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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 Anorectal anomalies are atresias or stenoses of the anal canal and rectum with or without fistulous connections with the bladder, urethra, perineum, or vestibule. The aim of this study was to describe the epidemiology of anorectal anomalies, including associated anomalies, prevalence, and temporal trends. METHODS Anorectal anomalies occurring in late miscarriages (>20 gestation weeks), terminations of pregnancy for fetal anomaly (any gestation), stillbirths (≥24 gestation weeks) and live births, delivered from 1985 to 2010, notified to the Northern Congenital Abnormality Survey (NorCAS) were included in this population-based case series. RESULTS There were 278 cases among 892,194 live births. Twenty (7.2%) cases occurred in twin pregnancies and 24 (8.7%) with chromosomal anomalies/genetic syndromes were excluded. There were 234 cases (total prevalence = 2.7, 95% confidence interval [CI]: 2.4, 3.1 per 10,000 live and stillbirths). There was no evidence of a trend in prevalence over time (Chi2 test for trend: p = 0.789). There was a male predominance (70.9%). Of the 234 cases, 167 (71.4%) were live born, two (0.9%) were late miscarriages, seven (3.0%) were stillbirths, and 58 (24.8%) were terminations of pregnancy for fetal anomaly. There was no significant association with maternal age at delivery ( p = 0.095). 7.2% of isolated cases (cases with no other congenital anomalies) were diagnosed prenatally. CONCLUSION The prevalence of anorectal anomalies in this study is comparable to other case series. There was no evidence of a temporal increase in prevalence. We confirmed a male predominance of anorectal anomalies and no significant association with maternal age. Birth Defects Research (Part A) 103:597-602, 2015. © 2015 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Background: Hirschsprung's disease is a congenital gut motility disorder, characterised by the absence of the enteric ganglion cells along the distal gut. The aim of this study was to describe the epidemiology of Hirschsprung's disease, including additional congenital anomalies, total prevalence, trends, and association with maternal age. Methods: Cases of Hirschsprung's disease delivered during 1980 to 2009 notified to 31 European Surveillance of Congenital Anomaly registers formed the population-based case-series. Prevalence rates and 95% confidence intervals were calculated as the number of cases per 10,000 births. Multilevel Poisson regression was performed to investigate trends in prevalence, geographical variation and the association with maternal age. Results: There were 1,322 cases of Hirschsprung's disease among 12,146,210 births. The total prevalence was 1.09 (95% confidence interval, 1.03-1.15) per 10,000 births and there was a small but significant increase in prevalence over time (relative risk=1.01; 95% credible interval, 1.00-1.02; p=0.004). There was evidence of geographical heterogeneity in prevalence (p<0.001). Excluding 146 (11.0%) cases with chromosomal anomalies or genetic syndromes, there were 1,176 cases (prevalence=0.97; 95% confidence interval, 0.91-1.03 per 10,000 births), of which 137 (11.6%) had major structural anomalies. There was no evidence of a significant increased risk of Hirschsprung's disease in cases born to women aged ≥35 years compared with those aged 25 to 29 (relative risk=1.09; 95% credible interval, 0.91-1.31; p=0.355). Conclusion: This large population-based study found evidence of a small increasing trend in Hirschsprung's disease and differences in prevalence by geographic location. There was also no evidence of an association with maternal age. [ABSTRACT FROM AUTHOR]

INTRODUCTION Hirschsprung's disease is the commonest congenital gut motility disorder, characterized by the absence of the enteric ganglion cells along the distal gut, which causes intestinal obstruction. Few publications report its epidemiology and temporal trends. METHODS Cases of Hirschsprung's disease delivered during 1990 to 2008 in the North of England reported to the Northern Congenital Abnormality Survey (NorCAS) formed this population-based case series. RESULTS Of 612,916 live births, 105 cases were reported to NorCAS. After excluding one diabetic and four multiple pregnancies, the live birth prevalence was 1.63 (95% confidence interval [CI], 1.33-1.98) per 10,000 live births. There was a significant temporal increase in the prevalence of Hirschsprung's disease ( p = 0.020), from 1.26 (95% CI, 0.80-1.89) in 1990 to 1994 to 2.29 (95% CI, 1.53-3.29) in 2005 to 2008. The ratio of male to female cases was 2:1. Ten (10.0%) cases occurred with Down syndrome, one with Smith-Lemli-Opitz Syndrome, and six (6.0%) with associated structural anomalies. The remaining 83 (83.0%) cases were isolated. All cases were live born, but nine (9.0%) died in the first year of life. Hirschsprung's disease was not prenatally suspected in any case. Half the cases were diagnosed within 5 days postpartum, but time of diagnosis ranged from birth to 5 years of age. CONCLUSION This study confirmed a male predominance and an association with Down syndrome, but also founda temporal increase in Hirschsprung's disease prevalence. No cases were suspected prenatally, but half were diagnosed within 5 days of life. Birth Defects Research (Part A), 2012. © 2012 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]