Fontan completion rate and outcomes after bidirectional cavo-pulmonary shunt

Objective: To determine outcomes of patients undergoing a bidirectional cavo-pulmonary shunt (BCPS) in the Fontan era.

Methods: From 1990 to 2000, 212 patients underwent a BCPS in a single institution at a mean age of 2.6+/-5 years.

Results: Hospital mortality was 6% (13 patients). Five patients were lost to follow-up (3%). After a mean of 9+/-4 years, 15 patients had not yet been referred for Fontan surgery and their status was deemed satisfactory (11 1(1/2) ventricle repair, three BCPS and one patient converted to a bi-ventricular repair). Out of the remaining 179 patients who could potentially have reached a Fontan status, 141 had undergone the procedure and seven were awaiting Fontan completion. The expected completion rate of Fontan surgery after BCPS was 83% (148/179). Thirteen patients died and heart transplantation was necessitated in nine patients. Nine were deemed unsuitable for Fontan surgery for cardiac (seven), respiratory (one) or neurologic (one) reasons. After BCPS, the only predictors of late failure (death, heart transplantation or NYHA class III-IV) were not reaching Fontan status and the presence of bilateral superior vena cava (SVC). Ten-year survival was 99% (95% confidence interval (CI): 95-99%) for Fontan patients and 67% (95%CI: 50-80%) for those left with a BCPS.

Conclusion: In the present era, staging with BCPS represents a selection process in the Fontan pathway. Almost a fifth of the patients will not reach Fontan status. Outstanding results after Fontan surgery may hide a high attrition rate between BCPS and Fontan.