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Background Whereas no global severity score exists for congenital heart defects (CHD), risk (Risk Adjusted Cardiac Heart Surgery‐1: RACHS‐1) and/or complexity (Aristotle Basic Complexity: ABC) scores have been developed for those who undergo surgery. Population‐based studies for assessing the predictive ability of these scores are lacking. Objective To assess the predictive ability of RACHS‐1 and ABC scores for the risk of infant mortality using population‐based cohort (EPICARD) data for newborns with structural CHD. Methods The study population comprised 443 newborns who underwent curative surgery. We assessed the predictive ability of each score alone and in conjunction with an a priori selected set of predictors of infant mortality. Statistical analysis included logistic regression models for which we computed model calibration, discrimination (ROC), and a rarely used but clinically meaningful measure of variance explained (Tjur's coefficient of discrimination). Results The risk of mortality increased with increasing RACHS‐1 and the ABC scores and models based on both scores had adequate calibration. Model discrimination was higher for the RACHS‐1‐based model (ROC 0.68, 95% CI, 0.58‐0.79) than the ABC‐based one (ROC 0.59, 95% CI, 0.49‐0.69), P = 0.03. Neither score had the good predictive ability when this was assessed using Tjur's coefficient. Conclusions Even if the RACHS‐1 score had better predictive ability, both scores had low predictive ability using a variance‐explained measure. Because of this limitation and the fact that neither score can be used for newborns with CHD who do not undergo surgery, it is important to develop new predictive models that comprise all newborns with structural CHD.

Background Assisted reproductive techniques (ART) are associated with a higher risk of tetralogy of Fallot (TOF) and multiple pregnancies may be associated with a higher risk of congenital anomalies. We assessed the extent to which the association between ART and risk of TOF may be mediated by the higher risk of multiple pregnancies associated with ART. Methods We conducted a case–control study using population-based data from the Paris Registry of Congenital Malformations for the period 1987–2009 and a cohort study of congenital heart defects (EPICARD). The study population included 395 cases of TOF and 4104 malformed controls with no known associations with ART. The analysis was based on a path-analysis model using a counterfactual approach, which allows decomposition of the total effect of ART into an indirect effect (that mediated by the association between ART and multiple pregnancies) and a direct effect. Results ART (all methods combined) were associated with a 2.6-fold higher odds of TOF after adjustment for maternal and paternal characteristics and year of birth (adjusted OR 2.6, 95% CI, 1.5-4.5). Most (79%) of the effect associated with ART was a direct effect (i.e., not mediated by multiple pregnancies), whereas 21% of the effect of ART was due to its association with multiple pregnancies (i.e., the indirect effect). In vitro fertilization with intracytoplasmic sperm injection was associated with a 3.5-fold higher odds of TOF (adjusted OR 3.5, 95% CI, 1.1-11.2); 11% of this effect was mediated through the association of ICSI with multiple pregnancies. Conclusions By far, most of the higher risk of TOF associated with ART is a direct effect and only a small proportion of the effect may be mediated by multiple pregnancies.

ObjectivesTo quantify the risk of preterm birth (PTB) for newborns with congenital heart defects (CHDs) conceived following infertility treatments, and to examine the role of multiple pregnancies in the association between infertility treatments and PTB for newborns with CHD.MethodsWe used data from a population-based, prospective cohort study (EPICARD EPIdémiologie des CARDiopathies congénitales) including 2190 newborns with CHD and excluding cases with atrial septal defects born to women living in the Greater Paris area between May 2005 and April 2008. Statistical analysis included logistic regression to take into account potential confounders (maternal characteristics, invasive prenatal testing, CHD prenatal diagnosis, medically induced labour/caesarean section before labour, birth year). The role of multiple pregnancies was assessed using a path-analysis approach, allowing decomposition of the total effect of infertility treatments on the risk of PTB into its indirect (mediated by the association between infertility treatments and multiple pregnancies) and direct (mediated by mechanisms other than multiple pregnancies) effects.ResultsPTB occurred for 40.6% (95% CI 28.7 to 52.5) of newborns with CHD conceived following infertility treatments vs 12.7% (95% CI 11.3 to 14.2) for spontaneously conceived newborns (p<0.001). After taking into account potentially confounding factors, infertility treatments were associated with a 5.0-fold higher odds of PTB (adjusted OR=5.0, 95% CI 2.9 to 8.6). Approximately two-thirds of this higher risk of PTB associated with infertility treatments was an indirect effect (ie, due to multiple pregnancies) and one-third was a direct effect (ie, not mediated by multiple pregnancies).ConclusionNewborns with CHD conceived following infertility treatments are at a particularly high risk of PTB, exposing over 40% of them to the ‘double jeopardy’ of CHD and PTB.