Long-Term Outcomes of the Non-Fenestrated Strategy for Extracardiac Total Cavopulmonary Connection

The discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy.

Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy. Primary outcomes were Fontan-related events, including death, reoperation, catheter intervention, and postoperative complications such as arrhythmias, protein-losing enteropathy, plastic bronchitis, thrombosis, bleeding, and drainage of pleural effusion after discharge.

The median follow-up duration was 15.7 years (interquartile range, 15.3-18.4). The 1-, 2-, 3-, 5-, 10-, and 15-year occurrence of Fontan-related events was 24%, 43%, 44%, 55%, 67%, and 76%, respectively. The 1-, 2-, 3-, 5-, and 10-year occurrence of venovenous collaterals was 16%, 60%, 65%, 72%, and 81%, respectively. Ventricular end-diastolic pressure was higher in patients with venovenous collaterals (median, 5.0 mm Hg; interquartile range, 4.0-7.0 mm Hg) than in those without (median, 3.5 mm Hg; interquartile range, 3.0-4.25 mm Hg) (P = .01). Multivariable Cox regression analysis showed that higher ventricular end-diastolic pressure and younger age at Fontan completion significantly increased the risk of developing venovenous collaterals, with hazard ratios of 1.22 for each (95% CI, 1.052-1.41; P = .0085 and .016, respectively).

The occurrence rate of Fontan-related events was acceptable with the non-fenestrated Fontan strategy, whereby venovenous collateral development was common. The results suggest that patients with high ventricle end-diastolic pressure and young patients might benefit from fenestration.