NO. The intervention targets adverse outcomes in patients who are supported by ECMO and who have high expected mortality and morbidity. The study will be one of the largest randomized controlled trials performed among pediatric patients supported by ECMO. TRIAL REGISTRATION: Australian New Zealand Clinical Trials Registry ACTRN12619001518156; https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=376869. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/43760.

Ultrasonographic assessment of aortic flow characteristics in a paediatric patient with sepsis treated with extracorporeal life support: defining the mixing zone.

We describe a case of severe sepsis in a 14-year-old boy who was treated with veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) support. The haemodynamic challenges inherent to femoro-femoral VA ECMO are discussed, and the use of ultrasonography to define the location of the mixing zone in the abdominal aorta is demonstrated. We propose that the use of ultrasound is able to assist clinicians in understanding perfusion of abdominal organs in the presence of suspected differential oxygenation.

Hybrid procedure as an alternative to surgical palliation of high-risk infants with hypoplastic left heart syndrome and its variants.

OBJECTIVES: Despite improvements in the surgical management of neonates with the hypoplastic left heart syndrome and its variants, there is a subset of children who have a high predicted mortality. We have analyzed and report our results with the hybrid approach in the management of these high-risk patients. METHODS: Children identified by our selection criteria underwent the initial hybrid procedure performed in the catheterization laboratory. This consists of surgical banding of the branch pulmonary arteries and stenting of the arterial duct. This was followed 3 to 6 months later by the combined stage I and II Norwood operation or biventricular repair. RESULTS: Between December 2005 and May 2008, 21 neonates were selected for the hybrid procedure, of whom 16 had typical hypoplastic left heart syndrome. The remaining 5 neonates had severe aortic stenosis with borderline left ventricular dimensions or poor left ventricular function at presentation. Four neonates had a virtually intact interatrial septum and required urgent intervention immediately after birth. There were 6 deaths as inpatients or up to 30 days after the procedure and 1 interstage death before the combined stage I and II operation. Thus far, of 12 babies who have undergone the next operation, 9 have had the combined stage I and II operation and 3 have had a biventricular repair. Two of these 12 babies have died after the combined stage I and II operation. CONCLUSION: A hybrid approach may be a valuable alternative to the management of high-risk neonates with hypoplastic left heart syndrome and its variants.

Large subpectoral hematoma: possible complication of sternotomy post-cardiac surgery.

A 62-year-old female patient developed a significantly enlarged left breast 6 hours after sternotomy for a coronary artery bypass graft operation. This was the result of subpectoral bleeding caused by a sternal wire.