Surgical timing and mortality in nonsevere paediatric congenital heart disease: A EUROlinkCAT cohort analysis across Europe

Background: Management and outcomes amongst children with congenital heart defects (CHD) are relatively heterogeneous. Traditionally, the highest-risk conditions and procedures have received the most attention, with less focus on outcome for less severe CHDs. Objectives: Evaluate the management of children born with certain less severe CHDs across Europe. Methods: This population-based linkage cohort study included children diagnosed with ventricular septal defect (VSD), atrial septal defect (ASD), pulmonary valve stenosis (PS), and patent arterial duct (PDA) from nine European regions. Data on surgery/intervention and survival from 1995 to the age of 10 years or until the end of 2014 were collected through electronic linkage to mortality and hospital databases. Kaplan-Meier survival estimates and meta-analyses were used to analyze the proportion of children undergoing surgery/intervention and survival rates. Results: The study included 20,608 children with the less severe CHDs. Surgical correction rates for the anomaly within the first year of life varied significantly, particularly for VSD. The median age for initial surgery/intervention was consistent for VSD but varied for other conditions. Most conditions were corrected in a single procedure, except for PS, which required a median of 1.7 (95 % CI; 1.4–2.0) interventions. Postoperative mortality was highest for neonates undergoing VSD surgery/intervention, 8.7 (95 % CI; 4.0–15.8) deaths per 100 surgeries. The overall 5-year survival rate for all conditions was approximately 98 %. Conclusion: There was considerable variation in the management of less severe CHDs across Europe, particularly in the timing of surgical interventions. Despite these differences, the overall 5-year survival rate is comparable and high.