Long-term outcomes of hypoplastic left heart syndrome with analysis of the Norwood procedure in infants following bilateral pulmonary artery banding.

OBJECTIVE

To investigate the early and long-term outcomes of the deferred Norwood procedure by bilateral pulmonary artery banding (BPAB) versus the neonatal Norwood procedure.

METHODS

This retrospective study examined 46 patients with hypoplastic left heart syndrome and its variants undergoing the Norwood procedure for single ventricle physiology between 2004 and 2022 at 3 institutions. The patients were divided into 2 groups: neonatal Norwood procedure (group N; n = 23) and staged Norwood procedure in infants following BPAB (group I; n = 23). Preoperative risk factors, surgical results, survival rates, Fontan candidacy, and long-term complications were compared.

RESULTS

Early survival rates after the Norwood procedure were 91.3% (21 of 23) in both groups. Late survival rates after the Norwood procedure were similar at the 10-year follow-up (group N, 76.3%; group I, 68.7%;  = .63). Fontan completion rates also were comparable in the 2 groups (group N, 77.8%; group I, 85.7%;  = .67). Group N showed a higher median pulmonary artery (PA) index before bidirectional cavopulmonary connection (group N, 177 [interquartile range (IQR), 147-243] mm/m; group I, 152 [IQR, 146-163] mm/m;  = .03); this trend continued until 5 years after Fontan completion ( = .01). Group N also had a lower rate of freedom from protein-losing enteropathy (PLE) at 9.0 years after the Fontan operation (90.0% vs 52.5% for group I;  = .04), although the incidences of other Fontan-associated events were not significantly different.

CONCLUSIONS

Fontan candidacy and survival rates were similar regardless of the timing of the Norwood procedure. Early performance of the Norwood procedure may lead to lower rates of late Fontan-associated events, such as PLE.