A cohort evaluation of surgical pathways reported to a national audit of children undergoing treatment for congenital heart disease in England and Wales.

Objective: To ascertain rates of completion of essential cardiac procedures and their overall contribution to longer-term mortality in children with congenital heart disease (CHD).

Methods: In this cohort study using the United Kingdom National CHD Audit, we described the pathway operations required for treatment for 9 sentinel CHDs-hypoplastic left heart syndrome (HLHS), non-HLHS functionally univentricular heart, ventricular septal defect, tetralogy of Fallot, coarctation, aortic stenosis, atrioventricular septal defect, transposition of the great arteries, and pulmonary atresia-that were undertaken at the population level and report the mortality associated with these pathway operations by age 5 years.

Results: Among 28,806 patients, over a median follow-up of 9.8 years (interquartile range, 4.2-15.4 years), 839 (2.9%) had undergone pre-pathway procedures only, 1135 (3.9%) had undergone initial palliation only (inclusive of functionally univentricular and biventricular reparative pathways), 2001 (6.9%) had undergone stage 2 palliation and/or total cavopulmonary connection (TCPC), 2254 (7.8%) had undergone staged biventricular repair after palliation, and 22,572 (78.3%) had undergone primary biventricular repair. Of the 23,239 children with 5 years of follow-up, 1794 (7.7%) had died by age 5 years, consisting of 409 (1.8%) who died following an initial palliative procedure, 43 (0.2%) who died following a stage 2 palliation or TCPC, 379 (1.6%) who died following a biventricular reparative procedure, and 963 (4.1%) who died in other circumstances, such as interstage or following reinterventions.

Conclusions: The outcome metrics of surgical pathway completion (biventricular repair or TCPC) and overall mortality at age 5 years can be evaluated using registry data and could contribute to future assessment of overall CHD service quality.