Surgical reinterventions following the Fontan procedure.

OBJECTIVE: The Fontan procedure is utilized as a final reconstructive procedure for patients with functional single ventricle. Short- and long-term outcomes have improved significantly, however, some patients require additional cardiac procedures following the Fontan operation. The outcomes for these reinterventions are not known.

METHODS: Cardiac Surgery and Cardiac Intensive Care Unit databases at The Children's Hospital of Philadelphia were reviewed to identify all patients who underwent cardiac surgery after a previous Fontan operation between January 1, 1995 and December 31, 2001.

RESULTS: During the study period, 123 procedures were performed in 71 patients. The median time from Fontan to reoperation was 3.6 years (range 0.1-20 years). Indications for reintervention included arrhythmia, cyanosis, 'failing' Fontan circulation or exercise intolerance, protein losing enteropathy, atrioventricular valve (AVV) regurgitation, and other indications. Procedures included pacemaker insertion or revision (n = 59, 48%), reinclusion of previously excluded hepatic veins (n = 16, 13%), revision to either a lateral tunnel or extra-cardiac conduit Fontan (n = 13, 11%), cardiac transplantation (n = 9, 7%), enlargement or creation of a baffle fenestration (n = 6, 5%), isolated AVV repair or replacement (n = 2, 2%), and other procedures (n = 18, 14%). There were five early and five late deaths. Hospital mortality was greatest for patients undergoing cardiac transplantation (4/9, 44%), accounting for 80% of the early deaths.

CONCLUSIONS: Surgical reinterventions following the Fontan procedure may be necessary for multiple indications which result in impairment of the Fontan circulation. Most reinterventions can be performed with minimal morbidity and mortality. Survival for patients requiring cardiac transplantation following the Fontan procedure remains poor.