Neurodevelopmental status and follow-up in preschool children with heart disease in London, UK.

OBJECTIVE: To describe neurodevelopment and follow-up services in preschool children with heart disease (HD). DESIGN: Secondary analysis of a prospectively collected multicentre dataset. SETTING: Three London tertiary cardiac centres. PATIENTS: Preschool children<5 years of age: both inpatients and outpatients. METHODS: We analysed results of Mullen Scales of Early Learning (MSEL) and parental report of follow-up services in a representative convenience sample evaluated between January 2014 and July 2015 within a previous study. RESULTS: Of 971 preschool children: 577 (59.4%) had ≥1 heart operation, 236 (24.3%) had a known diagnosis linked to developmental delay (DD) ('known group') and 130 (13.4%) had history of clinical event linked to DD. On MSEL assessment, 643 (66.2%) had normal development, 181 (18.6%) had borderline scores and 147 (15.1%) had scores indicative of DD. Of 971 children, 609 (62.7%) were not receiving follow-up linked to child development and were more likely to be under these services with a known group diagnosis, history of clinical event linked to DD and DD (defined by MSEL). Of 236 in known group, parents of 77 (32.6%) and of 48 children not in a known group but with DD 29 (60.4%), reported no child development related follow-up. DD defined by MSEL assessment was more likely with a known group and older age at assessment. CONCLUSIONS: Our findings indicate that a 'structured neurodevelopmental follow-up pathway' in preschool children with HD should be considered for development and evaluation as children get older, with particular focus on those at higher risk.

Prenatal screening for major congenital heart disease: assessing performance by combining national cardiac audit with maternity data.

OBJECTIVE: Determine maternity hospital and lesion-specific prenatal detection rates of major congenital heart disease (mCHD) for hospitals referring prenatally and postnatally to one Congenital Cardiac Centre, and assess interhospital relative performance (relative risk, RR). METHODS: We manually linked maternity data (3 hospitals prospectively and another 16 retrospectively) with admissions, fetal diagnostic and surgical cardiac data from one Congenital Cardiac Centre. This Centre submits verified information to National Institute for Cardiovascular Outcomes Research (NICOR-Congenital), which publishes aggregate antenatal diagnosis data from infant surgical procedures. We included 120 198 unselected women screened prospectively over 11 years in 3 maternity hospitals (A, B, C). Hospital A: colocated with fetal medicine, proactive superintendent, on-site training, case-review and audit, hospital B: on-site training, proactive superintendent, monthly telemedicine clinics, and hospital C: sonographers supported by local obstetrician. We then studied 321 infants undergoing surgery for complete transposition (transposition of the great arteries (TGA), n=157) and isolated aortic coarctation (CoA, n=164) screened in hospitals A, B, C prospectively, and 16 hospitals retrospectively. RESULTS: 385 mCHD recorded prospectively from 120 198 (3.2/1000) screened women in 3 hospitals. Interhospital relative performance (RR) in Hospital A:1.68 (1.4 to 2.0), B:0.70 (0.54 to 0.91), C:0.65 (0.5 to 0.8). Standardised prenatal detection rates (funnel plots) demonstrating inter-hospital variation across 19 hospitals for TGA (37%, 0.00 to 0.81) and CoA (34%, 0.00 to 1.06). CONCLUSIONS: Manually linking data sources produced hospital-specific and lesion-specific prenatal mCHD detection rates. More granular, rather than aggregate, data provides meaningful feedback to improve screening performance. Automatic maternal and infant record linkage on a national scale, requires verified, prospective maternity audit and integration of health information systems.