Predictors of survival in children born with congenital diaphragmatic hernia: a registry-based study

Rute Vieira, Judith Rankin

Research output: Contribution to journalAbstractpeer-review


Background: Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly associated with high neonatal mortality. This study aims to evaluate the predictors for survival in infants born with CDH. Methods: A cohort study of prospectively collected data on all liveborn infants with CDH notified to the population-based UK Northern Congenital Abnormality Survey (NorCAS), and delivered between 01-Jan-1985 and 31-Dec-2003, matched to hospital and national mortality records to 29-Jan-2008. Survival to 20 years was estimated using Kaplan-Meier methods. Multiple logistic regression was used to identify overall predictors of mortality while Royston-Parmar survival models were used to examine predictors of survival at 1-week and 1-month within the children that died. Results: There were 246 CDH singleton infants notified to NorCAS during the study period, a total prevalence of 3.8 (95%CI:3.3,4.3) CDH cases per 10,000 pregnancies. Of these, 18.3% resulted in termination of pregnancy, 7.3% in spontaneous fetal loss (miscarriage≥20 weeks and stillbirth) and 74.4% in live births. Forty-seven percent of the live births resulted in death. Year of birth (per year; OR=0.88;95%CI:0.82,0.95;p<0.001), birth weight (per 100g;OR=0.92;95%CI:0.86,0.98; p=0.007), most deprived (compared to moderately deprived;OR=2.98;95%CI:1.28,6.92;p=0.01), prenatal diagnosis (OR=5.0;95%CI:2.17,11.4;p<0.001) and presence of other congenital anomalies (OR=5.3;95%CI:1.46,18.7;p=0.01) were significant predictors of mortality. Within the children that died, year of birth (per year; Hazard Ratio(HR)=0.95;95%CI:0.91,0.99;p<0.001) was the only significant predictor of survival at 1-week and 1-month. Conclusions: Identifying predictors of survival is important for prenatal counselling of parents whose pregnancy is affected by CDH and for health care planning for the future care needs of affected children.
Original languageEnglish
Pages (from-to)503
JournalBirth Defects Research Part A: Clinical and Molecular Teratology
Publication statusPublished - 2016
EventEUROCAT Scientific Symposium 2016 - Milan, Italy
Duration: 16 Jun 201616 Jun 2016


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