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We sought to estimate the median post-operative length of stay (PLOS) and predictors of PLOS following tetralogy of Fallot (ToF) repair at a specialist surgical center in the North of England. The local National Congenital Heart Disease Audit dataset was used to identify patients aged < 2 years who underwent surgical repair for ToF between 1 January 1986 and 13 May 2022. Coefficients representing the median change in PLOS (days) according to predictors were estimated using Quantile regression. There were 224 patients (59.4% male, median age = 9 months, interquartile range (IQR) 5–13 months) with a median PLOS of 9 days (IQR 7–13). In the univariable regression, age (months) and weight (kg) at operation (β = − 0.17, 95% CI: − 0.33, − 0.01) and (β = − 0.53, 95% CI: − 0.97, − 0.10), previous (cardiac or thoracic) procedure (β = 5, 95% CI:2.38, 7.62), procedure urgency (elective vs urgent) (β = 2.8, 95% CI:0.39, 5.21), bypass time (mins) (β = 0.03, 95% CI:0.01, 0.05), cross-clamp time (mins) (β = 0.03, 95% CI:0.01, 0.06) and duration of post-operative intubation (days) (β = 0.81, 95% CI:0.67, 0.96), were significantly associated with PLOS. Previous procedure and intubation time remained significant in multivariable analyses. Some patient and operative factors can predict PLOS following complete ToF repair. Information on PLOS is important for health professionals to support parents in preparing for their child's discharge and to make any necessary practical arrangements. Health commissioners can draw on evidence-based guidance for resource planning. The small sample size may have reduced the power to detect small effect sizes, but this regional study serves as a foundation for a larger national study. [ABSTRACT FROM AUTHOR]

Background: Parents of children who have a congenital anomaly can experience significant worry about their child's health. Access to clear, helpful, and trustworthy information can provide a valuable source of support. In this study the aim was to explore the information needs of parents/carers of children with congenital anomalies across Europe.Method: A cross-sectional online survey was developed in nine languages to measure parents' information needs, including: (1) the 'helpfulness'/'trustworthiness' of information received from eight relevant sources, and (2) overall satisfaction with information received. Parents/carers of children (0-10 years) with cleft lip, spina bifida, congenital heart defect [CHD] requiring surgery, and/or Down syndrome were recruited online via relevant organisations in 10 European countries from March-July 2021. Quantitative analyses using multivariable logistic regressions were performed.Results: One thousand seventy parents/carers of children with a cleft lip (n = 247), spina bifida (n = 118), CHD (n = 366), Down syndrome (n = 281), and Down syndrome with CHD (n = 58) were recruited in Poland (n = 476), the UK (n = 120), Germany (n = 97), the Netherlands/Belgium (n = 74), Croatia (n = 68), Italy (n = 59), other European countries (n = 92), and not specified/non-European countries (n = 84). Most participants were mothers (92%) and aged 31-40 years (71%). Participants were most likely to rate support groups (63%), patient organisations (60%), specialist doctors/nurses (58%), and social media (57%) as 'very helpful' information sources. 'Very trustworthy' ratings remained high for specialist doctors/nurses (61%), however, they declined for support groups (47%), patient organisations (48%), and social media (35%). Germany had the highest proportion of participants who were 'very satisfied' (44%, 95% CI = 34%-54%) with information, whereas this percentage was lowest in Croatia (11%, 95% CI = 3%-19%) and Poland (15%, 95% CI = 11%-18%). Parents of children with Down syndrome had significantly lower satisfaction ratings than parents of children with CHD; 13% (95% CI = 8%-18%) reported being 'very satisfied' compared to 28% (95% CI = 23%-33%) in the CHD group.Conclusions: Findings suggest that informal sources of information (e.g. support groups) are of value to parents, however, they are not deemed as trustworthy as specialist medical sources. Satisfaction ratings differed across countries and by anomaly, and were particularly low in Croatia and Poland, as well as for parents of children with Down syndrome, which warrants further investigation. [ABSTRACT FROM AUTHOR]

Background: Following a reduction in global child mortality due to communicable diseases, the relative contribution of congenital anomalies to child mortality is increasing. Although infant survival of children born with congenital anomalies has improved for many anomaly types in recent decades, there is less evidence on survival beyond infancy. We aimed to systematically review, summarise, and quantify the existing population-based data on long-term survival of individuals born with specific major congenital anomalies and examine the factors associated with survival.Methods and Findings: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, and citations of the included articles for studies published 1 January 1995 to 30 April 2020 were searched. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate. We included original population-based studies that reported long-term survival (beyond 1 year of life) of children born with a major congenital anomaly with the follow-up starting from birth that were published in the English language as peer-reviewed papers. Studies on congenital heart defects (CHDs) were excluded because of a recent systematic review of population-based studies of CHD survival. Meta-analysis was performed to pool survival estimates, accounting for trends over time. Of 10,888 identified articles, 55 (n = 367,801 live births) met the inclusion criteria and were summarised narratively, 41 studies (n = 54,676) investigating eight congenital anomaly types (spina bifida [n = 7,422], encephalocele [n = 1,562], oesophageal atresia [n = 6,303], biliary atresia [n = 3,877], diaphragmatic hernia [n = 6,176], gastroschisis [n = 4,845], Down syndrome by presence of CHD [n = 22,317], and trisomy 18 [n = 2,174]) were included in the meta-analysis. These studies covered birth years from 1970 to 2015. Survival for children with spina bifida, oesophageal atresia, biliary atresia, diaphragmatic hernia, gastroschisis, and Down syndrome with an associated CHD has significantly improved over time, with the pooled odds ratios (ORs) of surviving per 10-year increase in birth year being OR = 1.34 (95% confidence interval [95% CI] 1.24-1.46), OR = 1.50 (95% CI 1.38-1.62), OR = 1.62 (95% CI 1.28-2.05), OR = 1.57 (95% CI 1.37-1.81), OR = 1.24 (95% CI 1.02-1.5), and OR = 1.99 (95% CI 1.67-2.37), respectively (p < 0.001 for all, except for gastroschisis [p = 0.029]). There was no observed improvement for children with encephalocele (OR = 0.98, 95% CI 0.95-1.01, p = 0.19) and children with biliary atresia surviving with native liver (OR = 0.96, 95% CI 0.88-1.03, p = 0.26). The presence of additional structural anomalies, low birth weight, and earlier year of birth were the most commonly reported predictors of reduced survival for any congenital anomaly type. The main limitation of the meta-analysis was the small number of studies and the small size of the cohorts, which limited the predictive capabilities of the models resulting in wide confidence intervals.Conclusions: This systematic review and meta-analysis summarises estimates of long-term survival associated with major congenital anomalies. We report a significant improvement in survival of children with specific congenital anomalies over the last few decades and predict survival estimates up to 20 years of age for those born in 2020. This information is important for the planning and delivery of specialised medical, social, and education services and for counselling affected families. This trial was registered on the PROSPERO database (CRD42017074675). [ABSTRACT FROM AUTHOR]

Background: The impact of socio-economic status (SES) on congenital heart disease (CHD)-related mortality in children is not well established.Objectives: We aimed to systematically review and appraise the existing evidence on the association between SES (including poverty, parental education, health insurance, and income) and mortality among children with CHD.Data Sources: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, citations, and key journals were searched.Study Selection and Data Extraction: We included articles reporting original research on the association between SES and mortality in children with CHD if they were full papers published in the English language and regardless of (a) timing of mortality; (b) individual or area-based measures of SES; (c) CHD subtype; (d) age at ascertainment; (e) study period examined. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate.Synthesis: Meta-analyses were performed to estimate pooled ORs for in-hospital mortality according to health insurance status.Results: Of 1388 identified articles, 28 met the inclusion criteria. Increased area-based poverty was associated with increased odds/risk of postoperative (n = 1), neonatal (n = 1), post-discharge (n = 1), infant (n = 1), and long-term mortality (n = 2). Higher parental education was associated with decreased odds/risk of neonatal (n = 1) and infant mortality (n = 5), but not with long-term mortality (n = 1). A meta-analysis of four US articles showed increased unadjusted odds of in-hospital mortality in those with government/public versus private health insurance (OR 1.40, 95% CI 1.24, 1.56). The association between area-based income and CHD-related mortality was conflicting, with three of eight articles reporting significant associations.Conclusion: This systematic review provides evidence that children of lower SES are at increased risk of CHD-related mortality. As these children are over-represented in the CHD population, interventions targeting socio-economic inequalities could have a large impact on improving CHD survival. [ABSTRACT FROM AUTHOR]

Background Studies have reported that advanced maternal age is a risk factor for congenital heart disease (CHD), but none of these have been performed in the United Kingdom. Currently, women in the United Kingdom are not referred for specialist fetal echocardiography based on maternal age alone. The aim of this study is to examine the association between maternal age at delivery and CHD prevalence in the North of England. Methods Singleton cases of CHD notified to the Northern Congenital Abnormality Survey and born between January 1, 1998, to December 31, 2013, were included. Cases with chromosomal anomalies were excluded. The relative risk (RR) of CHD according to maternal age at delivery was estimated using Poisson regression. Results There were 4024 singleton cases of nonchromosomal CHD, giving a prevalence of 8.1 (95% confidence interval [CI], 7.8-8.3) per 1000 live and stillbirths. There was no association between maternal age at delivery and CHD prevalence ( p = 0.97), with no evidence of an increased risk of CHD in mothers aged ≥35 compared to aged 25 to 29 (RR = 0.99; 95% CI, 0.89-1.09). There were no significant associations between maternal age at delivery and severity III CHD ( p = 0.84), severity II CHD ( p = 0.74), or severity I CHD ( p = 0.66), although there was a slight increased risk of severity I CHD in mothers aged ≥35 (RR = 1.27; 95% CI, 0.83-1.95). Conclusion We found little evidence that advanced maternal age is a risk factor for CHD. There is no evidence that women in the United Kingdom should be referred for specialist prenatal cardiac screening based on their age. Birth Defects Research (Part A) 106:461-467, 2016. © 2016 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Background Holt-Oram syndrome (HOS) is an autosomal dominant disorder characterised by upper limb anomalies and congenital heart defects. We present epidemiological and clinical aspects of HOS patients using data from EUROCAT (European Surveillance of Congenital Anomalies) registries. Methods The study was based on data collected during 1990-2011 by 34 registries. The registries are population-based and use multiple sources of information to collect data on all types of birth using standardized definitions, methodology and coding. Diagnostic criteria for inclusion in the study were the presence of radial ray abnormalities and congenital heart disease (CHD), or the presence of either radial ray anomaly or CHD, with family history of HOS. Results A total of 73 cases of HOS were identified, including 11 (15.1%) TOPFA and 62 (84.9%) LB. Out of 73 HOS cases, 30.8% (20/65) were suspected prenatally, 55.4% (36/65) at birth, 10.7% (7/65) in the first week of life, and 3.1% (2/65) in the first year of life. The prenatal detection rate was 39.2% (20/51), with no significant change over the study period. In 55% (11/20) of prenatally detected cases, parents decided to terminate pregnancy. Thumb anomalies were reported in all cases. Agenesis/hypoplasia of radius was present in 49.2% (30/61), ulnar aplasia/hypoplasia in 24.6% (15/61) and humerus hypoplasia/phocomelia in 42.6% (26/61) of patients. Congenital heart defects (CHD) were recorded in 78.7% (48/61) of patients. Isolated septal defects were present in 54.2 (26/48), while 25% (12/48) of patients had complex/severe CHD. The mean prevalence of HOS diagnosed prenatally or in the early years of life in European registries was 0.7 per 100,000 births or 1:135,615 births. Conclusions HOS is a rare genetic condition showing regional variation in its prevalence. It is often missed prenatally, in spite of the existence of major structural anomalies. When discovered, parents in 45% (9/20) of cases opt for the continuation of pregnancy. Although a quarter of patients have severe CHD, the overall first week survival is very good, which is important information for counselling purposes. [ABSTRACT FROM AUTHOR]

Background: Unilateral lung agenesis is an uncommon congenital abnormality, with a lack of reported accurate incidence estimates. Prognosis is also uncertain, with older literature reporting poor outcomes. Methods: The North of England register of congenital anomalies (Northern Congenital Abnormality Survey) records cases of congenital anomalies to mothers' resident in the region. We used the register to identify all patients with congenital lung agenesis born between 2004 and 2013 to calculate an accurate incidence estimate and report clinical outcomes with contemporary management. Results: Four patients with congenital lung agenesis were born during the study period, giving an estimated incidence in the North of England of 1.22 per 100,000 live births (95% confidence interval, 0.33-3.11). Two patients had associated congenital heart disease requiring corrective surgery, and one had musculoskeletal anomalies. All four patients are alive and well without a regular oxygen requirement. Conclusion: Contrary to previous reports, the medium term outcomes in our patients have been good, even when lung agenesis is associated with other congenital anomalies. Long-term prognosis with modern management remains unknown, and the potential for the development of pulmonary hypertension remains a concern. [ABSTRACT FROM AUTHOR]

Abstract: Maternal exposure to ambient air pollution has increasingly been linked to adverse pregnancy outcomes. The evidence linking this exposure to congenital anomalies is still limited and controversial. This case–control study investigated the association between maternal exposure to ambient particulate matter with aerodynamic diameter less than 10μm (PM10), sulfur dioxide (SO2), nitrogen dioxide, nitric oxide (NO), ozone (O3), and carbon monoxide (CO) and the occurrence of congenital heart disease in the population of Northeast England (1993–2003). Each case and control was assigned weekly average (weeks 3–8 of pregnancy) of pollutant levels measured by the closest monitor to the mother’s residential postcode. Using exposure as both continuous and categorical variables, logistic regression models were constructed to quantify the adjusted odds ratios of exposure to air pollutants and the occurrence of each outcome group. We found exposure to CO and NO to be associated with ventricular septal defect and cardiac septa malformations. CO was also associated with congenital pulmonary valve stenosis and NO with pooled cases of congenital heart disease and tetralogy of Fallot. Findings for SO2, O3 and PM10 were less consistent. [Copyright &y& Elsevier]

Recent studies have linked maternal exposure to air pollution with a range of adverse pregnancy outcomes. However, the available evidence linking this exposure to congenital anomalies is still limited and controversial. The present case-control study tested the hypothesis that maternal exposure to ambient black smoke and sulfur dioxide is a risk factor for the occurrence of congenital heart disease. The authors used registry-based data on congenital heart disease for the population of the northeast of England in 1985–1996. A 2-stage spatiotemporal model was developed to predict weekly black smoke and sulfur dioxide levels at each maternal place of residence. Controls were frequency-matched to cases by year of birth (control-to-case ratio of 4:1). Two sets of analyses were performed, using predicted mean values of exposure and 1,000 simulated scenarios of exposure. The analyses were adjusted for birth year, socioeconomic status, infant sex, season of conception, and degree of urbanity. The authors found a weak association between maternal exposure to black smoke and congenital malformations of cardiac chambers and connections only when using exposure as a continuous variable. When the authors used quartiles of exposure, odds ratios did not show a dose-response relation for consecutive quartiles. For sulfur dioxide, the results were not indicative of any association. [ABSTRACT FROM PUBLISHER]

Congenital heart disease (CHD) is the most prevalent group of congenital anomalies. There is considerable variation in the reported epidemiology of CHD, mainly attributable to methodological differences. Using register-based data, the current study describes the epidemiology of CHD in a geographically well-defined population of the North of England during 1985–2003. The total prevalence of CHD was 85.9 per 10 000 births and terminations of pregnancy for fetal anomaly. Livebirth prevalence was 79.7 per 10 000 livebirths. Both total and livebirth prevalence increased during the study period. Ninety-two per cent of affected pregnancies resulted in a livebirth, 5% were terminated, 2% resulted in a stillbirth, and 1% in a late miscarriage. Almost a quarter (23%) of cases had one or more coincident anomalies of other organs, with chromosomal abnormalities the most frequent group. A total of 89.2% of cases survived to 1 year and the survival improved during the study period. This population-based study has demonstrated an increasing trend in both prevalence and survival among children with CHD. [ABSTRACT FROM AUTHOR]