Cross-sectional assessment of haemostatic profile and hepatic dysfunction in Fontan patients.

BACKGROUND: Fontan-associated liver disease is accompanied by a hypercoagulable state. While hepatic dysfunction in Fontan patients is common, its relationship with haemostatic changes and clinical outcomes in this patient population remains unclear. OBJECTIVE: To correlate liver dysfunction and haemostatic profiles with clinical outcomes in the Fontan population. PATIENTS/METHODS: Patients were enrolled in a multicentre, cross-sectional study in Australia and New Zealand. Hepatic structure and function were assessed using serum-based calculations (Fibrotest and model for end-stage liver disease excluding international normalised ratio scores). Haemostatic profiles were assessed by Thrombin Generation. Platelet function was assessed via Platelet Factor 4 (PF4) and P-selectin (P-SEL). Clinical outcomes were obtained from the Australian and New Zealand Fontan Registry. RESULTS: Seventy-three patients participated in the study (mean age 18.9±8.5 years with a mean of 13.5±6.9 years post-Fontan). The Endogenous Thrombin Potential (ETP) for patients who suffered thrombotic events (TE) (1366.4±66.2 nM/min) was higher compared with patients with major bleeding events (1011.1±138.4 nM/min) (p=0.03). Except for a negative correlation between Fibrotest-score and PF4 (p=0.045), PF4 and P-SEL concentrations did not correlate with markers of hepatic dysfunction or structural abnormality. CONCLUSIONS: Increased ETP is associated with TE during clinical follow-up after Fontan. This study reinforces that hepatic dysfunction may contribute to the derangement of coagulation factors, impacting the individual risk of haemostatic complications for the Fontan population.

introduce nationwide pulse oximetry screening for the detection of critical congenital heart disease and other hypoxaemic conditions in the newborn.

The mortality risk for infants with critical congenital heart disease (CCHD) unrecognised at the time of birth is high. Pulse oximetry has been utilised as a screening tool for the detection of these anomalies in the newborn as the majority will have a degree of hypoxaemia. This screening strategy has a moderate sensitivity and excellent specificity for the detection of CCHD, and a low false-positive rate. Respiratory and infective diseases are responsible for a large number of positive test results. The early recognition of these diseases can also improve health outcomes. Different approaches have been taken to introduce screening, ranging from hospital-led initiatives to mandatory state-wide policies. A study conducted in New Zealand demonstrated that sector-led screening initiatives are unlikely to result in equitable outcomes. In this midwifery-led maternity setting a nationwide pulse oximetry screening programme with adequate human and material resources should be introduced.

Feasibility study assessing equitable delivery of newborn pulse oximetry screening in New Zealand's midwifery-led maternity setting.

OBJECTIVES: The aim of this study was to conduct New Zealand-specific research to inform the design of a pulse oximetry screening strategy that ensures equity of access for the New Zealand maternity population. Equity is an important consideration as the test has the potential to benefit some populations and socioeconomic groups more than others. SETTING: New Zealand has an ethnically diverse population and a midwifery-led maternity service. One quaternary hospital and urban primary birthing unit (Region A), two regional hospitals (Region B) and three regional primary birthing units (Region C) from three Health Boards in New Zealand's North Island participated in a feasibility study of pulse oximetry screening. Home births in these regions were also included. PARTICIPANTS: There were 27 172 infants that satisfied the inclusion criteria; 16 644 (61%) were screened. The following data were collected for all well newborn infants with a gestation age ≥35 weeks: date of birth, ethnicity, type of maternity care provider, deprivation index and screening status (yes/no). The study was conducted over a 2-year period from May 2016 to April 2018. RESULTS: Screening rates improved over time. Infants born in Region B (adjusted OR=0.75; 95% CI 0.67 to 0.83) and C (adjusted OR=0.29; 95% CI 0.27 to 0.32) were less likely to receive screening compared with those born in Region A. There were significant associations between screening rates and deprivation, ethnicity and maternity care provider. Lack of human and material resources prohibited universal access to screening. CONCLUSION: A pulse oximetry screening programme that is sector-led is likely to perpetuate inequity. Screening programmes need to be designed so that resources are distributed in the way most likely to optimise health outcomes for infants born with cardiac anomalies. ETHICS APPROVAL: This study was approved by the Health and Disability Ethics Committees of New Zealand (15/NTA/168).