Early educational attainment in children with major congenital anomaly in the UK.

OBJECTIVE: To describe early educational attainment and special educational needs (SEN) provision in children with major congenital anomaly (CA) compared with peers. DESIGN: Analysis of educational data linked to the ongoing Born in Bradford cohort study. Confounders were identified via causal inference methods and multivariable logistic regression performed. SETTING: Children born in Bradford Royal Infirmary (BRI), West Yorkshire. PATIENTS: All women planning to give birth at BRI and attending antenatal clinic from March 2007 to December 2010 were eligible. 12 453 women with 13 776 pregnancies (>80% of those attending) were recruited. Records of 555 children with major CA and 11 188 without were linked to primary education records. OUTCOMES: Key Stage 1 (KS1) attainment at age 6-7 years in Maths, Reading, Writing and Science. SEN provision from age 4 to 7 years. RESULTS: 41% of children with major CA received SEN provision (compared with 14% without), and 48% performed below expected standards in at least one KS1 domain (compared with 29% without). The adjusted odds of children with CA receiving SEN provision and failing to achieve the expected standard at KS1 were, respectively, 4.30 (95% CI 3.49 to 5.31) and 3.06 (95% CI 2.47 to 3.79) times greater than their peers. Those with genetic, heart, neurological, urinary, gastrointestinal and limb anomalies had significantly poorer academic achievement. CONCLUSIONS: These novel results demonstrate that poor educational attainment extends to children with urinary, limb and gastrointestinal CAs. We demonstrate the need for collaboration between health and education services to assess and support children with major CA, so every CA survivor can maximise their potential.

Fetal single ventricle journey to first postnatal procedure: a multicentre UK cohort study.

OBJECTIVES: UK single ventricle (SV) palliation outcomes after first postnatal procedure (FPP) are well documented. However, survival determinants from fetal diagnosis to FPP are lacking. To better inform parental-fetal counselling, we examined factors favouring survival at two large UK centres. DESIGN: Retrospective multicentre cohort study. SETTING: Two UK congenital cardiac centres: Leeds and Birmingham. PATIENTS: SV fetal diagnoses from 2015 to 2021. MAIN OUTCOME MEASURES: Survival from fetal diagnosis with intention to treat (ITT) to birth and then FPP. Maternal, fetal and neonatal risk factors were assessed. RESULTS: There were 666 fetal SV diagnoses with 414 (62%) ITT. Of ITT, 381 (92%) were live births and 337 (81%) underwent FPP. Survival (ITT) to FPP was notably reduced for severe Ebstein's 14/22 (63.6%), unbalanced atrioventricular septal defect 32/45 (71%), indeterminate SV 3/4 (75%), mitral atresia 8/10 (80%) and hypoplastic left heart syndrome 127/156 (81.4%). Biventricular pathway was undertaken in five (1%). After multivariable adjustment, prenatal risk factors for mortality were increasing maternal age (OR 1.05, 95% CI 1.0 to 1.1), non-white ethnicity (OR 2.6, 95% CI 1.4 to 4.8), extracardiac anomaly (OR 6.34, 95% CI 1.8 to 22.7) and hydrops (OR 7.39, 95% CI 1.2 to 45.1). Postnatally, prematurity was significantly associated with mortality (OR 6.3, 95% CI 2.3 to 16.8). CONCLUSIONS: Around 20% of ITT fetuses diagnosed with SV will not reach FPP. Risk varies according to the cardiac lesion and is significantly influenced by the presence of an extracardiac anomaly, fetal hydrops, ethnicity, increasing maternal age and gestation at birth. These data highlight the need for fetal preprocedure data to be used in conjunction with procedural outcomes for fetal counselling.

Educational attainment of children with congenital Heart disease in the United Kingdom.

BACKGROUND: Educational attainment in children with congenital heart disease (CHD) within the UK has not been reported, despite the possibility of school absences and disease-specific factors creating educational barriers. METHODS: Children were prospectively recruited to the Born in Bradford birth cohort between March 2007 and December 2010. Diagnoses of CHD were identified through linkage to the congenital anomaly register and independently verified by clinicians. Multivariable regression accounted for relevant confounders. Our primary outcome was the odds of 'below expected' attainment in Maths, Reading and Writing at ages 4-11 years. RESULTS: Educational records of 139 children with non-genetic CHD were compared to 11 188 age-matched children with no major congenital anomaly. Children with CHD had significantly higher odds of 'below expected' attainment in Maths at age 4-5 years (Odds Ratio 1.64, 95% CI 1.07-2.52), age 6-7 (OR 2.03, 95% CI 1.32-3.12), and age 10-11 (OR 2.28, 95% CI 1.01-5.14). Odds worsened with age, with similar results for Reading and Writing. The odds of receiving special educational needs support reduced with age for children with CHD relative to controls (age 4-5: OR 4.84 (2.06-11.40); age 6-7: OR 3.65 (2.41-5.53); age 10-11: OR 2.73 (1.84-4.06)). Attainment was similar for children with and without exposure to cardio-pulmonary bypass. Lower attainment was strongly associated with the number of pre-school hospital admissions. CONCLUSIONS: Children with CHD have lower educational attainment compared to their peers. Deficits are evident from school entry and increase throughout primary school.

Clinical applications of contactless photoplethysmography for monitoring in adults: A systematic review and meta-analysis.

Contactless photoplethysmography (cPPG) is a method of physiological monitoring. It differs from conventional monitoring methods (e.g., saturation probe) by ensuring no contact with the subject by use of a camera. The majority of research on cPPG is conducted in a laboratory setting or in healthy populations. This review aims to evaluate the current literature on monitoring using cPPG in adults within a clinical setting. Adhering to the Preferred Items for Systematic Reviews and Meta-analysis (PRISMA, 2020) guidelines, OVID, Webofscience, Cochrane library, and clinicaltrials.org were systematically searched by two researchers. Research articles using cPPG for monitoring purposes in adults within a clinical setting were selected. Twelve studies with a total of 654 individuals were included. Heart rate (HR) was the most investigated vital sign (n = 8) followed by respiratory rate ((n = 2), Sp02 (n = 2), and HR variability (n = 2). Four studies were included in a meta-analysis of HR compared to ECG data which demonstrated a mean bias of -0.13 (95% CI, -1.22-0.96). This study demonstrates cPPG can be a useful tool in the remote monitoring of patients and has demonstrated accuracy for HR. However, further research is needed into the clinical applications of this method.

Out-of-hours versus in-hours delivery of antenatally diagnosed transposition of the great arteries: outcomes from a United Kingdom Tertiary Centre.

OBJECTIVE: To study the impact of out-of-hours delivery on outcome for neonates with antenatally diagnosed transposition of the great arteries. SETTING: Tertiary paediatric cardiology centre (Yorkshire, United Kingdom), with co-located tertiary neonatal unit. PATIENTS: Neonates with antenatally diagnosed simple transposition of the great arteries delivered out-of-hours (Monday to Friday 17:00-08:00 and weekends) versus in-hours between 2015 and 2020. OUTCOME: The primary outcome was survival to hospital discharge. Secondary outcomes included neurological morbidity, length of stay, and time to balloon atrial septostomy. RESULTS: Of 51 neonates, 38 (75%) were delivered out-of-hours. All neonates born in the tertiary centre survived to discharge. Time to balloon atrial septostomy was slightly longer for out-of-hours deliveries compared to in-hours (median 130 versus 93 mins, p = 0.33). Neurological morbidity occurred for nine (24%) patients in the out-of-hours group and one (8%) in-hours (OR 3.72, 95% CI: 0.42-32.71, p = 0.24). Length of stay was also similar (18.5 versus 17.3 days, p = 0.59). Antenatal diagnosis of a restrictive atrial septum was associated with a lower initial pH (7.03 versus 7.13; CI: 0.03-0.17, p = 0.01), longer length of stay (22.6 versus 17.3 days; CI: 0.37-10.17, p = 0.04), and increased neurological morbidity (44% versus 14%; OR 4.80, CI 1.00-23.15, p = 0.05). A further three neonates were delivered in surrounding hospitals, with a mortality of 67% (versus 0 in tertiary centre; OR 172, CI 5-5371, p = 0.003). CONCLUSION: Neonates with antenatally diagnosed transposition of the great arteries have similar outcomes when delivered out-of-hours versus in-hours. Antenatal diagnosis of restrictive atrial septum is a significant predictor of worse outcomes. In our region, delivery outside the tertiary cardiac centre had a significantly higher risk of mortality.

Hemodynamic Profiles Before and After Surgery in Bicuspid Aortic Valve Disease-A Systematic Review of the Literature.

Bicuspid aortic valve (BAV) disease presents a unique management challenge both pre- and post-operatively. 4D flow MRI offers multiple tools for the assessment of the thoracic aorta in aortic valve disease. In particular, its assessment of flow patterns and wall shear stress have led to new understandings around the mechanisms of aneurysm development in BAV disease. Novel parameters have now been developed that have the potential to predict pathological aortic dilatation and may help to risk stratify BAV patients in future. This systematic review analyses the current 4D flow MRI literature after aortic valve and/or ascending aortic replacement in bicuspid aortic valve disease. 4D flow MRI has also identified distinct challenges posed by this cohort at the time of valve replacement compared to standard management of tri-leaflet disorders, and may help tailor the type and timing of replacement. Eccentric pathological flow patterns seen after bioprosthetic valve implantation, but not with mechanical prostheses, might be an important future consideration in intervention planning. 4D flow MRI also has promising potential in supporting the development of artificial valve prostheses and aortic conduits with more physiological flow patterns.