Trends in one-year survival and pregnancy termination in congenital heart defects in 1994–2024: a population-based registry

To assess trends in one-year survival among infants with congenital heart defects (CHD) and in termination of pregnancy for fetal anomaly (TOPFA), with particular attention to isolated and severe isolated CHD. 5,898 CHD from the Paris Registry of Congenital Anomalies (1994–2024) were included. Isolated CHD were defined as CHD without associated genetic or extracardiac anomalies, as opposed to non-isolated CHD. One-year survival was estimated using Kaplan–Meier methods. Temporal trends in survival and TOPFA among pregnancies with a recorded prenatal diagnosis of CHD, were analyzed using logistic regression. Non-linearity (fractional polynomials) and slope changes (segmented models) were assessed. Among 5,898 CHD cases, 4,391 were live births, 1,361 TOPFA and 146 stillbirths. One-year survival was 91.0% (95% CI 90.1–91.8), reaching 95.0% (95% CI 94.2–95.7) in isolated CHD and 84.0% (95% CI 81.4–86.2) in severe isolated CHD. Overall survival improved significantly over time (p < 0.001), with a change in slope around 2007–2009; the absolute annual gain decreased from approximately + 0.5% (95% CI 0.26–0.68) at the beginning of the study period to + 0.2% (95% CI 0.16–0.23) in recent years. Similar improvement was observed in isolated CHD, with smaller gains after approximately 2009, and in severe isolated CHD (+ 0.56% per year; 95% CI 0.28–0.84). Among prenatally diagnosed pregnancies, TOPFA decreased in isolated CHD (− 0.50% per year; 95% CI − 0.74 to − 0.26) and severe isolated CHD (− 0.36%; 95% CI − 0.69 to − 0.03). One-year survival improved over three decades, although gains became smaller over time rather than showing a uniform plateau across CHD subgroups. Improvement extended to severe isolated CHD and occurred alongside declining TOPFA among prenatally diagnosed isolated and severe isolated CHD.