Abstract

Introduction: Little is known regarding optimal timing of the Fontan operation and whether timing affects outcomes in patients with single-ventricle heart disease. The association of patient-level factors with age at the Fontan operation and the degree of practice variation among surgical centers have not been quantified in large studies. Methods: A retrospective cohort study of patients who underwent a Fontan operation between 1/1/2007 and 12/31/2017 at Pediatric Health Information Systems Database centers was performed to evaluate associations between patient characteristics and center on timing of Fontan operation. Mixed-effects multivariable linear regression was used to adjust for patient characteristics and center-level covariance. Results: In all, 7,840 patients (41% male, 57% white) at 46 centers were included. Median age at Fontan was 3.3 years (IQR 2.5-4.3). In an adjusted model hypoplastic left heart syndrome (-16 months, 95% CI -18 to -13), heterotaxy (-9 months, 95% CI -13 to -5), and ECMO history (-11 months, 95% CI -17 to -5) were associated with younger age at Fontan. Non-cardiac diagnoses (+4 months, 95% CI 0.1-9) and technology-dependence (+21 months, 95% CI 16-25) were associated with older age at Fontan ( Figure 1A ). There was no evidence of racial differences in Fontan timing. Children with Medicaid insurance were significantly younger at Fontan than counterparts with other insurance. We observed center effects on age at Fontan (p<0.001, ICC 0.03 95% CI 0.02-0.05) with youngest median age of 0.9 years (IQR 0.6-2.8) at one center and oldest median age of 5.0 years (IQR 4.6-5.9) at another center ( Figure 1B ). Conclusion: In this study, age at Fontan was significantly associated with cardiac anatomy, non-cardiac diagnoses, ECMO history, and insurance but not race. There was practice variation between centers in Fontan timing. Future studies will examine associations between timing of Fontan and patient outcomes.

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