• Matteo Trezzi, Enrico Cetrano, Salvatore Giannico, Fiore S Iorio, Sonia B Albanese, and Adriano Carotti.
• Department of Pediatric Cardiac Surgery, Bambino Gesú Children's Hospital IRCCS, Rome, Italy. Electronic address: trezzim@hotmail.com.
• Ann. Thorac. Surg. 2018 Feb 1; 105 (2): 599-605.

BackgroundAcute failure of the Fontan circulation is rare but remains associated with high morbidity and mortality rates. Little is known about the long-term outcome of patients who underwent Fontan takedown to an intermediate palliative circulation and their potential candidacy for redo Fontan completion.MethodsPatients followed up at a single institution who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 2 months of extracardiac Fontan completion were reviewed.ResultsBetween October 1990 and December 2015, 18 patients underwent Fontan takedown to a superior cavopulmonary connection (with or without an additional shunt) at a median age of 3.3 years (range, 1.8 to 8.0) and median weight of 13.8 kg (range, 8.0 to 27.0 kg). Takedown was required during the Fontan procedure itself in 2 patients, and within the first 2 postoperative months in 16 patients (median time to takedown, 3 days). Seventeen patients survived the post-takedown period and 3 ultimately underwent successful redo Fontan. Four patients required heart transplantation, with 2 deaths. In patients with extended intermediate palliation, median arterial oxygen saturation was 84% (range, 76% to 92%) at a median follow-up of 6.3 years (range, 0.7 to 25.9).ConclusionsTakedown to a superior cavopulmonary connection is an effective treatment option and, in some patients, acts as a bridge to subsequent redo Fontan completion or heart transplantation. An extended intermediate palliative circulation is tolerated for several years with reasonable oxygen saturation levels at rest. In our experience, an early takedown strategy to a superior cavopulmonary connection is the treatment of choice for acute Fontan failure.Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

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