Long-term outcome of patients 25 years after Fontan operation – benefit of early Fontan completion

Background: Despite the improvement of perioperative mortality, functional deterioration late after Fontan operation still remains a relevant clinical issue. This study evaluates long-term outcome after Fontan operation.

Methods: We included 143 consecutive patients that underwent Fontan operation between 1984 and 2009at our institution. Modifications of Fontan operation included atriopulmonary anastomosis (APA; n=28), total cavopulmonary connection (TCPC; n=63), and fenestrated TCPC (f-TCPC; n=52). Mean age at operation was 7.8 (APA), 6.7 (TCPC), and 2.9 (f-TCPC) years, respectively. Mean follow-up was 10.7 years. A bidirectional Glenn shunt was performed in 48 patients (92%) before f-TCPC. Postoperative mortality, morbidity, and hemodynamics were analyzed.

Results: Actuarial survival was 71% at 25 years after APA, 78% at 20 years after TCPC, and 91% at 15 years after f-TCPC. There were 10 early deaths, 12 late deaths, and 2 takedowns followed by successful conversion and heart transplantation. Freedom from reoperation was 82% at 25 years. Freedom from re-intervention was 56% at 25 years. Freedom from tachyarrhythmia or pacemaker implantation was 28% and 85%, respectively at 25 years. The incidence of reoperations and late tachyarrhythmias was reduced in patients after f-TCPC. Postoperative cardiac index was significantly higher in patients after f-TCPC compared to other types of procedures.

Conclusion: Early Fontan completion with f-TCPC followed by bidirectional Glenn shunt provided better hemodynamics and improved survival and late morbidity. However, emerging complications should still be managed by surgical and interventional procedures. Fenestration in Fontan circulation may favorably influence long-term outcome.