Modified Aortic Arch Reconstruction Combined With Pulmonary Artery Reconstruction in the Comprehensive Stage II Operation Following a Hybrid Stage I for Hypoplastic Left Heart Syndrome

The hybrid approach for hypoplastic left heart syndrome is a well-accepted alternative strategy to the conventional staged surgical approach. However, in the typical comprehensive stage II operation, bilateral branch pulmonary artery stenosis is a risk factor for postoperative catheter and/or surgical interventions. In addition to addressing the stenosis from the bands, the branch pulmonary arteries are at risk of compression from the dilated main pulmonary trunk and the resultant large neo-aorta. Here, we present a new aortic arch reconstruction technique combined with the pulmonary artery reconstruction in the comprehensive stage II operation to address these issues. In our experience of 11 patients who underwent hybrid strategy, Fontan completion was achieved in 8 cases (73%) during a median follow-up duration of 84 months (range; 45-161 months), and the 3-year, 5-year, and 10-year survival rates were 73% each. While 6 of 8 cases (75%) required catheter interventions to the branch pulmonary arteries, the Nakata Index was 204 ± 11 before the stage II and 185 ± 19 before Fontan completion, indicating that good pulmonary artery growth. Our modified comprehensive stage II technique optimizes the pulmonary arteries for the Fontan and beyond by addressing both anatomic stenoses and external compression for future growth.