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

Objective: We aimed 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: Cohort study using the United Kingdom National CHD Audit. 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) we described the pathway operations required for treatment that were undertaken at population-level, and the mortality associated with these pathway operations by age 5-years.

Results: Amongst 28,806 patients, over a median follow-up of 9.8(IQR:4.2-15.4) years, 839(2.9%) had undergone pre-pathway procedures only, 1,135(3.9%) had undergone initial palliation only (inclusive of functionally univentricular and biventricular reparative pathways), 2,001(6.9%) had undergone stage 2 palliation and or total cavopulmonary connection (TCPC); 2,254(7.8%) had received staged, biventricular repair after palliation, and 22,572(78.3%) had undergone primary biventricular repair. Of 23,239 children who had 5-years of follow-up, 1,794(7.7%) had died by the age of 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.