Surgical results of patients with a functional single ventricle

Overall mortality of the patients with a functional single ventricle is still high, though excellent mid- and long-term results of the modified Fontan procedure have been reported. This study assessed the factors that affect the surgical outcomes mainly in the pre-Fontan stage and performed long-term survival analysis.

Between January 1988 and December 2000, 405 patients with a functional single ventricle underwent surgical interventions and were followed up until June 2001. The mean follow-up period was 74.5+/-69.4 months and 95% of the patients were followed up completely. Their median age was 2.5 months at the time of shunt or pulmonary artery banding (PAB), 8.6 months at BCPS, and 28.6 months at the Fontan operation. The variables of the anatomical lesions with single ventricle physiology, combined abnormalities, surgical pathways leading to the Fontan stage, age at operation, study periods, and type of the Fontan procedure were analyzed. The role of BCPS in the long-term results was evaluated.

Overall survival after birth was 60.1+/-2.8% at 10 years. In multivariate analysis, complete atrioventricular septal defect-typed lesion, pulmonary venous obstruction, total anomalous pulmonary venous connection, and interruption of aortic arch were risk factors for long-term survival, while pulmonary stenosis was demonstrated as a favorable prognostic factor. In this study, there was no significant survival difference between the early and late study period. Actuarial mortality in the pre-Fontan stage was 41.3% in the non-BCPS group and 16.3% in the BCPS group (P<0.001). The 10-year survival rates of the populations in staged and primary Fontan groups were not significantly different (P=0.24). The long-term survival rate of the atriopulmonary Fontan group was significantly lower than that of lateral tunnel Fontan (60.3+/-6.3% vs. 86.8+/-3.1% at 10 years, P=0.0001).

This study revealed that the overall survival was disappointing and there were still problems that need to be solved in the pre-Fontan stage to improve the overall survival. The role of BCPS was not to contribute to the longer survival after Fontan operation, but to lower mortality in the pre-Fontan stage, which can offer a higher probability to proceed to the Fontan procedure successfully.