042 Challenging the “Ten Commandments” – Early Age at Fontan is not a Risk Factor for Long-Term Failure

Abstract

Age older than 4 years at time of Fontan is one of the original “Ten Commandments” (published in the early 1970s) for case selection. The relevance of this commandment in the modern era is unclear. A retrospective analysis of the ANZ Fontan Registry was undertaken. Patients were stratified by age at Fontan. Fontan prior to 3 years of age (early Fontan) was compared to Fontan undertaken in the combined second and third quartiles by age in the Registry (3.6-6.1 years; control). Outcomes included long-term failure (death, transplantation, NYHA III/IV heart failure, Fontan takedown or conversion, protein losing enteropathy, plastic bronchitis), arrhythmia and re-interventions. Between 1975 and 2017, there were 191 early and 781 control patients. Risk of developing early postoperative complications, duration of pleural effusion and hospitalisation were comparable between the two groups. Incidence of long-term failure was similar between the two groups (early: 1.08 failures per 100 patient-year follow-up; versus 0.99 for controls). The 5, 10, 15 and 20-year freedom from failure were 95%, 91%, 88% and 83% respectively in the early Fontan group, and 97%, 92%, 86% and 80% respectively in the control group (log rank p=0.8; Fig. 1). In the adjusted analyses, including for surgical era, early age at Fontan completion was not a risk factor for long-term failure (HR 1.29, 95% CI 0.84-1.97, p=0.24), new-onset arrhythmia (HR 1.01, 95% CI 0.67-1.52, p=0.95) or re-intervention (HR 1.18, 95% CI 0.88-1.58, p=0.28). Subgroup analysis (lateral tunnel and extracardiac conduit) produced similar results. Earlier age at operation (<3 years) is not a risk factor for short- or long-term adverse outcomes after the Fontan procedure.