Long-term outcomes following Fontan takedown in Australia and New Zealand.

Objective: Fontan takedown remains an option for the management of Fontan failure. We sought to evaluate early and late outcomes after Fontan takedown.

Methods: The Australia and New Zealand Fontan Registry was interrogated to identify all patients who had a Fontan takedown.

Results: Over a 43-year study period (1975-2018), 36 of 1540 (2.3%) had a Fontan takedown. The median age at takedown was 5.1 years (interquartile range [IQR], 3.7, 7.0). Nine (25%) patients had a takedown within 48 hours, 6 (16%) between 2 days and 3 weeks, 14 (39%) between 3 weeks and 6 months, whereas 7 (19%) had a late takedown (>6 months). Median interval to takedown was 26 days (IQR, 1.5, 127.5). Sixteen (44%) patients died at a median of 57.5 days (IQR, 21.8, 76.8). The greatest mortality occurred between 3 weeks and 6 months (<2 days: 1/9, 11%; 2 days to 3 weeks: 2/6, 33%; 3 weeks to 6 months: 11/14, 79%; >6 months: 2/7, 28%; P = .007). At median follow-up of 9.4 years (IQR, 4.5, 15.3), 11 (31%) patients were alive with an intermediate circulation (10 in New York Heart Association class I/II). Five (14%) patients underwent a successful second Fontan. Freedom from death/transplant after Fontan takedown was 59%, 56%, and 52% at 1, 5, and 10 years, respectively.

Conclusions: The incidence of Fontan takedown is low, but mortality is high. The majority of takedowns occurred within 6 months. Mortality was lowest when takedown occurred <2 days and highest between 3 weeks and 6 months. A second Fontan is possible in a small proportion of survivors.