Fontan palliation in the modern era: factors impacting mortality and morbidity

Advances in management of the Fontan patient include interval superior cavopulmonary shunt, total cavopulmonary connection, either lateral tunnel or extracardiac conduit, and the use of a fenestration. Coincident with these improvements, Fontan palliation has been applied to a wider ranger of anatomic subgroups.

A cross-sectional analysis of 256 consecutive patients undergoing a total cavopulmonary connection Fontan after superior cavopulmonary shunt between January 1, 1994, and June 30, 2007 were studied. Fenestration was used selectively. Fontan failure was defined as death, transplant, or takedown. Event-free survival was defined as freedom from death, transplant, Fontan takedown, functional class III to IV, pacemaker, antiarrhythmic medication, protein-losing enteropathy, stroke, or thrombus.

Survival was 97% +/- 1%, 96% +/- 1%, and 94% +/- 2%, respectively, at 1, 5, and 10 years. Event-free survival was 96% +/- 1%, 87% +/- 3%, and 64% +/- 6%, respectively, at 1, 5, and 10 years. Factors predicting worse event-free survival included longer cross-clamp time (p = 0.003), fenestration (p = 0.014), and longer hospital length of stay (p = 0.016). Ventricular morphology did not predict outcome. Left ventricle (n = 113, 44%) versus right ventricle (n = 142, 56%) failure-free survival (death, transplant, or Fontan takedown) at 10 years was 92% +/- 4% versus 91% +/- 3%, respectively (p = 0.19). Left ventricle versus right ventricle event-free survival at 10 years was 75% +/- 7% versus 67% +/- 9%, respectively (p > 0.1).

Survival for patients undergoing a completion Fontan in the current era is excellent, but patients remain at risk for morbid events. In the intermediate follow-up period, we could not identify a difference in outcome between dominant left and right ventricle morphology.