Objectives: In 1992, an analysis of our experience with the cavopulmonary shunt (CPS) demonstrated equivalent long-term survival, with or without subsequent conversion to a Fontan circulation. Before 1992 (era 1) intervention was deferred until mandated by clinical deterioration. Since 1992 (era 2), timing of both CPS and Fontan was compressed in an effort to improve survival. Survival following CPS is analyzed to ascertain whether Fontan confers any survival advantage over no further definitive intervention.
Methods: From 1962 to 1997 inclusive, 490 patients underwent CPS, excluding those who had a CPS concomitant with a Fontan. In 55 patients the CPS was performed at or after a biventricular repair (BVR), or after a Fontan, and these patients are excluded. The 435 patients remaining followed a surgical protocol which included a subsequent BVR (n = 28), or a subsequent Fontan operation (n = 220), or no further definitive surgery (CPS only, n = 187). Between eras the mean age at surgery decreased for all procedures.
Results: Long-term survival 20 years after a CPS in 435 patients is 56 +/- 5%. Survival at 20 years among the 220 patients who were subsequently converted to a Fontan circulation is 65 +/- 8% compared to 50 +/- 11% for the 187 patients who did not have a Fontan. However, most of their survival difference is because all early deaths after a CPS occurred in the non-Fontan group. Multivariable analysis demonstrated that proceeding to a Fontan did have a small survival advantage which was not evident by univariate analysis. Independent risk factors for death, at any time, are a common atrioventricular valve, or pulmonary artery banding. The era had no effect on survival.
Conclusions: The single ventricle circulation appears to have a limited durability of, an average, 30-40 years. There is a slight survival advantage in converting patients after a CPS to a Fontan circulation. A marked reduction in age at CPS and at Fontan has, as yet, not improved survival.