Survival and risk factors for mortality among individuals with congenital heart disease

Abstract

With advances in medical, surgical and intensive care interventions, more individuals with congenital heart disease (CHD) are surviving infancy. However, long-term survival is not well researched. Given that UK paediatric cardiovascular services are undergoing reforms to ensure there are adequate health-care provisions, further information is required on CHD prevalence and survival. An analysis of data from six British Isles Network of Congenital Anomaly Registers (BINOCARs), showed no overall trend in CHD prevalence between 1991 and 2010. However, there was an increasing trend in the prevalence of tetralogy of Fallot, equating to a yearly excess of 16 cases in England and Wales. There was an increased risk of CHD in twins, particularly monochorionic (MC) twins. The prevalence of CHD in MC twins increased over time, equating to a yearly excess of seven cases in England and Wales. Using a systematic review and meta-analysis, pooled five and 10-year survival was 85.4% and 81.4%, respectively. Year of delivery, preterm delivery, extra-cardiac anomalies (ECAs) and birth weight were associated with mortality. In an analysis of data from one BINOCAR linked to death registrations, one-year survival was 89.1%, decreasing to 85.2% at 20 years. Less recent year of delivery, lower gestational age, low birth weight, prenatal diagnosis and the presence of ECAs increased the risk of mortality. The predicted 20-year survival of individuals born with isolated CHD in 2015 was 98.7%. The predicted prevalence of CHD was 74.0 and 68.8 per 10,000 live births in 2015 and 2020, respectively. Using ONS data to extrapolate, this equates to approximately 296,000 cases of CHD being born between 2012-2017 in the UK. Given that infants with CHD require complex surgeries, the predicted prevalence and survival estimates described in this thesis are important for health service planning and for providing accurate information to parents when a CHD is diagnosed prenatally.