The changing epidemiology of Ebstein's anomaly and its relationship with maternal mental health conditions: a European registry-based study

OBJECTIVES: The aim of this study was to describe the epidemiology of Ebstein's anomaly in Europe and its association with maternal health and medication exposure during pregnancy.

DESIGN: We carried out a descriptive epidemiological analysis of population-based data.

SETTING: We included data from 15 European Surveillance of Congenital Anomalies Congenital Anomaly Registries in 12 European countries, with a population of 5.6 million births during 1982-2011. Participants Cases included live births, fetal deaths from 20 weeks gestation, and terminations of pregnancy for fetal anomaly. Main outcome measures We estimated total prevalence per 10,000 births. Odds ratios for exposure to maternal illnesses/medications in the first trimester of pregnancy were calculated by comparing Ebstein's anomaly cases with cardiac and non-cardiac malformed controls, excluding cases with genetic syndromes and adjusting for time period and country.

RESULTS: In total, 264 Ebstein's anomaly cases were recorded; 81% were live births, 2% of which were diagnosed after the 1st year of life; 54% of cases with Ebstein's anomaly or a co-existing congenital anomaly were prenatally diagnosed. Total prevalence rose over time from 0.29 (95% confidence interval (CI) 0.20-0.41) to 0.48 (95% CI 0.40-0.57) (p<0.01). In all, nine cases were exposed to maternal mental health conditions/medications (adjusted odds ratio (adjOR) 2.64, 95% CI 1.33-5.21) compared with cardiac controls. Cases were more likely to be exposed to maternal β-thalassemia (adjOR 10.5, 95% CI 3.13-35.3, n=3) and haemorrhage in early pregnancy (adjOR 1.77, 95% CI 0.93-3.38, n=11) compared with cardiac controls.

CONCLUSIONS: The increasing prevalence of Ebstein's anomaly may be related to better and earlier diagnosis. Our data suggest that Ebstein's anomaly is associated with maternal mental health problems generally rather than lithium or benzodiazepines specifically; therefore, changing or stopping medications may not be preventative. We found new associations requiring confirmation.

Presence and possible cause of periodicities in 20th-century extreme coastal surge: Belfast Harbour, Northern Ireland.

Identifying 20th-century periodic coastal surge variation is strategic for the 21st-century coastal surge estimates, as surge periodicities may amplify/reduce future MSL enhanced surge forecasts. Extreme coastal surge data from Belfast Harbour (UK) tide gauges are available for 1901–2010 and provide the potential for decadal-plus periodic coastal surge analysis. Annual extreme surge-elevation distributions (sampled every 10-min) are analysed using PCA and cluster analysis to decompose variation within- and between-years to assess similarity of years in terms of Surge Climate Types, and to establish significance of any transitions in Type occurrence over time using non-parametric Markov analysis. Annual extreme surge variation is shown to be periodically organised across the 20th century. Extreme surge magnitude and distribution show a number of significant cyclonic induced multi-annual (2, 3, 5 & 6 years) cycles, as well as dominant multi-decadal (15–25 years) cycles of variation superimposed on an 80 year fluctuation in atmospheric–oceanic variation across the North Atlantic (relative to NAO/AMO interaction). The top 30 extreme surge events show some relationship with NAO per se, given that 80% are associated with westerly dominant atmospheric flows (+ NAO), but there are 20% of the events associated with blocking air massess (− NAO). Although 20% of the top 30 ranked positive surges occurred within the last twenty years, there is no unequivocal evidence of recent acceleration in extreme surge magnitude related to other than the scale of natural periodic variation.